_id
stringlengths 7
16
| description
stringlengths 55
95.2k
|
|---|---|
pmc-6032628-1 | A 64-year-old man presented at the emergency department with colic pain in the left lumbar region. The pain started acutely and was ongoing for more than one day. There were no urinary complaints, no vomiting, no respiratory complaints and normal stools. His medical history showed a lung carcinoma for which he was currently being treated with chemotherapy, hypercholesterolemia and a compression fracture of D4. On clinical examination, there was a left costovertebral angle tenderness. Urinary analysis was negative for proteins, glucose, bilirubin, hemoglobin, red and white blood cells. Laboratory results showed only slightly elevated C-reactive protein of 5.4 mg/L (<5 mg/L), slightly decreased hemoglobin of 12.1 g/dl (13.1–17.2 g/dL), elevated urate of 71 mg/dl (16.6–48.5 ml/dL), elevated creatinine of 1.6 mg/dL (0.67–1.17) and estimated glomerular filtration rate > 60 mL/min/1.73m2.
B-mode abdominal ultrasound revealed normal anatomy of both kidneys and the bladder wall.
Unenhanced abdominal computed tomography (CT) showed no abnormalities. The patient was admitted for intravenous pain management and observation. Pain persisted and a follow-up abdominal ultrasound was performed. The kidneys remained morphologically normal on B-mode. Color Doppler ultrasound showed normal vascularization of the right kidney (Figure ). There was no arterial signal in the kidney hilum or in the renal cortex of the left kidney (Figure ) that exhibited only weak, alternating venous flow (Figure ). These findings were highly suspicious of left renal artery occlusion.
Because of progressively decreasing kidney function, a contrast-enhanced ultrasound (CEUS) was performed. This showed normal vascular supply of the right kidney with homogeneous enhancement of the cortex (Figure ). On the left, there was only minimal cortical enhancement which extended from the periphery to the hilum, representing perforating branches of the renal capsular artery (Figure ). This finding is also known as the cortical rim sign which is seen on contrast-enhanced CT- or Magnetic Resonance-images in case of renal infarction. The findings confirmed an occlusion of the left renal artery.
Because complaints started more than 72 hours prior to diagnosis, there was no indication for thrombolysis. Therapeutic doses of low molecular weight heparines were started to prevent new thrombotic events and an adequate level of pain killers was continued. Kidney function increased over the next few days with eGFR of 53 mL/min/1.73m2 and creatinine of 1.4 mg/dL at hospital discharge. |
pmc-6032629-1 | A 49-year-old woman presented at the otorhinolaryngology department with symptoms of repeated upper airway infections for six months. She complained of nasal obstruction, headaches, sneezing, hyposmia, postnasal drip and coughing. Treatment with antibiotics and oral steroids had no effect. She had already undergone functional endoscopic sinus surgery with septal correction and partial reduction of a right-sided hypertrophic concha media bullosa in 2008. Endoscopic nasal examination showed a bilateral oedematous mass located medially and cranially in the nose, originating anteriorly of the attachment of the concha media. A computed tomography (CT) (Figure ) was performed and demonstrated the presence of a bilateral well-delineated soft-tissue mass in the olfactory cleft. There was bone remodelling resulting in widening of the olfactory clefts, but no bone erosion. The mucosa in the paranasal sinuses was only modestly thickened and there was no evidence of sinonasal polyposis. The patient underwent a magnetic resonance (MR) scan for further work-up. On MR (Figure ) the lesions appeared T1- and T2-isointense compared to white matter. The cribriform plate was intact and there was no intracranial involvement. A biopsy was performed and the presence of REAH was histologically confirmed. Endoscopic non-aggressive resection was performed (Figure ) and in the follow-up consultation three weeks later the patient was free of symptoms. Nasal endoscopic control four months after surgery showed no signs of recurrence. |
pmc-6032648-1 | A 62-year-old woman was admitted to the Department of Gynecology at the Institute of Oncology Vojvodina with the complaint of vaginal bleeding for one year. Her past medical history was uneventful. The biopsy results of fractional curettage identified endometrial cancer (HP: carcinoma endometriodes, G2). She underwent total hysterectomy with bilateral adnexectomy. Histological findings confirmed the presence of endometrial cancer (HP: adenocarcinoma endometriodes endometrial, HG2, pT1c, FIGO Ic and Lieomyoma uteri). The right ovary was without pathological lesions, but the left ovary had a mature teratoma with dominant thyroid tissue and lesion of papillary cancer, 1,3 mm in diametar – malignant struma ovarii (Figs. , ).
Three months after completing brachytherapy, she underwent total thyroidectomy. Histological findings were without evidence of papillary cancer (HP: Struma colloides polynodosa glandule thyroideae).The stimulated thyroglobulin (tumor marker in histological confirmation of thyroid cancer) level was detectable (Tg, 8.8 ng/ml; TSH, 25.49 mIU/ml) and negative antithyroglobulin antibodies. We decided to apply the radioiodine therapy in a dose of 3,7GBq 131-J. Post therapy whole body scintigraphy did not show distant metastases. Two foci of 131-I uptake were seen in the neck (Fig. ). The patient receives suppressive hormone L-thyroxin therapy. One month after radioiodine ablation, she continued treatment of endometrial cancer (external beam therapy). The first post therapy check of hormonal status, Tg and ATA were in an optimal range. |
pmc-6032649-1 | A 25-year-old woman was referred to our radiology department by her family doctor for an MRI examination of the left knee because of a “crackling” noise of three months duration. There were no complaints of instability, swelling or pain. Physical examination showed anterior laxity of the knee. There was no recent history of trauma. The patient is known with a congenital shortening of the left leg for which she has already undergone a leg lengthening procedure.
The MRI examination shows multiple anatomic anomalies. The most notable is the absence of the anterior cruciate ligament (Fig. ). The posterior cruciate ligament is present but appears hypoplastic (Fig. ). The lateral intercondylar spine is absent and the lateral meniscus is hypoplastic (Fig. ). There is severe trochlear dysplasia due to hypoplasia of the lateral femoral condyle and medial patellar facet hypoplasia (Fig. ). Sequellae of earlier leg lengthening procedure can be seen: the left fibula is absent and metallic artefacts are present in the tibia.
Up to this date, the patient was treated conservatively. |
pmc-6032652-1 | We present the case of a 27-year-old woman who was referred to our hospital for further investigation, after worrying findings during a routine check-up performed in another hospital. Five years prior to this check-up, the patient was diagnosed with cancer of the left breast at the very young age of 22. The tumor was staged as pT2N0M0, with the histologic examination showing a poorly differentiated invasive ductal adenocarcinoma with strong estrogen and progesterone receptor expression and negative herceptin status. The treatment consisted of a wide excision and sentinel node procedure, followed by adjuvant chemotherapy, radiotherapy and hormonal therapy. Because of her young age, 3 cycles of cyclophosphamide-epirubicin-fluorouracil (FEC) and 3 cycles of docetaxel were given. Chemotherapy was followed by radiotherapy of the left breast up to a dose of 50 Gy, with a boost of 16 Gy on the tumor bed. Hormonal therapy consisted of a combination of tamoxifen and triptorelin. There was no relevant personal medical history, nor family history of breast cancer. Genetic analysis failed to show any BRCA1 or BRCA2 mutations.
The patient recovered well and follow-up examinations were normal. At the time of the check-up, five years after surgery, coinciding with the conclusion of the hormonal therapy, the follow-up mammogram (Fig. ) and first ultrasound showed the surgery related changes. (Fig. ) Because of her young age, magnetic resonance imaging (MRI) of the breasts was also performed (examination performed on Siemens Magnetom Symphony 1.5T), showing a multifocal nodular contrast enhancement in the retro-areolar region and the lower-outer quadrant of the right breast (Fig. ). These findings were not present on the previous MRI, performed two years after surgery. There were no clinical abnormalities in the region of this contrast enhancement. Because of the unclear etiology of these findings, the woman was referred to our department for further investigation. On ultrasonography, we were able to visualize some parenchymal distortion and an ill-defined hypoechoic lesion with a diameter around 1 cm and mild posterior acoustic shadowing in the region of the contrast enhancement on MRI (Fig. ). Because of the ultrasound and MRI findings and the patient’s history of breast cancer, the examinations were categorized as BIRADS 4. An ultrasound-assisted core biopsy (4 × 14 Gauge) was performed. Histologic examination showed a dense lymphocytic infiltrate of predominantly B-lymphocytes surrounding the ductulolobular unit and the vessels, in combination with a fibrous stroma of low cellularity and an increase in fibroblasts. These findings led to a diagnosis of sclerosing lymphocytic lobulitis. No signs of malignancy were detected.
After diagnosis of sclerosing lymphocytic lobulitis, follow-up consisted of a breast ultrasound every six months and a yearly mammogram and MRI of the breasts. The volume of the hypoechoic lesion on ultrasonography and the intensity of the gradual multifocal contrast enhancement on MRI have both progressively diminished on consecutive examinations, to a level where it is barely perceptible (Fig. and ). |
pmc-6032658-1 | A 46-year-old man with no relevant medical history presented at the emergency department with nausea and a vague epigastric abdominal pain. An initial ultrasound examination demonstrated an ileus of the small intestine with small bowel wall distention mainly in the peri-umbilical region.
Computed tomography (CT) confirmed a large mesenteric tumoral mass extending towards the ileum, where circumferential small bowel wall invasion caused intestinal obstruction (Figures and ). There was only a moderate amount of ascites. No signs of peritoneal carcinomatosis, distant metastases or free intra-peritoneal air were present.
The patient was subsequently referred for surgery, revealing an obstructive tumoral lesion in the ileum and a mass in the adjacent mesentery (Figure ). There was no peritoneal spread of disease. The affected ileum and mesentery were resected and an entero-enteric anastomosis was made.
The pathology examination confirmed an ileum tumor five centimeters in length, invading all layers of the bowel wall and a second, mesenteric mass six centimeters in length. Two out of nine lymph nodes were positive. On histology, the resected mass consisted of atypical cells with a high mitotic activity and an increased nuclear-cytoplasmatic ratio. Immunohistologic staining showed a high Ki-67 expression and highly positive myeloid markers such as MPO, CD-43, CD-117 and Lysozyme (Figure ). As such, the diagnosis of myeloid sarcoma was made.
The patient was referred to a tertiary center for further haematological work-up. Bone marrow aspiration showed no tumoral invasion. Induction chemotherapy was initiated and a stem cell transplantation was scheduled. PET-CT evaluation and haematological follow-up confirmed disease remission at the date of this publication. |
pmc-6032662-1 | A 28-year-old multiparous female presented to the Emergency Department with complaints of mild abdominal pain associated with nausea for two days. She described her pain episodes as mild in nature, located in the epigastric/left upper quadrant, nonradiating, and slight worsening with food intake. She denied fever, chills, vomiting, or diarrhea.
Her past medical history was significant for a similar presentation about 6 months before when she was diagnosed with idiopathic acute pancreatitis. She denied any alcohol intake and was not on any medications or herbal supplements.
On admission, she was afebrile with a pulse rate of 84 beats per minute and a blood pressure of 116/80 mm Hg. Examination of the abdomen revealed mild tenderness in the epigastric region/left upper quadrant region and also with a palpable mass in the left lower quadrant. Laboratory workup revealed elevated lipase (4337), unremarkable CBC, and, with normal liver functions tests, lipid panel and IgG panel. Ultrasound of abdomen showed minimal sludge in the gall bladder without any obvious stones. CT abdomen with contrast demonstrated a spleen in the anterior left lower abdomen, elongated pancreatic tail which was coiled in conjunction with the splenic vessels, and with mild inflammation of the pancreatic tail (Figures and ). She improved clinically with conservative management with IV fluids. She finally underwent splenopexy at an outside facility. |
pmc-6032663-1 | We discuss the case of a 74-year-old woman treated for breast cancer with bone metastases. Her past medical history also included pulmonary embolism, hypertension, appendectomy, hysterectomy, and aortic valvuloplasty. Annual follow-up ultrasonographic examination of the abdomen showed an unexplained pneumobilia (Fig. ). She was asymptomatic and during physical examination the patient’s abdomen was soft and she had neither jaundice nor diarrhea. Her blood workup was normal.
CT of the abdomen was then performed and confirmed massive pneumobilia and common bile duct dilatation with suspicion of lithiasis (Fig. ). A T2-weighted sequences magnetic resonance cholangiography (MRCP) displayed a wide bile duct dilatation with numerous large calculi filling the choledocus (Fig. ). The gallbladder was small and its fundus neighbored the hepatic flexure of the colon and duodenum (Fig. ). To clarify any potential biliary enteric fistulas, a second MRI was carried out using gadoxetic acid (Gd-EOB-DTPA, Primovist®). The opacification of bile ducts was unusually delayed. The MRI at 90 minutes finally showed the filling of the intrahepatic bile ducts, gallbladder, upper choledocus and opacification of the hepatic flexure of the colon via a fistula (Fig. ).
Because of her medical history, endoscopic treatment with sphincterotomy and common bile duct stone extraction was favored over surgery. |
pmc-6032667-1 | A 46-year old man presented to the emergency department with pain localized to the right costovertebral angle and associated shoulder pain. Laboratory findings showed raised inflammatory parameters (C-reactive Protein (CRP) 116 mg/dL, normal range 0–1.2 mg/dL). The patient had no fever (36°C). A non-contrast-enhanced CT-scan was performed to exclude kidney stones. No urinary tract calculi could be revealed. However, in liver segment 7, a high-density structure was retained surrounded by a hypodense zone of 25 mm, containing small air bubbles suggestive for an intrahepatic abscess (Figure and ). Review of the contrast enhanced CT-scan performed two weeks earlier on the occasion of an acute appendicitis learned that this intrahepatic calcification had the same characteristics (800 Hounsfield Units, 10 mm, round shape) as the appendicolith on the previous scan (Figure ). At that time, the patient was treated with laparoscopy, which revealed a necrotizing appendicitis with a small covered perforation. Following a five day course of antimicrobial therapy the patient was discharged home.
Due to the recent laparoscopic appendectomy for acute appendicitis, the CT-findings of this admission suggest a dropped appendicolith, which had spontaneously migrated into the liver parenchyma causing an intrahepatic abscess. There are no arguments for an iatrogenic lesion of the liver capsule during the recent appendectomy. During the second laparoscopic exploration, the appendicolith was extracted and the abscess was drained. Microbiology was positive for Escherichia coli. Intravenous antibiotics were administered over the following four days and the patient discharged. Up to now, the patient has remained well. |
pmc-6032668-1 | A 29-year-old Pakistani male was referred to the Oral Surgery Department for rehabilitation of the left edentulous mandible secondary to partial mandibulectomy surgery. He had undergone two operations for the left body of a mandible keratocyst odontogenic tumour (KCOT). The patient has been diagnosed with (KCOT) in Pakistan, where he received his first surgical treatment. A second surgical partial mandibulectomy was attempted due to recurrence. He has been reviewed regularly and he requested to have a replacement of his missing teeth on the lower left side due to a difficulty in eating and the effect on his appearance ().
Clinical examination reveals an asymmetrical face with a slightly depressed left lower body of the mandible on a class I skeletal pattern (Figures and ). The patient reported an absence of paraesthesia on the left mandible. The smile line was high exposing the gingiva on the upper maxillary incisors.
Intraorally, the oral hygiene was fair with the presence of mild gingivitis. The dentition on the maxillary arch was unrestored (). The left edentulous mandible was irregular basal bone with firm mucosa covering the bone from 41 until 37. There were marked loss of bony structure horizontally and vertically. This has caused the tongue to occupy the space that used to be occupied by teeth and alveolus in the left mandibular segment (Figures , , and ). The healing of the operation site was uneventful (Figures and ).
An orthopantomogram was taken to evaluate the remaining bony structure of the mandible (). Radiographically, the operation site (lower left posterior segment) has no abnormalities. The remaining basal bone was adequate in thickness to support the mandible with an irregular margin. There are no radiopaque abnormalities suggestive of new pathology. |
pmc-6032763-1 | A 55-year-old male patient with nuchal pain at C3–C4 level radiating to the left arm was referred for exclusion of a disc herniation. He underwent a cervical CT, not showing a disc herniation. However, it revealed a right-sided accessory articulation between the anterior transverse processes of C6 and C7. The transverse foramina of C6 and C7 showed a partial defect, respectively posterior and anterior. Clearly, the accessory articulation was an incidental finding, as being contralateral to the symptomatic side. |
pmc-6032769-1 | This was a rare case of PMB-iSH in a 21-year-old female in China. In her postpartum period, the patient suffered from chest pain, fever and even coma for a fortnight. She was sent to the local hospital due to cardiac arrest by 4 times on 27th January 2017. After CPR, she regained consciousness gradually but still was in a continuous febrile state. Klebsiella pneumoniae was isolated from the samples of blood and sputum cultures. Besides, anti-microbial therapy hadn’t worked effectively since she was treated with cefepime, imipenem and tigecycline.
The patient was soon admitted to the emergency intensive care unit (EICU) of Ruijin Hospital affiliated to Shanghai Jiao Tong University on 26th April. Upon admissionto our hospital, she was still in fever, unconscious in a dyspneic state, and mechanical ventilation was initiated after tracheotomy with metal tracheal tube.
A full-body computed tomography (CT) scan identified thickened pericardium, bilateral pleural effusion with multiple exudative focuses, hepatosplenomegaly and pelvic effusion in this patient. Empiric antibiotic treatment was started for Klebsiella pneumoniae infection with piperacillin-tazobactam (4.5 g, intravenously, q.8 h). The sample of microbial sputum culture on 29th April revealed that a large amount of multi-drug resistant Klebsiella pneumoniae (MDR-KP) grew, which was merely susceptible to tigecycline, sulfamethoxazole (SMZ) and PMB. The infection parameters from laboratory examination increased remarkably: hypersensitive C-reactive protein 37.0 mg/L [0~ 3 mg/L] and procalcitonin 3.37 ng/mL [< 0.50 ng/mL]. In light of the above-mentioned examination results, we replaced piperacillin-tazobactam with meropenem (2 g, intravenously, q.8 h) and tigecycline (100 mg intravenously q.12 h) with the addition of colistin (1-million unit by aerosol inhalation q.8 h) on 4th May.
After the adjustment for the treatment, her body temperature dropped to normal and remained stable. During this period, repeated cultures of blood, sputum, vaginal secretion and fluid drained from the hip joint were carried out. MDR-KP, susceptible to tigecycline, SMZ and PMB was detected in all the samples. On 18th May, multiple exudations were aggravated in bilateral lungs compared to that upon admission, according to a chest CT scan. As a result, PMB (500,000 units, intravenously, q.12 h) was administered at once. Four days later, there were multiple red rashes spread on the whole body skin. With the rashes fading away, SH with dark round spots was seen on 23rd May (5 days after intravenous PMB) without pain or pruritus. More dark brown spots were spread on the skin of trunk and limbs, especially on that of lower abdomen, right hand and right foot as depicted in Figs. , , and , although the skin of the head and neck was also darkened evidently. Despite this adverse event, the therapeutic regimen was still applied in consideration of MDR-KP infection of multiple organs. PMB was utilized in the same dosage until the patient was transferred back to the local hospital, and skin biopsy wasn’t performed.
On 21st May, she suffered from diarrhea suddenly with intra-abdominal pressure (IAP) increasing to 19 cm H2O. About 500 ml black stool was found in the patient’ s excrement. Her serum creatinine was elevated dramatically to 430 μmol/L [53~ 97 μmol/L], and anuria occurred. The laboratory examinations revealed the following results: the level of hemoglobin was significantly reduced to 57 g/L [113~ 151 g/L] and platelet counts 63 × 109/L [101~ 320 × 109/L]. Her liver function was impaired moderately: total bilirubin was 43.6 μmol/L [4.7~ 24 μmol/L], direct bilirubin was 26.0 μmol/L [0~ 6.8 μmol/L], and γ-GT went up to 103 IU/L [7~ 64 IU/L]. The results of coagulation test were as follows: APTT 50.1 s [22.3~ 38.7 s], PT 12.9 s [10.0~ 16.0 s], TT 24.80s [14.00~ 21.00s], Fg 1.5 g/L [1.8~ 3.5 g/L], FDP 12.5 mg/L [0~ 5 mg/L] and D-Dimer 2.66 mg/L [< 0.55 mg/L]. They indicated that the patient suffered from coagulation abnormality and hyperfibrinolysis, hence disseminated intravascular coagulation (DIC). After phlebotomy, the transfusion of cryoprecipitate prepared from plasma frozen within 24 Hours (PF24) started immediately. Meanwhile, low-dose heparin (3 U/kg/h) was administered prudently for anticoagulation therapy. 2 days later, bleeding disappeared. On 25th May, urged by the relatives, the patient was transferred back to the local hospital for further treatment. At that time, her skin color didn’t recover to the previous state. Owing to her relatives’ will, we failed to make a follow-up visit. The treatment timeline is shown in Fig. . |
pmc-6032794-1 | We present the case of 33-year-old woman with genetically confirmed osteogenesis imperfecta type I. During childhood, she presented with the pathognomonic features of osteoporosis with multiple fractures and blue sclerae. At the time of consultation in our institution, she had complaints of progressive hearing loss and persist vertigo.
A spiral CT-scan with one millimetre thick sections of the temporal bone was performed. Symmetrical extensive lucencies in the pericochlear bony otic capsules, including the promontories, were demonstrated (Figure ). An additional 3T MRI was performed and included axial FLAIR imaging, axial diffusion-weighted imaging and gadolinium-enhanced 3D fast field echo imaging (3D FFE) through the entire brain. Furthermore, 3D balanced steady-state gradient echo through the skull base completed the exam. The MRI images showed symmetric areas of increased signal intensity in the pericochlear regions on the FLAIR and 3D balanced steady-state images (Figure & ). These areas showed moderate enhancement on the 3D FFE-images after contrast administration (Figure ). |
pmc-6032798-1 | A 42-year-old woman presented at the emergency department with acute onset of right flank pain. The patient had an extensive past medical history of endometriosis for which she underwent several surgeries. Contrast enhanced computed tomography (CT) of the abdomen showed right perinephric hemorrhage and an exophytic hypervascular mass arising from the lower pole of the right kidney with a maximum diameter of 5.5 cm (Fig. ). In the lung bases multiple thin-walled cysts were observed (Fig. ). Though the tumor did not demonstrate the intratumoral fat density typical for angiomyolipoma, the concomitant presence of cystic lung disease in a premenopausal woman was suggestive of spontaneous retroperitoneal hemorrhage from a renal angiomyolipoma in a patient with lymphangioleiomyomatosis. Based on the imaging characteristics hypernephroma could not be ruled out however. Subsequent CT of the thorax, performed several days later, showed multiple thin-walled cysts of various sizes spread throughout the lung parenchyma. The lung apices and lung bases were equally involved. No nodules were present. Based on these imaging findings a definite radiological diagnosis of LAM was made (Fig. ). A brain MRI performed to rule out tuberous sclerosis complex (TSC) was normal. The retroperitoneal hemorrhage was treated conservatively. The patient underwent a follow-up CT abdomen two months later. The perirenal blood had completely disappeared. The renal tumor was unchanged in volume and imaging characteristics. Once again no intratumoral fat could be detected. The patient was transferred to an academic center with expertise in interstitial lung diseases and lung transplantation for further follow-up. Because hypernephroma could not be ruled out based on the CT characteristics and because, even if the lesion was to be a benign angiomyolipoma, the patient was prone to rehemorrhage, a right partial nephrectomy was performed. Pathology confirmed the presence of an angiomyolipoma. |
pmc-6032803-1 | An asymptomatic 62-year-old man presented at the urologist for a check-up with a normal digital rectal examination and a PSA of 1.05 ng/mL. The prostate volume approximated 37 cc on transrectal ultrasound (TRUS) that also showed an hypoechoic structure of 5 cm, extending beyond the prostate, which was interpreted as an utricular cyst. The multiparametric prostate Magnetic Resonance Imaging (MRI) showed a sharply demarcated structure of 5.8 × 6.5 × 5.2 cm (asterisk, Figure : a, sagittal, and b, transverse T2w TSE) originating in the right transition zone of the midprostate, with posterior bulging and no invasive behavior. Relative to the muscle, the lesion was overall T1 isointense and slightly T2 hyper-intense, though it contained small T2 hyperintense foci (arrowheads, Figure , ). The lesion had restricted diffusion (Figure : a (b1400) and b (ADC)). Dynamic contrast-enhancement showed a homogeneous uptake with a slow first pass and progressive enhancement on second pass (Figure : a, transverse post-contrast T1, and b, time-enhancement curve). A benign stromal tumor was suggested. TRUS-guided biopsy was performed, and the tissue diagnosis was a leiomyoma. Considering the absence of clinical complaints and exclusion of malignancy, clinical and imaging follow-up with MRI at six months was advised. |
pmc-6032804-1 | A 68-year-old man was examined for bitemporal hemianopsia and falling episodes. Computed tomography (CT) revealed a 32 mm spontaneously hyperdense suprasellar mass (Fig. ). No calcification was noted. Complementary preoperative magnetic resonance imaging (MRI) showed a well delineated lesion with a nearly isointense signal on the T1-weighted images and a low signal intensity on the T2-weighted images. T1-weighted images following gadolinium administration demonstrated an inhomogeneous moderate to intense enhancement. Diffusion-weighted images showed a slight hypointensity with apparent diffusion coefficient (ADC) measured at 0.70 × 10−3 mm2/s (Fig. ). The adjacent pituitary gland was estimated as normal. Meningioma was the first diagnostic hypothesis.
A surgical intervention was planned but only subtotal resection was possible because of the lesion attachment to the chiasm and optic nerves. Histological examination provided the diagnosis of granular cell tumor (GCT). The postoperative course was uneventful and the patient’s progress was satisfactory. No evidence of recurrence has been identified after two years of observation. |
pmc-6032805-1 | A 69-year-old woman who has been treated for a colorectal cancer suffered recurrent episodes of acute pancreatitis seven years later. She was initially treated by rectosigmoidectomy, for which further analyses revealed a T2 adenocarcinoma with mucinous differentiation, no distant metastasis, and a N2 nodal stage, leading to a complimentary chemotherapy scheme based on Bevacizumab and FOLFIRI. Follow-up revealed a 3 cm liver metastasis four years later, which required bisegmentectomy (segments VI and VII) with a complete excision of the mass and a new scheme of chemotherapy with Oxaliplatine, Fluorouracil and Folinic acid.
Six years after the initial diagnosis, she was admitted with postprandial epigastralgia and elevated levels of lipase (1502, normal range (NR) 5–60UI/L), liver transaminases (AST 546, normal range 10–40UI/L and ALT 417, NR 4–44UI/L), alkaline phosphatase (284, NR 32–104IU) and direct bilirubin (1, NR 0–0.3 mg/dl). Further workup with Sonography, Computed Tomography and Echoendoscopy showed comprehensive dilatation of the bile ducts that contained a dense sludge, but neither lithiasis nor any other cause of obstruction. Endoscopic retrograde cholangiopancreatography (ERCP) revealed bulging of the duodenal papilla and heterogeneous opacification of the bile ducts. The patient was discharged shortly after aspiration of the sludge, sphincterectomy and normalization of the blood tests. The sampled sludge was later identified as mucus containing neoplastic cells. Two months later, after the patient had been readmitted twice for similar episodes and failure to demonstrate any metastasis, MRI with gadoxetic acid disodium (Primovist®), an MRI contrast agent with hepatocyte affinity and biliary excretion was performed, and eventually revealed a filling defect in the liver, a lesion infiltrating the right intrahepatic biliary duct (IHBD) (Figure ). Its additional properties were restricted diffusion on diffusion-weighted MRI (Figure ). Two weeks later, a new bisegmentectomy (segments V and VIII) with resection of the right IHBD, saving the hilar plate, were performed. Histopathological examination identified the lesion as a liver mucinous adenocarcinoma metastasis invading the lumen of the bile duct, diffusely replacing the biliary epithelium and infiltrating nerves. Unfortunately, the subsequent care was palliative, as the section was invaded on histology. |
pmc-6032808-1 | A 65-year-old man suffering from severe and sporadic hemophilia A developed a large
abdominal mass 15 years before seeking treatment.
The patient’s medical history included among other noteworthy facts
transfusion-related chronic hepatitis C, multiple hemophilic arthropathies, and
cerebral hemorrhage.
Laboratory findings were as follows: partial thromboplastin time, 81 sec (normal
values 25 to 39 sec), and factor VIII assay, 1%, without inhibitors (normal values
50% to 200%).
The treatment of his hemophilia consisted of two injections of 2000 units of
plasmatic factor VIII per week. His hepatitis C was left untreated.
He was referred to our Department of Medical Imaging for the characterization of the
mass. Abdominal computed tomography (CT) and a magnetic resonance imaging (MRI) were
performed to document this abdominal lump. No ultrasound was performed. |
pmc-6032810-1 | A 46-year-old woman presented at our institution in April 2013 with acute pain in the left hip. She had a skiing accident, being hit by another skier. Since then, she experienced severe persistent pain in the left hip. The medical history included few relevant findings, except bilateral recurrent calcific tendinitis of the rotator cuff, treated with corticosteroid injections, physiotherapy and arthroscopic exploration (with debridement of the calcific deposition, bursectomy and decompression of the subacromial space). Clinical examination showed pain and significant tenderness of the trochanteric region. There was a normal, however painful, range of motion.
A plain radiography excluded fractures, but demonstrated the presence of perithrochanteric calcifications (Fig. ). A CT-scan of the pelvis confirmed the absence of fractures, and the presence of a large, well-defined calcification of low density, 18 millimeters in diameter, located anteriorly in the gluteus medius tendon (Fig. ). Acute calcific tendinitis of the gluteus medius tendon was suggested as the cause of the patients’ pain.
Since ultrasound-guided needle lavage is a well-described therapy for hydroxyapatite depositions in the rotator cuff tendons of the shoulder, we proposed this treatment approach for this patients’ calcific tendinits. An ultrasonography was performed to assess the feasibility. The calcification was demonstrated as a hyperechogenic structure in the gluteus medius tendon within reach of a needle (3,1 cm beneath the skin) (Fig. ). Reactive thickening of the overlying bursa was also seen. In consultation with the patient and the orthopedic surgeon, an ultrasound-guided needle lavage and corticosteroid injection was performed. Linisol 2% was used as local anesthetic in the subcutaneous tissue, in the overlying bursa and in the peritendinous tissue around the calcification. This reduced the pain instantaneously. A 21 Gauge long spinal needle was then inserted into the hydroxyapatite deposition. The deposition was rinsed using small syringes of linisol 1%, which were gently and alternatingly injected and aspirated. There was progressive aspiration of a large amount of white crystals into the syringes. Once no more crystals could be aspirated, the region of the calcification and the overlying bursa was infiltrated with 40 milligrams of depoMedrol, dissolved in 4 milliliters of marcaine 0,5%. The patient’s symptoms resolved within a few days following the procedure, and she could quickly resume her daily activities. No follow-up imaging was performed given the good response.
18 months later, in November 2014, the patient returned to the hospital with similar complaints, now in the right hip. The pain had an acute but non-traumatic onset. The orthopedic surgeon clinically suspected bursitis, and requested a magnetic resonance imaging-study (MRI) to confirm this hypothesis and to exclude intra-articular pathology.
The MRI showed extensive edema and infiltration of the soft tissue around the greater trochanter, between the proximal muscle heads of the quadriceps, and along the tensor fasciae latae muscle and around the insertion of the gluteus medius and minimus (Fig. ). A hypo-intense, sharply defined structure with a length of almost 15 millimeters and a thickness of 8 millimeters, was identified in the gluteus medius tendon. There were no abnormalities of the joint. Due to the non-traumatic onset of the pain, a rupture or elongation of the vastus intermedius muscle of the quadriceps was unlikely. Because of the history of acute calcific tendinitis of the gluteus medius tendon on the left side, we interpreted the hypo-intese intratendineous structure as a calcification, with calcific tendinitis with manifest reactive edema as the most probable diagnosis. A radiography of the right hip confirmed a large, well-defined calcification cranial to the greater trochanter (Fig. ).
An ultrasound-guided needle lavage of the calcification with corticosteroid injection was performed, in consultation with the referring orthopedic surgeon and the patient (Fig. ). This procedure was carried out the same way as 18 months earlier on the left side. There was a similar good clinical response to the procedure (i.e. nearly complete alleviation of the pain within days and eventually a complete pain-free state), so no follow-up imaging was performed. |
pmc-6032811-1 | A 52-year-old man, referred to us for coughing with sputum and dyspnea, was hospitalized because of septic shock following bilateral pneumonia. Two months previously, he had been hospitalized for a pancreatic pseudocyst during an acute phase of a head pancreatitis. Computed tomography (CT) at that time showed extensive collection around the liver, with a communication with the pancreas. The endoscopic retrograde cholangiopancreatography (ERCP) revealed a large fistula from the Wirsung to this pseudocyst, and a sphincterotomy was realized with the placement of a 7Fr 5-cm prosthesis. During follow-up, about 3 weeks after the patient’s hospitalization because of pneumonia, a new increase in the biological inflammatory syndrome was noticed.
A control radiography showed a persistent parenchymatous condensation in the middle lobula, as well as a right pleural effusion. A small, unusual aeric crescent-shaped picture was seen under the right section of the diaphragm, suggesting a pneumoperitonea (Figure ).
CT revealed that the size of the pseudocyst had decreased significantly, and a large amount of gas was observed inside the cyst, suggestive of spontaneous fistula. A distal bronchi communicating by a fistulous way to the pseudocyst was visualized (Figure ). Multiples areas of centrilobular nodules with a linear branching (tree-in-bud pattern) in the right inferior lobula and a condensation with air bronchogram in the middle lobula were also noticed (Figure ). Further, the 7Fr 5-cm prosthesis placed in January had fallen in the abdomen. Based on multiple detector computed tomography (MDCT) findings, the diagnosis of pancreaticobronchial fistula was suggested and confirmed by analysis of bronchial expectorations that showed raised lipasis and amylasis.
Conservative treatment by somatostatin was instaured, and the pancreatic duct was stented with a new 10Fr 5-cm stent. The evolution was favorable with improvement on the 1-week follow-up CT scan. |
pmc-6032818-1 | A 40-year-old gravida five, para four woman presented for workup and management of abnormal uterine bleeding. Her past medical history was significant only for hypertension and anemia. On review of her social history, she admitted to drinking six packs of beer on the weekends but denied further substance use. She denied previous treatments for her bleeding including any previous intrauterine device usage.
Ultrasonography revealed a 7 cm fundal fibroid with otherwise normal pelvic anatomy. She was initially offered medical management of her bleeding. She declined any medical treatment and strongly desired definitive surgical treatment. She then underwent a total vaginal hysterectomy with adnexal conservation. Due to the large size of the uterus, a myomectomy was performed to facilitate vaginal removal. Her postoperative hospital course was relatively uncomplicated and she was discharged home on postoperative day three.
On postoperative day ten, she presented to the Emergency Department (ED) for fever, worsening abdominal pain, and new onset of nausea and vomiting. In the Emergency Department, she was tachycardic and tachypneic but afebrile. Her exam was significant for abdominal tenderness to minimal palpation, vaginal cuff erythema, and significant tenderness to palpation of the vaginal cuff. Lab work showed an elevated white blood cell count. She was admitted for management of presumed pelvic infection.
A CT of the abdomen and pelvis was obtained and showed a 6.2 x 9.7 cm pelvic abscess adjacent to the vaginal cuff (Figures and ).
Interventional Radiology placed a drain into the abscess and the patient was started on IV piperacillin/tazobactam. She was transitioned to oral amoxicillin/clavulanate potassium after four days on intravenous antibiotics and her drain was removed on hospital day 5. Vaginal wound cultures remained pending; however, due to continued clinical improvement on the oral antibiotic regimen, she was discharged home on hospital day 5 with a two-week course of amoxicillin/clavulanate potassium.
The patient then returned for her outpatient visit approximately one week later. The results of the vaginal wound cultures revealed a large growth of Actinomyces meyeri. The patient's case was discussed with an Infectious Disease (ID) specialist who recommended an additional two-week course of amoxicillin/clavulanate potassium.
The patient then returned to the ED on postoperative day 25 for pleuritic chest pain with mild cough but denied gynecologic complaints. She reported compliance with the oral amoxicillin/clavulanate potassium regimen. Exam and lab work were unremarkable. A chest X-ray showed left basilar heterogeneous opacities, likely subsegmental atelectasis. A CT angiogram was obtained due to concern for a possible pulmonary embolism (PE). The imaging was negative for a PE; however, it was concerning for possible pneumonia. The patient was discharged home with a five-day course of levofloxacin for treatment of pneumonia.
On postoperative day 27, the patient represented to the ED with worsening shortness of air and chest pain. Again, she reported compliance with her antibiotic prescriptions. Exam and lab work were again unremarkable. A repeat chest X-ray showed a slight progression of right basilar heterogeneous opacities thought to be infectious. Her antibiotic regimen was again discussed with ID specialists and an intravenous antibiotic regimen was felt preferable to an oral antibiotic course. She then completed an outpatient two-week course of IV ampicillin/sulbactam as recommended.
On postoperative day 37, a repeat CT of the abdomen and pelvis showed near complete resolution of the previous pelvic abscess. HIV testing was obtained and returned negative result. She reported significant improvement of her symptoms. She was placed on a six-month course of oral amoxicillin per ID recommendations with plans for continued follow-up in their clinic, as well as with gynecology. She has not shown any signs of recurrent infection after approximately 1 year of follow-up. |
pmc-6032954-1 | A 52-year-old male patient presented with the complaint of sudden vision loss in his left eye 3 days earlier. Past medical history was significant for chronic kidney disease, secondary hypertension, chronic hepatitis C virus infection and arrhythmia. Ophthalmologic examination revealed best corrected visual acuity of 10/10 in the right eye and 4/10 in the left eye from the temporal field. Confrontation test revealed inferonasal visual field loss in the left eye. Direct and indirect light reflexes were normal in both eyes and there was no relative afferent pupillary defect. Anterior segment examination was normal and intraocular pressure was 13 mmHg in both eyes. Dilated fundus exam demonstrated soft exudates consistent with hypertensive retinopathy in the right eye. Fundoscopy of the left eye revealed an area of pallor in the superotemporal quadrant and the macula with macular cherry red spot, which were consistent with occlusion of the superotemporal branch of the left retinal artery (). On OCT, peripapillary retinal nerve fiber layer (RNFL) thickness was within normal limits (). In the patient’s visual field, there was an inferonasal defect in the left eye corresponding to the occluded region (). The patient was treated with a single dose of 500 cc intravenous dextran-40 and 200 mg intravenous pentoxifylline. In etiologic studies, Doppler ultrasonography revealed an atherosclerotic stenosis in the right and left main carotid arteries and a calcified plaque causing luminal narrowing in the left internal carotid artery. Transthoracic echocardiography revealed second- to third-degree aortic valve regurgitation and first-degree tricuspid valve regurgitation. There was no improvement in visual acuity or visual field despite treatment. At follow-up 7 months later, OCT showed thinning of the superior, inferior and temporal peripapillary RNFL (). On the thickness map, ganglion cell layer was thinner in the superior and temporal areas (). Decreased vascular density in the superficial and deep capillary plexus consistent with ischemia in the regions supplied by the superotemporal branch of the retinal artery was observed in a 6x6 mm macular field on OCTA (). The borders of the ischemic area were more clearly seen in en face images (). In optic disc OCTA, capillary density was reduced in the superotemporal region and collateral vessels were present in the area (). When compared to the fellow eye, there was a decrease in the macular deep and superficial capillary density in the superior and temporal quadrants () and a decrease in peripapillary capillary density in the superior quadrant (). Visual field loss persisted in post-treatment threshold perimetry (). |
pmc-6032957-1 | A 65-year-old female presented to our tertiary eye centre with 6 weeks’ history of painless right eye vision distortion and no history of eye injury or trauma. On examination, VA was 6/24 in the right eye and 6/5 in the left eye. Following slit-lamp biomicroscopy and fundoscopy a diagnosis of right eye full-thickness macular hole was made and optical coherence tomography showed right eye cuff of subretinal fluid, left eye epiretinal membrane and posterior vitreous detachment. Ten years before presentation she had uneventful bilateral phacoemulsification and intraocular lens implantation with no other significant past ocular or medical history.
Eight weeks later, she underwent right eye pars plana vitrectomy, internal limiting membrane peeling and cryotherapy with C3F8 12% gas tamponade. Two weeks postoperatively, she exhibited a flat retina, closed macular hole and her VA had improved to 6/18 with normal intraocular pressure (IOP). Unfortunately, 7 weeks postoperatively, she developed right eye macula-on RD due to proliferative vitreoretinopathy (PVR) in the inferior retina. RD repair was done within 3 days with silicone oil (Densiron 68) tamponade and retinectomy to release the PVR. After 4 months, VA of the right eye after removal of silicone oil was 6/12 with flat retina and closed macular hole.
Four months later, her VA declined to 6/36 in the right eye and remained 6/5 in the left eye and fundus fluorescein angiogram confirmed severe right eye CMO. She underwent right eye posterior sub-Tenon’s triamcinolone injection and was started on ketorolac trometamol eye drops (Acular) 3 times/day and oral acetazolamide 250 mg slow-release 2 times/day. Treatment of the CMO during the follow-up period is summarised in Table 1. She received 3 posterior sub-Tenon’s triamcinolone injections and 2 intravitreal triamcinolone injections within 14 months with no complications. The CMO initially responded to each triamcinolone injection but later recurred ().
The patient then received 4 intravitreal dexamethasone 0.7-mg implants (Ozurdex; Allergan, Inc.) uneventfully within 15 months, which maintained a dry fovea for a longer period but the CMO recurred again (). She also received a trial of anti-vascular endothelial growth factor (Avastin) but there was no response. At that point, the patient decided that she no longer wanted repeated injections and decided to wait until her fund application to receive ILUVIEN implant as special case was approved.
Her refractory CMO persisted after 2 years without treatment. Finally, she received ILUVIEN intravitreal implant. In the first week she developed mild right eye anterior uveitis; IOP was 27 mmHg in the right eye and 18 mmHg in the left eye. These markedly regressed within a week on dexamethasone drops and latanoprost drops and topical medications were stopped within 4 weeks. At the time of this report, it is 20 months since receiving the ILUVIEN implant and she still has a dry fovea with right eye VA of 6/18 (). |
pmc-6032958-1 | A 21-year-old male patient presented to our clinic with pain and redness in his right eye. On physical examination, visual acuity using Snellen chart was 20/20 in both eyes and intraocular pressures were 14 and 15 mmHg in the right and left eyes, respectively. On slit-lamp biomicroscopic examination, minimally inflamed pinguecula was noted on the nasal conjunctiva of the right eye. No pathology was observed in the left eye except pinguecula (). Fundus examination revealed no pathology in either eye. The patient reported no disease or drug use in his systemic medical history. Treatment was initiated with ophthalmic nepafenac (Nevanac 0.1%, Alcon) four times daily and the patient was scheduled for follow-up one week later. The next day, the patient returned to the outpatient clinic due to redness and itching on his body. He stated that an itchy rash had formed on his trunk and arms the previous day, approximately 1-2 hours after instilling the nepafenac eye drop and he had been treated for allergy that night in the emergency department. A similar reaction had occurred 1-2 hours after instilling the drop that morning and the dermatology department was consulted. Erythematous, edematous plaque lesions were observed on the arms, neck and abdomen on dermatologic examination and the patient was diagnosed with allergic urticaria by the dermatologist (). The dermatologist instructed the patient to discontinue the nepafenac drops and prescribed oral antihistamines to treat the urticaria. The ophthalmology department recommended preservative-free lubricating drops and scheduled the patient for follow-up. At follow-up three days later, the patient’s skin lesions and symptoms had completely regressed and his ocular complaints had also improved. |
pmc-6032963-1 | A 42-year-old male refugee under follow-up for PMD had an uncorrected visual acuity (UCVA) in the right eye of counting fingers from 4 m and best corrected visual acuity (BCVA) of 2/10 with refraction values of -5.00, -12.00 α 35, topographic astigmatism (TA) of 21.2 dioptri (D) α 95. In the left eye, UCVA was counting fingers from 2 m, BCVA was 1/10 with refraction of -6.00, -14.00 α 45 and TA of 23.8 D α 93.5 (, ). Bilateral CLWR was planned for both eyes due to insufficient visual improvement with spectacles and contact lens incompatibility.
The borders of the area to be excised were mapped onto the cornea preoperatively under the biomicroscope light using a 27-gauge needle. Under general anesthesia, a crescent blade was used to make a crescent-shaped incision in the cornea including the area of thinning between 4-8 o’clock, 1-2 mm from the limbus. Stromal dissection from the incision to just above the Descemet’s membrane was done and the thinned corneal stroma was resected using a crescent blade and scissors. After ensuring the Descemet’s membrane was intact, the upper and lower normal-thickness corneal tissue was reapposed using five 10/0 sutures, followed by paracentesis through the limbus to reduce intraocular pressure. The five previously placed sutures were knotted and eight 10/0 polypropylene sutures were added. Topical antibiotic (0.5% moxifloxacin, 4 times daily), topical corticosteroid (1% prednisolone acetate, 4 times daily) and artificial tear drops were prescribed postoperatively. Topography was performed at each postoperative visit. Loose sutures were replaced. The same surgical procedure was performed in the right eye 3 months after the left eye.
On postoperative day 15, UCVA was 5/10, BCVA of 7/10 with refraction of +1,00, -4.50 α 55 and TA was 15.3 D α 167 in the right eye and UCVA was 6/10, BCVA was 9/10 with refraction of +2.00, -4.00 α 70 and TA was 9.4 D α 10 in the left eye ().
Slit-lamp examination at postoperative 5 months revealed a single loose suture at 5 o’clock on the resection line in the left eye, a 1x2 mm area of stromal infiltrate, mild edema surrounding the infiltrate and inflammatory reaction in the anterior chamber (+2 cells) (). After taking samples for direct microscopy and culture, treatment with topical fortified vancomycin 50 mg/mL 8 times daily, ceftazidime 50 mg/mL 8 times daily and 2% fluconazole 6 times daily was initiated. Direct microscopy of corneal scraping showed yeast and culture produced Candida parapsilosis. Antibiogram results indicated sensitivity to fluconazole, voriconazole and amphotericin B. Based on these findings, the fortified vancomycin and ceftazidime were discontinued and treatment was continued with 2% fluconazole drops hourly and oral fluconazole 200 mg daily. Initial response to this therapy was good. However, after 5 weeks the patient exhibited enlargement of the lesion, extensive keratic precipitates throughout the cornea and hypopyon in the anterior chamber. UCVA was 2/10 and fundus examination and ultrasonography revealed no signs of endophthalmitis. Suspecting resistance to the antifungal therapy, the agent was changed to topical 0.15% amphotericin B (amph B) hourly. After taking a sample from the anterior chamber under local anesthesia, 3 injections of 7.5 µg/0.1 mL amph B were administered at 72-hour intervals. Four days after the procedure, the hypopyon disappeared, the anterior chamber reaction regressed and the lesion was diminished in size. However, after the third injection, the patient developed hyphema nearly filling the anterior chamber. The hyphema regressed on day 7, revealing lens opacification and posterior synechia at 5 o’clock, just opposite the incision (). During follow-up, the patient experienced three infectious episodes with hypopyon at intervals of five to seven weeks after discontinuing antifungal therapy. Infection was controlled by resuming antifungal therapy. Lensectomy and synechotomy were performed without intraocular lens implantation while the patient continued antifungal therapy due to cataract progression and recurrence of the infection after treatment was discontinued (). At the end of the procedure, 7.5 µg/0.1 mL amph B was administered to the anterior chamber and topical antifungal therapy was continued for another month. Four months after cataract surgery, an intraocular lens was implanted in the sulcus in a second procedure (). Two months later, corneal stability was achieved in the left eye by performing corneal cross-linking therapy ().
There were no intraoperative or early postoperative complications in the right eye ().
At 23 months after the first operation, the right eye had BCVA of 8/10 with refraction of -1.00, +2.00 α 135 and TA of 6.1 D α 104, while the left eye had BCVA of 9/10 with refraction of +1.50, -4.00 α 65 and TA of 1.4 D α 50 (). |
pmc-6032967-1 | A 61-year-old male had been aware of a right parotid mass for about 10 years; however, he did not seek treatment as the mass was painless. On experiencing serious right parotid pain, he visited our affiliated hospital. Physical examination revealed a painful mass in his right parotid gland of approximately 30 mm in diameter and ipsilateral facial nerve palsy of House–Brackmann (HB) grade III. Laboratory findings showed a leukocyte count of 12,190/μL and C-reactive protein (CRP) of 0.18 mg/dL. Computed tomography (CT) revealed an enhanced irregularly shaped mass in the right parotid gland (). T1-weighted SE MR imaging of the mass showed lower intensity than that of the native parotid tissue (). T2-weighted SE MR imaging also showed intermediate signal intensity and partial hyperintensity (). At this stage, a malignant neoplasm of the parotid gland was suspected.
Nine days after the appearance of symptoms, he was referred to our hospital. On physical examination, the mass was found to be still present but the pain had eased. In addition, the facial nerve palsy showed some improvement to HB grade II. Ultrasound examination revealed an inhomogeneous, lobulated mass (approximately 30 × 25 mm) in the right parotid gland. CT showed that the tumor had become smaller than at the time of the previous scan in our affiliated hospital (). We tried ultrasound-guided fine needle aspiration cytology (FNAC) twice, with an initial finding of necrotic material and a subsequent finding of a large number of histiocytes and acinar cells and a small number of eosinophilic cells with no atypical findings observed. However, no definitive diagnosis was provided.
We suspected a malignant tumor because of the associated facial nerve paralysis and parotid pain. On the contrary, we considered the possibility of a benign tumor with inflammation due to the reduction in tumor size and pain. We planned a total parotidectomy including exeresis and reconstruction of the facial nerve. However, we also made preparations to preserve the facial nerve should a benign tumor be suggested during the surgery.
The patient underwent surgery at 31 days after the appearance of symptoms. A modified Blair incision was made, and a dumbbell-shaped parotid tumor, extending from the superficial to the deep lobe of the parotid gland, was identified. The buccal and marginal mandibular branches of facial nerve were pinched by the tumor (). The nerve branches were in contact with the tumor, but not involved. The temporal and zygomatic branches were present on the tumor capsule. A portion of the tumor was cut off and used for frozen section diagnosis (FSD). It was found to consist of solid and cystic components. The solid component was surrounded by a fibrous capsule, and the wall of the cyst component was lined by a stratified squamous epithelium, which did not show any atypical changes. Based on the above results, we considered the tumor to be definitely benign. We managed to keep the facial nerve away from the tumor by use of microscissors, and the tumor was removed between the zygomatic and buccal branches.
Histopathological examination showed the tumor contained clear cells, which formed a clear boundary between the normal parotid gland tissues (). The Ki-67 labelling index was exceedingly low and the mitotic count was negative. These findings were consistent with oncocytoma of the parotid gland.
Throughout the postoperative period, the nasalis muscle of the affected side was slightly weakened but it was completely recovered at 2 months after surgery. No recurrence was observed during the 18-month follow-up period. |
pmc-6032970-1 | A 17-year-old female student with known SCD (HbSS), on folic acid (FA) since early childhood and on hydroxyurea (HU) 500 mg/day for the past 5 years, was admitted to the medical ward with difficulty in breathing, persistent fever, chest pain, dry cough, and general body malaise for four days prior to admission. Her previous history included a stroke 5 years ago (from which she had made a good recovery) and avascular necrosis of the right hip joint. Prior to this admission, her last transfusion had been 5 years ago.
On physical examination, the patient was febrile (38°C), pale, mildly jaundiced, tachypnoeic, and dyspnoeic with no lower limb edema. Her respiratory rate was 50 breaths/min, pulse rate was 124 bpm, and blood pressure was 124/64 mmHg. Her oxygen saturation (SPO2) was 65% on room air and 100% on oxygen. On chest examination, there were reduced breath sounds in the right base. She had no palpable enlargement of the liver or spleen.
Hemoglobin was 84 g/L, leukocytosis of 19.8 × 109/L, with a predominance of neutrophils (12.4 × 109/L), and platelets of 535 × 109/L (). From previous records, her steady-state hemoglobin was about 70 g/L. Urine culture revealed no bacterial growth after 24 hours; renal function tests and serum electrolytes were within the normal range (). A chest X-ray showed right-sided consolidation, bilateral mid-zone changes, and cardiomegaly. A diagnosis of ACS was made, but pneumonia and septicemia could not be excluded. The patient was placed on oxygen supplementation and was started on IV cefoperazone/sulbactam 2 g·bd, IV normal saline (NS) 3 L/24 hours, and oral ibuprofen; hydroxyurea and folic acid were continued. Pain was managed by syrup morphine 5 mg 4 hourly. Two units of packed red cells were cross-matched and transfused.
After 7 days, the difficulty in breathing worsened, and the patient was transferred to a High Dependency Unit (HDU). Pleurocentesis was performed, and a yellowish frothy fluid was aspirated and taken for culture, biochemistry, and cytology. Culture revealed no bacterial growth after 72 hours, and the biochemistry results were normal (glucose 0.5 mol/l and protein 35 g/L). Cytology showed presence of neutrophils, histiocytes, and lymphocytes. The results of these tests as well as serum adenosine deaminase (22.7 IU/L) and ESR (10 mm/hr) were regarded as not compatible with pulmonary tuberculosis. Viral serology for hepatitis B surface antigen, hepatitis C antibody, and ELISA for HIV I and II were all negative. Oral co-trimoxazole was added to her therapy as a cover for possible atypical pneumonia.
Despite improvement in the right basal consolidation on the repeat chest X-ray, the bilateral mid-zone changes were still present and there was no improvement in her clinical condition. On day 3 in the HDU, the patient's condition deteriorated: her RR was 72/min with PR of 124 b/min and SPO2 ranging between 60 and 66% on room air. The patient was prepared for manual RCE. Prior to exchange transfusion, the hemoglobin was 95 g/L () and HbS quantified by HPLC was 66.4% (), as a result of the recent blood transfusion.
RCE was performed in two steps 20 hours apart. In the first step, a total of 500 ml of the patient's blood was replaced by 250 ml of normal saline (NS) and 250 ml of packed red cells. The patient developed a febrile nonhemolytic transfusion reaction which was managed by IV paracetamol 1 g. After this initial step, the Hb was 98 g/L and HbS was 57.2% (). 12 hours after the RCE, the patient was afebrile, and the SPO2 was 83–88% on room air. In the second step, 450 ml of the patient's blood was exchanged with 250 ml of NS and 200 ml of packed red cells. In total, 950 ml were replaced, corresponding to an estimated 30% of the total blood volume.
Twelve hours after the second RCE, the hemoglobin was 92 g/L and HbS was 48.9%. The patient was afebrile, with oxygen saturation of 97–100% on room air (), RR of 28–30/min and pulse rate of 90 bpm. The patient was weaned off oxygen and 48 hours after exchange, the patient was moved back to the general ward, from where she was discharged three days later. A week later, she attended the outpatient hematology clinic, where she was found to be in good condition, with stable vital signs and a close to normal blood count. In her most recent follow-up (4 months after RCE), she is doing well and has no complaints. She is currently on folic acid 5 mg daily and hydroxyurea 500 mg daily. |
pmc-6032971-1 | A 91-year-old female was admitted to the hospital after suffering a burn to her forehead by a salon hair dryer approximately four months prior to diagnosis. Following the development of the burn, she reported pruritic red patches appearing on the scalp. Over the four months, several clinical diagnoses, including eczema, cellulitis, and hematoma, were rendered by her primary care physician. For her presumed diagnoses, courses of therapy including topical hydrocortisone, moisturizers, and antibiotics were prescribed. While she was compliant with the outlined regimen, her symptoms did not improve. She was then referred to the hospital after she reported mild but persistent bleeding from the lesions. On initial examination, a large purplish mass on the skin of the frontal scalp was identified []. An incisional biopsy was performed, which revealed angiosarcoma [Figures , , and ]. Computerized tomography was ordered for evaluation in case of metastatic disease. Imaging showed frontal scalp swelling with multiple enlarged lymph nodes concerning metastatic disease []. She was not interested in pursuing surgery or chemotherapy but agreed to consider radiation therapy to control the bleeding and be comfortable enough to wear a wig. We agreed with the treatment plan and offered palliative radiation for symptom control. The patient was treated with 4500 centigray (cGy) in 300 cGy daily fractions. She tolerated the treatment with no unanticipated side effects. After the intended course she had flattening of the tumor but with residual bleeding. She passed away at home a few weeks later. |
pmc-6032975-1 | A 73-year-old female with a past medical history of atrial fibrillation and mitral valve prolapse was referred due to intermittent right-sided abdominal pain and a right-sided abdominal bulge. Approximately 10 years ago, she experienced the initial onset of the symptoms which resolved spontaneously. This episode was temporally related to a severe coughing episode. Since then, the same pain and right-sided bulge would recur and then remit spontaneously. She went to an ED for these symptoms in both 2013 and 2015, and in both times, the evaluations, including a CT abdomen, were negative (). A colonoscopy in 2016 was normal. In 2017, the symptoms recurred and prompted yet another ED visit. A CT at this time showed colon interposed between the liver and the abdominal wall along with some mild periappendiceal stranding ().
Because of the long duration of symptoms which appeared to be increasing in frequency and severity and the radiologic findings, it was decided that the best course of action was surgical intervention. She underwent an uneventful laparoscopic right colectomy. During initial laparoscopic exploration, it was noted that the colon was no longer interposed above the liver, and the only abnormal gross finding was a very redundant proximal transverse colon which could easily be maneuvered into the configuration noted on her CT scan. The appendix was grossly normal on laparoscopic evaluation. The patient was discharged on postoperative day 3 with no complications. Incidentally, the final pathology showed a small invasive appendiceal adenocarcinoma arising in the background of goblet cell carcinoid with negative lymph nodes and negative margins staged as T3N0. |
pmc-6033235-1 | A 52-year-old man with a history of hypertension, coronary artery disease and end-stage renal disease under continuous ambulatory PD treatment for 3 years presented to the PD clinic with cloudy dialysate effluent and diffuse abdominal pain lasting 2 h. Two weeks before this presentation, he was diagnosed with community-acquired pneumonia, which was treated with 400-mg oral moxifloxacin daily for 10 days. However, persistent intractable cough did not seem to improve. Two days before this presentation, he visited the emergent department due to epigastric pain and an intractable cough. He had no nausea, vomiting or fever. Physical examination showed epigastric tenderness without guarding and rebound pain. Laboratory studies indicated a white blood cell (WBC) count 12,000 cells/mm3 with 87% neutrophils and 8% lymphocytes, hemoglobin 8.0 g/dL, alanine transaminase 35 U/L, amylase 19 U/L, total bilirubin 0.56 mg/dL, creatinine 16.7 mg/dL, sodium concentration 133 mEq/L and potassium concentration 4.2 mEq/L. Chest radiography indicated no signs of pneumonia. PD dialysate effluent analysis revealed WBC 2/μL without polymorphonuclear leukocyte (PMN). Abdominal ultrasonography only showed a distended gallbladder with sludge. Severe strains of abdominal muscles from persistent and intense cough were impressed. He was discharged with antitussives and analgesics.
At the PD clinic, his vital signs were body temperature 36 °C, heart rate 104 per minute, respiratory rate 19 per minute and blood pressure 121/91 mmHg. Physical examination confirmed diffuse abdominal tenderness with peritoneal irritation and clean exit site of PD catheter. PD dialysate effluent analysis revealed WBC 1783/μL including 50% PMN. He was diagnosed with PD peritonitis and admitted to ward. Cefuroxime and amikacin were administered intraperitoneally empirically. He had diffuse abdominal pain, nausea and vomiting. Daily analysis of PD dialysate effluent showed WBC counts of 1565/μL(PMN 71%) on day 2 and 3755/μL (PMN: 66%) on day 3 and 4805/μL (PMN:86%) on day 4. PD effluent culture was positive for Klebsiella pneumoniae on day 3. Cefuroxime and amikacin were replaced by Cefepime intraperitoneally. Owing to persistent symptoms, we repeated dialysate culture on Day 3 and it grew yeast-like organism on day 6. Amphotericin was administered immediately and surgery to remove the PD catheter was scheduled. On the same day, dark yellow dialysate was obtained (Fig. ). The color became green after a 24-h fast (Fig. ). An enhanced computed tomography (CT) scan of abdomen only revealed a distended gallbladder with modest wall thickening and distended bowel (Fig. ).
Exploratory laparotomy was performed on day 8. Despite a thorough examination of the intestine and stomach, no perforation was identified. The gallbladder was neither reddish nor edematous. A 0.3-cm rupture was found on the fundus of gallbladder. Primary closure of gallbladder was done with external drainage. The PD catheter was removed uneventfully. After antibiotic treatment and intensive care, he recovered without sequelae. He was discharged after 37 days hospitalization and received maintenance hemodialysis three times a week. |
pmc-6033239-1 | A 52-year-old Japanese man with alcoholic liver cirrhosis and a history of previous esophageal varices and hepatic encephalopathy was referred to our hospital. At admission, he was afebrile, icteric, and anemic and complained of abdominal pain due to accumulated ascites. He had none of the neurological symptoms of neuroacanthocytosis []. He was alert, with a blood pressure of 126/66 mmHg, a heart rate of 108/min, a respiratory rate of 20/min, and an SpO2 of 95% (room air). Laboratory data are shown in . He was found to have pleural fluid, ascites associated with liver cirrhosis (Child–Pugh C with 12 points) and hypoalbuminemia, and chronic kidney dysfunction. His indirect bilirubin concentration and reticulocyte counts were increased, and his haptoglobin concentration was decreased, while his vitamin B12 and folate levels were normal. A blood smear showed spur cells rather than fragmented red cells (, ). Assessments of serum lipid concentrations showed markedly reduced triglyceride, HDL cholesterol, LDL cholesterol, lipoprotein (a), phospholipid, apo-AI, and apo-AII concentrations (). Mild splenomegaly was noted. He was slightly diabetic with high serum glycoalbumin (21.5%; reference 11.6–16.4%) and showed coagulopathy, including thrombocytopenia, prolonged APTT, and hypofibrinogenemia, as well as reduced AT-III. Assessments of coagulation factors showed that factor II, V, VII, and IX activities were all reduced, while factor VIII was not. ADAMTS13 activity was within the normal range (). Blood smear preparations after incubating the patient's serum with control RBCs of the same blood type for 24 hours clearly showed formation of spur cells, indicating that this patient's dyslipidemia was responsible for spiculated control RBCs (data not shown). The patient was diagnosed with SCA.
He had been drinking prior to his admission; however, during his stay in the hospital for 4 weeks, he was in a state of complete abstinence. After admission, he was first treated with ascites drainage, RBC transfusions, occasional albumin, and FFP infusions. Thereafter, two courses of plasmapheresis (2,400 mL/course) were performed over 3 weeks' duration.
The patient's reticulocyte counts decreased from 22.4% before to 4.5% after plasmapheresis. He also experienced increases in concentrations of total cholesterol, from 121 mg/dL to 214 mg/dL; phospholipids, from 112 mg/dL to 197 mg/dL; and apo-AI, from 48 mg/dL to 89 mg/dL. However, his lipoprotein (a), LDL cholesterol, and apo-AII concentrations, all of which were low before plasmapheresis, remained unchanged after, and his serum concentrations of indirect bilirubin and LDH did not improve significantly (). Plasma fibrinogen concentrations improved rapidly after each course of plasmapheresis, but gradually declined to subnormal levels. The percentage of spur cells was unchanged or increased slightly. The patient's general condition gradually improved with the disappearance of pleural fluid and ascites, and Hb concentration >8.0 g/dL without RBC transfusions. He was subsequently able to be discharged. |
pmc-6033244-1 | A 30-year-old male, with no significant past medical history, came to the emergency department with complaints of fever and chills for a duration of 3 days. He also complained of myalgia and abdominal pain for the same duration. He reported diffuse abdominal pain, which was 6/10 in severity, nonradiating, and not related to food. He also reported 2 episodes of diarrhea without any blood or mucous, brown in color. He denied cough, shortness of breath, chest pain, night sweats, weight loss, history of trauma, extreme exercise, or any history of travel.
His abdominal examination was significant for abdominal tenderness in all four quadrants, but was soft and nondistended and bowel sounds were present. The rest of his examination was unremarkable. Triage vitals were significant for a temperature of 103.3°F, heart rate of 88 beats per minute, respiratory rate of 18 breaths per minute, blood pressure of 109/49, and pulse oximetry of 99% on room air.
Admission laboratory workup revealed leukocyte count of 6.1 × 103 µL (reference range 4.5–11.0 × 103) with 72% neutrophils, hemoglobin of 16.2 g/dL (reference range 13.0–17.0), hematocrit of 48.2% (reference range 39–53), platelet count of 149 × 103 µL (reference range 130–400 × 103), ESR of 4 mm/hr (reference range 0–20), BUN of 34 mg/dL (reference range 8–20), creatinine of 1.8 mg/dL (reference range 0.4–1.3), potassium of 4.7 mmol/L (reference range 3.6–5.1), phosphorous of 3.0 mg/dL (reference range 2.4–4.7), aspartate aminotransferase of 1,146 IU/L (reference range 15–41), alanine aminotransferase of 243 IU/L (reference range 17–63), and creatinine kinase of 39,125 IU/L (reference range 38–398). Urinalysis was significant for large blood, and urine red blood cells were 0–2.
In view of the high-grade fever, we attempted to find an infectious source to explain his history and presentation. Chest X-ray () and CT scan were obtained and showed no signs of pneumonia. Urinalysis and influenza rapid antigen test were performed and did not reveal any abnormalities. CT of the abdomen and pelvis was obtained and did not reveal any focal sources. Stool culture with ova and parasites, blood cultures, urinary toxicology including synthetic THC (K2), HIV, herpes simplex virus, hepatitis panel, Mycoplasma pneumoniae IgM antibody, urine Legionella antigen, QuantiFERON® test for tuberculosis, and TSH were all performed as well. All the test results were negative except immunoglobulin M antibodies for Mycoplasma pneumoniae that were detected by enzyme-linked immunosorbent assay. The antibodies were initially 781 U/mL, which is considered a low positive (reference range 770–950).
Our patient was empirically started on levofloxacin and metronidazole for possible gastroenteritis initially while culture and laboratory results were pending. He was managed for his acute renal failure and rhabdomyolysis with aggressive parenteral hydration. However, he continued to spike fevers during days 1–4 of the admission, but his diarrhea, abdominal pain, and myalgia resolved by day 3. One week later, mycoplasma antibody was recorded again and it was 976 U/ml, which is considered a true positive (reference range 950+). The fact that the antibody titer was trending up was clinically significant for a recent Mycoplasma pneumoniae infection. After mycoplasma was found to be positive, metronidazole was discontinued and he was continued with levofloxacin. His creatinine, liver function tests, and CK trended down to normal reference ranges (), and he was discharged on day 9 with planned outpatient follow-up. |
pmc-6033245-1 | A 49-year-old previously healthy female presented with a 1-week posterior-region knee pain post direct fall from a standing position on her left knee. This was directly followed by knee joint blockage with a limited range of motion of only 30 to 60 degrees of flexion. The patient started taking NSAID and had minimal pain relief. She reported increased pain upon standing from a sitting position and vice versa with associated tingling and numbness at the level of the calf region especially upon standing.
On examination, she showed good lower-limb alignment, no pain was provoked on meniscal and ligament testing, and there was an absence of muscular atrophy. Her range of motion was limited to only 30 to 60 degrees of flexion, and the patient had pain on the active range of motion. She also had pain upon active and passive extension of the knee joint with tingling and numbness over the calf region extending from the knee posteriorly. She was found to have a nonpitting edema posteriorly with moderate anterior joint effusion. On patellar examination, she felt pain originating from the posterior region of her left knee joint. To note, the patient has never had any symptoms related to her knee up until the direct fall from the standing position.
MRI revealed a multiloculated structure arising from the synovium around the cruciate ligaments within the femoral notch extending beyond the joint capsule posteriorly with significant displacement of the popliteal vessels (Figures and ).
It showed evidence of synovial thickening, and on gradient echography, it showed spotty and irregular hyposignals compatible with the presence of hemosiderin. There also was associated soft tissue edema around the above-described lesion. The patient underwent arthroscopic intervention in the left knee under spinal anesthesia. The posterior compartment of the knee was reached arthroscopically through the triangular space formed by the ACL laterally, PCL medially, and the femoral notch superiorly. Total resection of the lesion was done through only anterior knee portals without taking the risk of posterior portals preventing potential neurovascular injury. The pathology report confirmed the presence of PVNS. On 6-month MRI follow-up, the previously described soft tissue enhancement suggestive of residual inflammatory changes noted within the femoral notch along the cruciate ligaments as well as posterior to the distal metaphysis at the site of the previously present lesion has decreased (Figures and ). The synovial fluid had also decreased with no evidence of hemosiderin deposits. There was no evidence of interval appearance of new susceptibility artifacts or blooming effect on the T2 gradient sequence that could correspond to evident recurrence. The compressive effect previously present on the popliteal vessels has completely disappeared with no further tingling sensation and numbness over the calf region. Clinically, patient regained full ROM on flexion extension (0° to 135°) of the knee. The previously reported pain no longer exists, with no blocking upon flexion. Patient underwent physical therapy protocol and showed significant muscle strength and balancing improvement. She was scheduled to 1-year clinical follow-up. |
pmc-6033253-1 | This is a case of a 28-year-old male patient, previously healthy, with no past surgical history. He was transferred to our facility from a peripheral hospital due to multiple fractures and crush injuries sustained after a motor vehicle accident one week prior to presentation.
The patient had a butterfly fracture at the junction of the proximal and middle thirds of the left femur (already treated by ORIF using a long DCS plate), a left olecranon fracture (already treated by ORIF using tension band wiring), a right leg compartment syndrome (already treated by partial fasciotomy), a right calcaneal fracture (treated conservatively using a cast), a nondisplaced maxillary fracture, and a nondisplaced T12 vertebral body fracture.
Doppler sonography done prior to presentation revealed weak flow in the posterior tibial artery and absence of dorsalis pedis flux in the right lower extremity with normal flow in the left lower extremity. The patient was suffering from acute kidney injury and on daily dialysis (creatinine level on presentation was 6.04 mg/dl; normal range in adult males is 0.6–1.2 mg/dl), attributed to myoglobinuria caused by severe rhabdomyolysis (CPK : 115,000 U/L;normal range: 22–198 U/L).
Physical examination at presentation revealed an awake, oriented, and cooperative patient with several skin lacerations in the right lower extremity and mid-lower back (), right leg open incision sites after fasciotomy (), a left thigh lateral incision site, and a left elbow posterior incision site after ORIF. There were signs of right eye infection (blepharatis) and right tibial wound infection suspected by erythema and purulent discharge.
The laboratory tests done upon arrival revealed a C-reactive protein level of 7 mg/dl (normal range: 0-1.0 mg/dl) and a high WBC count 35 × 109/L (normal range: 4.00–11.0 × 109/L), suggesting a possible deep infection. On presentation, he was already receiving amoxicillin and metronidazole started at another facility for an unjustified reason.
On presentation, the patient was suffering from severe right leg pain associated with right foot paresthesia and pain upon passive and active range of motion. He was diagnosed with right lower extremity compartment syndrome necessitating an immediate extension of the right fasciotomies to the level of the ankle. For broader coverage, the patient was placed on imipenem, vancomycin, and nystatin by the infectious disease team awaiting cultures from blood, right eye, and right tibial wound.
Two days after presentation, the tibial wound culture grew Acinetobacter baumanii that was sensitive to imipenem.
Daily hemodialysis sessions and daily wound care was performed. An inferior vena cava filter was placed. A right internal jugular central catheter was also placed to allow adequate perfusion.
Extensive daily dressing changes and debridement were done to his right lower extremity wound. On day 4, no significant improvement was noted despite wound care on antibiotic therapy. The right tibial wound was found to be diffusely necrotic with irreversibility of drainage, and the patient started manifesting secondary systemic complications. He was therefore transferred to the Operating Room (OR) for exploration and a possible amputation at the level of the affected lower limb. A circumferential incision was made at the level of the proximal 1/3 of the right tibia, and muscles explored were found to be fully necrotic and nonviable with purulent secretions suggesting continuing active infection of the deep tissues. The best course of action determined was below the knee amputation, in an attempt to salvage as much of the proximal tissues as possible, giving them the chance to heal and recover while monitoring. Given the necessity and the importance of the procedure, it was performed on the spot in the OR.
The culture of the amputation stump grew extended-spectrum beta-lactamase (ESBL) producing Escherichia coli and Acinetobacter baumannii.
The patient's systemic overall deterioration with severe anemia (Hg = 6.4 g/dl), persistent acute renal failure, and necrosis of the edges of the right amputation stump dictated a transfer of the patient to the OR on day 8 after admission and debridement of the necrotic edges of the right amputation stump (). However, this intervention did not halt the progression of the ongoing myonecrosis at the amputation site.
On day 9 of his hospitalization, the left elbow suture line started showing signs of wound infection and progressively deteriorated showing necrotic tissue (). On day 13, the patient was taken to the OR for evaluation under local anesthesia. Needle puncturing of the arm, forearm, and fingers were performed and did not show the presence of blood. The decision was taken to perform anterior and posterior skin release and bicompartmental fasciotomies of the forearm (). All explored tissues (skin, subcutaneous tissue, and muscles) were found to be necrotic, with no twitching after cauterization stimuli testing. Inspection showed necrotic, tense, and circumferential 3rd degree burnt skin, 10 cm both proximal and distal to the left elbow wound. Examination revealed very weak radial pulse and cyanotic finger tips. The right BKA amputation stump still showed necrotic tissues so debridement till acceptable perfusion was performed. The left thigh wound was explored, and slight debridement was carried out.
After reviewing the facts at hand, due to the development of systemic toxicosis in the context of several infected myonecrotic wounds in nonviable limbs, and with no improvement in renal function, the decision for extensive debridement under general anesthesia was made. On day 14 of presentation, the patient was taken to the OR for reevaluation, exploration, and possible management of his progressively necrotic left thigh incision site. Incision and drainage revealed diffuse pyomyositis extending to the anterior and posterior compartment, which lead to performing an aggressive debridement and excision of necrotic tissues that reached the bone ().
The left upper extremity was reevaluated: due to the extensive necrosis extending from the fingers to the forearm and diffuse pyomyositis reaching up to 8 cm proximal to the elbow, the decision to perform a transhumeral amputation was made.
The right lower extremity amputated necrotic stump was reevaluated, showing diffuse necrosis and pyomyositis, resulting in the extension of the amputation to above the knee.
The lesion located in his lower back at the level of the lumbosacral spine was explored, where a 20 × 30 cm necrotic skin patch was incised. Deep necrotic lower back paraspinal muscles were also debrided. The debridement was stopped since there was a high risk of retroperitoneal breach at the level of the flanks.
The diagnosis of advanced unresponsive necrotizing myositis with poor prognosis and low survival rate was communicated to the family immediately post-operatively. The patient was transferred intubated from the OR to the ICU for adequate monitoring and critical management.
On the night of day 15 after presentation, the patient developed refractory septic shock due to extensive necrotizing myositis and infected wounds by Acinetobacter baumanii. These were complicated shortly after, by asystole that did not respond to full adult advanced cardiac medical and mechanical reanimation for around 45 minutes leading to the patient's death. |
pmc-6033282-1 | A 71-year-old Tunisian woman presented to our emergency department with atraumatic pain in her neck and shoulders, and fever that had evolved over 4 weeks. Her medical history was significant for arterial hypertension and calcium pyrophosphate dihydrate deposition (CPDD) disease managed by non-steroidal anti-inflammatory drugs. She had no medical family history, and she had not undergone any surgical intervention. She also sustained, 6 months ago, an infective endocarditis due to methicillin-resistant Staphylococcus aureus (MRSA) that was successfully managed with medical treatment (an adapted 2-month antibiotherapy). Infective endocarditis was diagnosed by suggestive findings on transesophageal echocardiogram (irregular 10–15 mm vegetations attached to the aortic and mitral valves) and isolation of MRSA on two consecutive blood cultures. Since she had a moderate aortic and mitral regurgitation, no operative treatment was necessary according to our cardiothoracic surgery team. She was given intravenously administered antibiotics using a combination of vancomycin at 30 mg/kg per day for 8 weeks and gentamicin at 3 mg/kg per day for 5 days. No other blood cultures were performed since she was afebrile from the third week of antibiotherapy with a negative C-reactive protein (CRP) at the last week of antibiotherapy (Table ).
At the current presentation, a physical examination revealed a painful and tender swelling over her right SCJ, and the overlying skin was stretched and shiny without any productive sinus. Her rectal temperature was 39 °C. There was a moderate decrease in her right shoulder’s range of motion. Her cardiac auscultation did not reveal any added sounds or other abnormalities. Laboratory investigations showed an erythrocyte sedimentation rate of 107 mm at the end of 1 hour, and a CRP at 222 mg/l.
Computed tomography (CT) scans revealed a destruction of the medial extremities of her two clavicles and bilateral collections in the soft tissues around the SCJs (Figs. and ). Magnetic resonance imaging (MRI) showed an osteolysis of both sternal and clavicular margins of her SCJs, with a subchondral edema and soft tissue collections (larger on the right side; Figs. and ).
Fine-needle aspiration of her right SCJ fluid was performed as well as synovial biopsy. Blood and joint fluid culture were positive for MRSA. Histologic examination was significant for pyogenic SA.
Intravenous antibiotherapy using an association of teicoplanin and ciprofloxacin was administrated for 1 month, followed by orally administered antibiotherapy combining pristinamycin and ciprofloxacin for 2 months.
We noticed a complete resolution of her fever and the swelling without any biological or clinical signs of recurrence at the last follow-up of 12 months. |
pmc-6033296-1 | A 6-year-old boy with no pathological history accidentally fell from the top of an approximately 3 m climbing pole and injured his right extended elbow and wrist joint. Due to pain and deformity in the right elbow and wrist joints, he visited our hospital. Swelling and a dinner fork deformity of the right wrist joint and pronounced swelling of the right elbow joint were observed. No skin damage was observed. No findings of nerve injury or arterial injury were obtained in the right upper limb. Radiography revealed lateral dislocation of the radial head, a fracture of the proximal ulnar metaphysis, and mild bending deformation at the fracture site. In addition, fractures of the distal radius and ulna, as well as dorsal displacement of the distal fragment, were seen (). Thus, the patient was diagnosed with Bado type III Monteggia injury with ipsilateral fracture of the distal radius and ulna.
Manual reduction under nerve block was attempted on the day of injury. However, because it was difficult to maintain the reduction of the radial head, as shown in , open reduction and percutaneous procedures were performed under general anesthesia. A Kirschner wire was inserted, percutaneously, from the olecranon into the ulnar diaphysis. When the Kirschner wire was in place, the dislocation of the radial head immediately showed good reduction. Further, open reduction and fixation of the fractured distal radius and ulna were performed with Kirschner wires (). A long-arm cast was used for external fixation with the elbow in 90° flexion and the forearm in an intermediate position.
Two weeks after surgery, callus formation at the fractured bone was observed. Therefore, the cast was removed, and range of motion (ROM) exercises of the elbow and wrist joints were initiated. Since bone union was achieved at 6 weeks postsurgery, the Kirschner wires were removed. Pain, ROM limitation, and lateral instability were not observed in the elbow or wrist joints at 3 months after surgery. Additionally, plain radiographs taken at the same time showed a radially convex curvature at the proximal portion of the ulna and lateral subluxation of the radial head (). However, a gradual correction in the outward displacement of the radial head was observed during the 3-year follow-up.
Twenty-one years after surgery, the patient returned to our hospital for another disorder. At that time, we obtained informed consent to perform an examination and take radiographs of the previous Monteggia injury. Neither spontaneous pain, pain during exercise, tenderness, nor ROM asymmetry were observed (). The biocompatibility of the radiocapitellar joint was good, and no malunion was found in the distal radius and ulna (). The patient reports that he has been working as a computer programmer and performs weight training as a hobby without limitations. |
pmc-6033302-1 | A 79-year-old man with a distant history of colon cancer treated with surgery and radiation and diffuse large B cell lymphoma presented with asymptomatic right hydroureteronephrosis to the level of the mid-ureter with associated right ureteral wall thickening found on surveillance CT scan for lymphoma (). With cystoscopy, the right ureteral orifice could not be identified because of prior pelvic radiation. Antegrade ureteroscopy facilitated biopsies taken with Piranha forceps (Boston Scientific, Marlborough, MA). Pathology analysis showed small fragments of denuded urothelial mucosa with small submucosal glandular structures composed of cuboidal cells with low nuclear to cytoplasmic ratios, lightly eosinophilic cytoplasm, mild nuclear pleomorphism, and small nucleoli. No mitoses were identified. The glandular structures stained positively for cytokeratin AE1/AE3 and PAX-8 and negative for GATA-3, suggestive of nephrogenic adenoma. Repeat biopsies through retrograde ureteroscopy ( and ) showed similar changes with rare small tubular structures within a fibromyxoid stromal background. Repeat immunohistochemical studies showed the small glands to stain positively with PAX8, highlighting rare foci of nephrogenic adenoma of the fibromyxoid type (). The patient elected for long-term management with interval ureteroscopic tumor debulking and ureteral stents. Retrograde ureteroscopy facilitated effective tumor debulking, which was achieved with five grasps of a 1.9 French Zero Tip Nitinol basket (Boston Scientific). |
pmc-6033302-2 | A 59-year-old male with no nephrolithiasis history underwent right ureteroscopy and laser lithotripsy for a right mid-ureteral stone. The procedure was complicated by a urinoma managed with an indwelling ureteral stent and retroperitoneal drain placement. He ultimately developed a mid-ureteral stricture. MAG-3 lasix renogram showed 50% split function. He then underwent effective robotic right ureteroureterostomy. Pathology analysis revealed scattered minute tubular structures within a fibromyxoid stroma and immunohistochemistry was positive for PAX8, consistent with fibromyxoid nephrogenic adenoma. |
pmc-6033610-1 | A 14-day-old baby girl was referred to our surgical team due to ongoing nasogastric bilious aspirates. Patient was born at 31 weeks of gestation in a poor general condition, initially required intubation with high frequency oscillatory ventilation, inotropic support, and surfactant administration. She had an antenatal diagnosis of left atrial isomerism, dextrocardia, and a right-sided stomach query situs inversus, which were confirmed postnatal. Enteral feeds were started on day 2 of life with maternal expressed breast milk via a nasogastric tube (NG) but had difficulties in reaching full feeds. She passed meconium on day 3 of life.
On physical examination, no gross phenotypic anomalies were noted, abdominal exam was unremarkable. Plain abdominal X-ray showed a normal gas pattern in consistent with situs inversus. An upper gastrointestinal contrast study was suggestive of intestinal malrotation: duodenojejunal flexure was demonstrated to the left of the midline with the stomach on the right; the proximal small bowel was on the left side of the abdomen (
).
Baby underwent an emergent laparotomy via a left upper quadrant incision. Exploration of the abdomen revealed a right-sided stomach, adhesion bands between cecum and duodenum, a broad base mesentery, and a PDPV crossing anteriorly at the level of second part of the duodenum. Ladd's bands were divided and the patency of the duodenum was checked by injecting 50 mL of air via the NG. The stomach and duodenum proximal to the PDPV were distended adequately by air; however, the duodenum distal to the aberrant crossing vein remained collapsed signifying the presence of extrinsic compression (
). A decision of duodenoduodenostomy was made and a diamond-shaped anastomosis performed anterior to this aberrant vein using 6/0 polydioxanone interrupted sutures. Air was injected again which passed distally without any hold up, showing resolution of the obstruction.
Patient had an uneventful postoperative course. Enteral feeds were commenced via NG on the second postoperative day and gradually build up to full feeds in 2 weeks. She was feeding well, opening bowels regularly, without any episodes of vomiting, and gaining weight adequately at 3 months and 1 year of age on clinic review. |
pmc-6033610-2 | A 1-month-old baby girl was referred to our surgical team due to recurrent nonbilious vomiting and inability to reach full enteral feeds. Patient was born at 38 weeks of gestation via an elective cesarean section with an antenatal diagnosis of congenital heart block and complex cardiac structural anomalies (left atrial isomerism, atrioventricular septal defect, dysplastic pulmonary valve, pulmonary stenosis, large patent ductus arteriosus, hypertrabeculated left ventricular myocardium). She was hemodynamically stable at birth, and commenced on enteral feeds on day 1 of life. She had an episode of suspected necrotizing enterocolitis on day 3 of life, and was kept nil by mouth, and received a 7-day course of intravenous antibiotics. Enteral feeds were restarted, but she was unable to reach full feeds. A cardiac pacemaker was inserted in the second week of life due to congenital heart block.
An upper gastrointestinal contrast study was performed, which was difficult to interpret in the presence of a large pacemaker in the epigastric region; however, the aberrant position of the duodenojejunal flexure and small bowel on the right side raised suspicion of malrotation.
Exploratory laparotomy was performed at the age of 2 months, being unfit due to cardiac status earlier. Abdominal exploration revealed malrotation, with a narrow mesentery and PDPV. Ladd's bands were divided and 50 mL of air was injected via NG tube. There was no evidence of obstruction or hold up at the level of aberrant crossing vein. Duodenoduodenostomy was not performed due to lack of any evidence of duodenal obstruction at this level.
Postoperative course was complicated by Staphylococcus epidermidis line sepsis/suspected necrotizing enterocolitis and was treated with 10-day course of intravenous antibiotics. Feeds were recommenced once recovered from this illness and gradually increased. Patient was discharged home on full enteral feeds on postoperative day 21. She was tolerating full feeds, adequately gaining weight, with no clinical symptoms of intestinal obstruction at 17 months' follow-up. She is still awaiting further cardiac surgery. |
pmc-6033840-1 | A 77-year-old Japanese man was referred to Kochi Medical School Hospital for the treatment of liver metastases from gastric cancer. The patient’s past medical history revealed that he had undergone laparoscopic total gastrectomy with D1+ regional lymph node dissection, according to Japanese gastric cancer treatment guidelines 30 months prior for early gastric cancer []. The primary gastric cancer located in the upper third of the stomach, measuring 2.2 cm. The final diagnosis was T1N0M0, stage IA according to the 8th International Union Against Cancer (UICC) TNM classification [], and the histological findings showed a well-differentiated adenocarcinoma coexisting with a solid-type poorly differentiated adenocarcinoma that had invaded the submucosal layer to a depth of > 2 mm. There was no lymph node metastasis in 35 dissected lymph nodes, no lymphovenous invasion. Twenty-eight months after the initial operation, abdominal computed tomography (CT) revealed a well-defined mass measuring 4.2 cm in diameter in the spleen, and 18F-2-deoxy-2-fluoro-glucose (FDG) positron emission tomography combined with CT imaging showed intense FDG uptake in the splenic mass, with no evidence of further metastatic lesions in any other organ. Under the clinical diagnosis of a solitary splenic metastasis, the patient underwent open splenectomy.
Histological examination confirmed the diagnosis of a solid-type poorly differentiated adenocarcinoma originating from the previous gastric cancer, and immunohistochemical analysis of the tumor showed no reactivity for human epidermal growth factor receptor 2 (HER2). Therefore, the patient was treated with chemotherapy using S-1 plus oxaliplatin. S-1 was given orally twice daily for the first 2 weeks of a 3-week cycle, at a dosage of 100 mg/day, and the patient received 100 mg/m2 of intravenous oxaliplatin on day 1 of each cycle. However, abdominal CT and magnetic resonance images showed multiple liver metastases 4 months after splenectomy, and was treated with ramucirumab plus paclitaxel as second-line treatment. The patients received ramucirumab 8 mg/kg intravenously on days 1 and 15, plus paclitaxel 80 mg/m2 intravenously on days 1, 8, and 15 of a 28-day cycle. After two courses of systemic treatment, abdominal contrast-enhanced CT revealed progression of the liver metastases.
The patient’s laboratory results, including serum carcinoembryonic antigen and cancer antigen 19–9, were within normal limits. Laboratory findings for markers of the systemic inflammatory response revealed normal total protein (6.6 g/dL; normal range, 6.6–8.1 g/dL), normal white blood cell (6.5 × 103 mm3; normal range, 3.3–8.6 × 103/mm3), neutrophil (3.8 × 104/mm3; normal range, 1.6–5.9 × 104/mm3), and lymphocyte (1.8 × 104/mm3; normal range, 1.1–3.3 × 104/mm3) counts, and slightly elevated C-reactive protein levels (0.7 mg/dL; normal range, < 0.14 mg/dL). Based on these findings, the Glasgow prognostic score (GPS) and peripheral neutrophil to lymphocyte ratio (NLR) were determined to be 0 and 2.1, respectively.
Abdominal contrast-enhanced CT showed multiple well-defined mass lesions located in the bilateral lobe of the liver (Fig. ). As third-line treatment for recurrent gastric cancer, the patient was administered 3 mg/kg, i.v., nivolumab every 2 weeks. After four cycles of systemic treatment with nivolumab, abdominal CT revealed a marked shrinkage of liver metastases, which indicated a partial response according to the Response Evaluation Criteria in Solid Tumors (RECIST 1.1) [], with a 55.0% decrease in liver target lesions compared with baseline (Fig. ). After eight cycles of nivolumab, abdominal CT revealed 82.6% decrease in liver target lesions (Fig. ). After 12 cycles of nivolumab, abdominal CT revealed that all target lesions had disappeared; thus, a complete clinical response was achieved (Fig. ). The patient did not develop any adverse reactions, including immune-reactive adverse events, during the course of treatment. The patient continues to receive nivolumab treatment, and there is no evidence of disease recurrence in the 8-month period since starting nivolumab. |
pmc-6034043-1 | The patient, an 8-year-old girl, came to the Lutheran University of Brazil for a routine dental consultation. Upon clinical examination, it was observed that the patient presented chronology (dentition development) and sequence of eruption compatible with her age; however, the left mandibular first permanent molar was absent. No color, texture, or volume changes on the mucosa were observed; the patient did not experience pain. The panoramic radiograph () showed an alteration on the nonerupted first permanent molar, represented by a radiolucent dentin area at the occlusal surface, more like mesial. Based on both clinical and radiographic aspects, the lesion was diagnosed as PECR.
The treatment was conducted in two parts; first, the crown was surgically exposed by removing the gingival mucosa that covered the unerupted tooth. This procedure allowed access to the occlusal surface of the tooth, which was sound.
After 6 months, during the second phase of the treatment, the first molar was partially erupted (). Clinically, there were no structural or color changes on the crown. After local anesthesia, the site corresponding to the radiolucent area was reached; enamel and dentin that covered the lesion were removed using rotating instruments, until the affected area was reached, where an “empty space” could be felt (). Restoration was made with glass ionomer cement (Vitremer-3M/ ESPE) ().
After a period of 6 months, the restoration was made with resin-based composite; the glass ionomer cement was kept as a lining for the restoration. After a follow-up period of 18 months, there were no reports of pain symptomatology and evolution of the rhizogenesis was also observed ( and ).
The project of this clinical case was submitted for evaluation to the Human Research Ethics Committee and approved under number 1.002.654. |
pmc-6034050-1 | An 8-year-old boy came to the Department of Pediat-ric and Preventive Dentistry in Dr. D Y Patil School of Dentistry, Navi Mumbai, India, with the chief complaint of a boil in the upper right back region enlarging since 6 months. Complete medical and dental history of the parents and the child was taken. The parents disclosed a similar lesion to have occurred 6 months ago in the same region, which had been excised with a scalpel by a general dentist ( to ).
No sutures were given, allowing it to heal by secondary intention. No other relevant medical history surfaced. On clinical examination, the “boil” was a sessile lesion of 1.5 × 0.5 × 1 cm in dimension. It exhibited a reddish hue, was fluctuant, and bled on slight examination with finger. There was no blanching or exudate seen.
Intraoral periapical radiograph showed a radiolu-cency surrounding the developing premolar. There was also constant trauma being inflicted to this area due to grossly carious lower right molars, which impinged the area. Extraction was considered for the same to eradicate the underlying irritant.
The differential diagnosis for the same lesion was pyogenic granuloma, PGCG, peripheral ossifying fibroma, inflammatory fibrous hyperplasia, and peripheral odontogenic fibroma. Excision with a soft tissue diode laser was carried forth. Local anesthesia was administered to ensure minimal bleeding in the region and reduce any discomfort for the child ( to ).
The child’s behavior rating was of Frankel rating 3 (positive). The excision was uneventful. The gingival mass was excised and sent for histopathological consideration. Vitamin E in the form of Evian oil-based capsule was topically applied. The patient’s parents were asked to apply it for the following 3 days twice daily. The patient was recalled the next day and then the next week.
The 7-day follow-up revealed the presence of the premolar erupting and gingiva to be coral pink and unharmed. The excised lesion was analyzed under hematoxylin and eosin stain. Histological report described nodular tumor in the subepithelium separated by fibrous tissue.
The report stated there to be frequent multinucleated giant cells in stroma containing ovoid to spindle-shaped cells. The stroma was elaborately vascularized and contained rare inflammatory cells, such as lymphocytes, plasma cells, and eosinophils along with hemosiderin at the tumor periphery. Bony tissue included was histologically unremarkable. The histological features confirmed it as PGCG.
Postoperative healing was uneventful. The patient was followed up at 1, 3, 6, 12, 15, and 18 months. The premolar surrounded by the excised lesion is seen to erupt as per physiological process. |
pmc-6034051-1 | An 11-year-old male child presented reported to the Department of Orthodontics and Dentofacial Orthopedics with a chief complaint of forwardly placed upper front teeth. Clinical examination showed a convex facial profile due to mandibular retrusion and a mesoprosopic facial pattern. The interlabial gap was 8 mm with a short hypotonic upper lip and everted lower lip (). The intraoral examination revealed that the overjet was 8 mm and the overbite was excessive, but incomplete. The buccal segments were class II on both sides with class II canine relation. The maxillary arch showed spacing in the anterior region with mild rotation of both the second premolars. The mandibular arch also had spacing in the anterior region ().
The cephalometric analysis confirmed a marked class II dental relationship with mandibular retrusion, average growth pattern, normal axial inclination of upper incisors, proclined lower incisors and acute nasolabial angle (). Orthopantomograph revealed that there was no underlying pathology or impacted teeth (). The visual treatment objective (VTO) of the patient was positive and suggested that the patient may be treated with growth modulation (). The mp3 radiograph and the cervical vertebra analysis suggested the acceleration of the curve of pubertal growth spurt and supported the growth modulation treatment plan ().
Correction of skeletal malocclusion first with a removable functional appliance followed by fixed orthodontic treatment to correct the dental malocclusion.
This two-step treatment would have increased the treatment duration. Patient cooperation is also essential for the completion of the treatment. So, this treatment plan was rejected.
Fixed twin block appliance to correct the overjet along with the placement of fixed appliances to align both arches. |
pmc-6034052-1 | An 8-year-old boy was referred to the Pediatric Dental Clinic at the Federal University of Ceará (Fortaleza, Ceará, Brazil) for the evaluation of a gingival enlargement of unknown duration situated in the anterior mandibular region associated with tooth pain. The patient showed normal stature and no other physical abnormalities. History of trauma, infectious diseases, or nutritional disorders was absent. Medical abnormalities or dental alterations were not observed among other members of his family. Intraoral examination revealed right mandibular malformed and hypoplastic teeth, deciduous teeth (inferior central incisors) associated with a localized gingival enlargement covered by a fibrous tissue, and teeth 83, 84, and 85 affected by caries. All other teeth were present and normal. At the first visit, clinical data were collected to establish the treatment plan and imaging exams were analyzed. However, after the return visit, the patient did not attend for dental follow-up.
Panoramic radiograph () showed primary mandibular teeth (81, 82, 83, 84, and 85), tooth 46 and dental germs (43, 44, and 45) with “ghost teeth” appearance due to the presence of extensive pulp chamber demarcated by thin layer of mineralized tissue in which it was not possible to observe a clear definition of enamel and dentin. In addition, it was revealed agenesis of lower incisor teeth (41 and 42) and images suggesting dental caries on teeth 84, 85, and 46.
A CBCT scan revealed: (1) a hypodense area suggestive of a periapical lesion associated with tooth 81, which did not show signs of dental caries (); (2) remarkable difference in the pulp chamber space when comparing deciduous teeth 84 and 74 (); (3) crows of the affected teeth surrounded by large hypodense areas (); (4) presence of GTs associated with unerupted teeth 43 and 45 (). In order to provide pulp chamber volume, it was used the semi-automated segmentation tool of the free access ITK-SNAP 3.4.0® software (Cognitica, Philadelphia, USA). It was observed that the volume of the RO-positive tooth pulp chamber () was approximately 25% greater than the volume of the opposite unaffected tooth (). In addition, RO tooth was of approximately 28% shorter length than the corresponding contralateral tooth ().
Imaging exams also showed an intense radiopacity pattern in the region of the trabecular bone surrounding adjacent RO teeth in comparison with the unaffected con-tralateral region. Thus, a fractal analysis was performed aiming to analyze the mandibular trabecular pattern of both regions (). ImageJ version 1.38x software (US National Institutes of Health, nih-image) was used to perform this analysis. Initially, a region of interest (ROI) of 100 × 100 pixels above the mandibular canal was selected between the mesial roots of the tooth 46 and the unerupted tooth 45. This ROI was similarly selected for both right and left mandibular sides. Then, the procedures for calculating the fractal dimension followed the methodology described by White and Rudolph. The bone surrounding RO teeth had a fractal dimension value of 1,171, differing from the unaffected bone (value of 1.309), which indicates an altered bone microarchitecture in the mandibular region containing the RO teeth. |
pmc-6034056-1 | A 4-year-old female child accompanied by her mother reported to a private clinic with a chief complaint of thumb sucking habit. Detailed history revealed from her mother indicated that the child used to suck her thumb when she felt bored and while sleeping. Her mother tried to stop the habit by applying a bitter neem oil substance over her thumb, which was unsuccessful.
The extraoral examination of the patient showed good facial symmetry and convex profile. The intraoral examination revealed anterior open bite, average-sized tongue, and proclination of maxillary anterior teeth; grade III mobility was seen with maxillary central incisors, and while swallowing, the tongue was placed in between maxillary and mandibular anterior teeth (tongue thrusting habit) (). The intraoral periapical radiograph revealed root resorption along the lateral and apical aspects of maxillary central incisors (). It was diagnosed based on the clinical and radiographic finding, ARR accompanied with thumb sucking, and compensated tongue thrusting habit.
The detailed treatment plan was formulated and explained to the mother and her consent was obtained. Local anesthesia was administered prior to the extraction of maxillary central incisors (LIGNOX 2% A, adrenaline, Lignocaine 1: 80000, Lic No: 557, B. No: LAK2K42, Indoco remedies Ltd). The extracted teeth were cleaned and preserved in saline, and the patient was scheduled after a week for further treatment. Treatment of thumb sucking and tongue thrusting was initiated on the second appointment by counseling the parent and the child regarding the adverse effect of the habits on the developing dentition. Based on the parental esthetic concern, we planned a habit reminder therapy using a customized bluegrass appliance with natural tooth pontics as a functional esthetic space maintainer. This modification justified both parental esthetic concern and habit reminder therapy. |
pmc-6034130-1 | She was 6 years old (BA 3.5 years) and 84.5 cm (−6.7 SDS) at the onset of rhGH (Fig C). Her GV also increased dramatically during the first 2 years on treatment (+14.6 and +8.4 cm/year, respectively; Table ). She reached the 3rd centile in height at age 12.3 years, remaining prepubertal and with BA retarded 1 year, with a 4.9 height-SDS increase after 6.5 years on treatment.
Patients 1 and 2 normalized serum IGFBP-3 after 1 month of rhGH and IGF-I after 6 months. In the youngest sister, IGF-I and IGFBP-3 did not reach reference ranges until 1 year on rhGH, remaining normal up to 4.5 years of therapy. In all patients, there was a 6-month period during the fourth year of therapy when GV, IGF-1, and IGFBP-3 levels decreased (Table and Fig ) due to lack of treatment adherence.
The three siblings exhibited mild hypercholesterolemia (positive paternal family history) before therapy that did not change significantly during treatment. |
pmc-6034243-1 | The patient was a pregnant, 28-year-old female of Swedish ethnicity with no previous medical conditions or familial history, who experienced a lump in the neck during the summer of 2016. Physical examination was normal apart from the neck tumor. She did not exhibit any signs of dysphagia, hoarseness or discomfort. A 30 mm nodule was visible on neck ultrasound, and a first round of cytology was inconclusive (Bethesda I). A second round of cytology was performed, and a diagnosis of papillary thyroid cancer (Bethesda VI) was put forward based on the findings of follicular epithelium with nuclear atypia, nuclear inclusions and nuclear grooves. The tumor cells were positive for CK19 and HBME1, and the cytological Ki-67-index was estimated as 3–5%. Pleomorphic giant cells were not reported. Subsequent ultrasonographical mapping revealed no evident lateral lymph node engagements, and a total thyroidectomy with an associated lymph node dissection of regio VI was performed. The operation was carried out during the 2nd trimester and was uneventful without any postoperative complications.
The thyroid specimen exhibited a weight of 33,1 g. In the right lobe, a 30 × 30 × 25 mm well-defined nodule with firm, white to gray cut surface was visualized during macroscopic grossing. No macroscopic evidence of additional nodules were found in the isthmus or left thyroid lobe. Microscopy revealed a partly encapsulated, infiltrating tumor with a predominant papillary growth pattern, in addition to areas with follicular and solid growth patterns. Within most of the tumor area, the tumor cell nuclei were medium-sized, oval and exhibited a light chromatin, in addition to nuclear pseudo-inclusions and nuclear grooves (Fig. ). A few psammoma bodies were also seen scattered across the tumor area. This histological phenotype is consistent with a conventional papillary thyroid carcinoma (PTC) []. No extrathyroidal extension or foci with angioinvasion were seen. In the solid areas (constituting less than 10% of the tumor), PTC-like nuclear changes were more infrequent, but still present in small subsets of nuclei. Multifocally, areas with tumor necrosis (Fig. ) and elevated mitosis counts (5 mitoses/10 high power fields, but no atypical mitoses) were observed – but since the vast majority of the nuclei carried PTC-associated features (inclusions, grooves) in addition to the fact that the predominant growth pattern was papillary and no blood vessel infiltration was seen, no clear-cut diagnosis of poorly differentiated thyroid carcinoma (PDTC) could be made either by the older 2004 WHO criteria [] or the proposed Turin criteria [] supported by the novel 2017 WHO classification of tumors of endocrine organs []. The observed necrosis was believed to be tumor-related, as it was multifocal and without other degenerative changes usually associated to previous cytology aspiration.
In several foci near the tumor capsule, pleomorphic tumor cells with bizarre features were observed, including scattered giant cells (Fig. and ). Strikingly, many of these giant nuclei exhibited prominent pseudo-inclusions, and a few had smudged chromatin. These cells were intermingled with more normal-sized tumor nuclei. The single, largest area with pleomorphic cells was 6 mm. The mitotic count was 5 mitoses/10 high power fields.
A tumor-free lymph node was found near the thyroid capsule, and an additional six tumor-free lymph nodes were visualized from the central compartment. As of this, no local metastatic disease could be found.
Immunhistochemical analyses were performed, and the tumor cells were uniformly positive for TTF1 (Fig. ), CK19 and Bcl-2. The cells were negative for chromogranin A and calcitonin, thereby excluding medullary thyroid cancer. Approximately 25% of all tumor cells expressed thyroglobulin. The p53 staining was weak and not diffuse, arguing against an underlying TP53 mutation. A positive signal was obtained using the V600E mutation-specific BRAF antibody, arguing in favor for an underlying BRAF V600E missense mutation. There were no differences in the immunohistochemical profile when comparing the pleomorphic areas to the more conventional PTC areas, except for the Ki-67 proliferation labeling index, which was 10% in the conventional areas and up to 30% in the pleomorphic areas (Fig. ).
Although the immunohistochemical profile suggested retained differentiation (TTF1 positivity) and thus supported the diagnosis of PTC-PC [], the focally observed high-proliferative areas with aggravated pleomorphism and bizarre nuclei led to the suspicion of tumor dedifferentiation into a local ATC component, consistent with PTC-SGC []. To better try to distinguish the two entities, a microdissection of formalin-fixated paraffin-embedded material representing the largest pleomorphic areas was performed. Tumor cell content was appreciated as 80%. Tissue was then deparaffinized and genomic DNA was extracted by established methods used in the clinical routine (Maxwell 16 FFPE Plus LEV DNA Purification Kit, Promega, WI, USA). Quality and quantity of genomic DNA was established using Nanodrop technology (NanoDrop Technologies). After DNA extraction, a control section was obtained and showed adequate representation of tumor tissue.
Three different genes were then assayed; BRAF (codon 600), TP53 (exons 4–8) and TERT (promoter hotspot mutations C228T and C250T).
A 116 bp sequence of BRAF including codon 600 (part of exon 15) was amplified and analyzed using a real time PCR technique (Cobas 4800 BRAF V600 Mutation Test Kit, Roche Molecular Systems, NJ, USA). An activating mutation was found at codon V600. The test does not discriminate between V600E, V600 K and V600D.
For TP53 and TERT, bi-directional Sanger sequencing was performed using conventional protocols (Genetic Analyzer 3500, Applied Biosystems, CA, USA). No TP53 mutation in exons 4–8 was found, however, a known TP53 single nucleotide polymorphism (SNP) in exon 4 was observed (c.215 C > G, p. P72R, rs1042522). This SNP has a reported minor allele frequency of 25,5% in the European American population according to the Exome Variant Server (). Moreover, no C228T or C250T TERT promoter mutations were detected.
The case was presented at a multidisciplinary tumor board meeting held weekly at the Karolinska University Hospital. If the conclusive diagnosis would have been PTC-SGC, the patient would have continued with a much more aggressive and urgent treatment which would have required to abort the fetus, however with regards to the histopathology and the molecular profile of the tumor, a diagnosis of PTC-PC was argued for. But given the uncertainty of the histology as well as the high Ki67-labeling index, the patient was referred for magnetic resonance imaging (MRI) of the neck and chest, which was negative for pulmonary involvement, however displayed enlarged left-sided cervical lymph nodes in regio 2. Using ultrasonographic mapping, a fine needle biopsy was performed. The subsequent cytological examination however, could not detect any malignant cells.
Postoperative radioiodine ablation using 5400 MBq was performed six weeks after childbirth, seven months post-operatively. Whole body scintigraphy showed uptake in the neck, but a subsequent SPECT-CT did not show any sign of residual tissue. Serum thyroglobulin during stimulation by recombinant TSH was 2.1 micrograms/L. Basal thyroglobulin was 0.2 micrograms/L, without detectable thyroglobulin antibodies. Today, 16 months after surgery, the woman is well with no biochemical signs of disease. |
pmc-6034265-1 | The first case is of a 65-year-old Irish woman with a background of schizoaffective disorder, which had been stable in recent years, and a medical history of chronic renal failure, type 2 diabetes mellitus, atrial fibrillation, arterial hypertension, previous stroke with a right arm contracture, and aortic stenosis. For her schizoaffective disorder she was on a risperidone depot and escitalopram 20 mg once a day. She was admitted medically in December 2015 to the MMUH with a urinary tract infection, acute renal failure, and deranged international normalized ratio (INR).
The Liaison Psychiatry service was consulted shortly after admission. The family gave a collateral history of low mood in our patient since her brother had become ill 2 months earlier and her dose of antidepressant had been increased a month earlier. On review, she was at her baseline mental state, engaging well in conversation and denying low mood, which was confirmed by the community mental health nurse, to whom the patient was well known. No changes were made to her management.
A week later the neurology service was asked to review the patient due to altered level of consciousness. On examination she presented with waxy flexibility, negativism, new onset increased tone of her left arm, posturing, and catalepsy. Her mobility had deteriorated, with selective speech, mute episodes, and poor oral intake noted by medical staff over the preceding day. The impression was that she was suffering from acute catatonia. An magnet resonance imaging (MRI) of her brain showed no acute changes. Nasogastric (NG) feeding was established to ensure oral intake.
The psychiatry service was again consulted, and acute catatonia was confirmed. She was diagnosed as having schizoaffective disorder with catatonia, as per DSM-5 (Table ). A trial of lorazepam was advised for the treatment of catatonia. The dose was titrated to 3 mg per day. The dose was well tolerated and her mental state improved significantly over the following 2 weeks. She became verbally interactive again and returned to her baseline verbal interaction; her mood was euthymic and tone normalized. She was discharged to her own home at a physical baseline that compared to her pre-admission physical state and had remained so at 6-month follow-up. The likely cause for this episode of catatonia was thought to be her medical deterioration. |
pmc-6034265-2 | The second case is a 75-year-old Irish woman with a psychiatric history of bipolar affective disorder, stable for several years on olanzapine and valproate, enabling her to lead an independent lifestyle. There was no history of cognitive impairment. She suffered from multiple medical conditions including: atrial fibrillation, type 2 diabetes mellitus, obstructive sleep apnea, and a recent mitral valve repair complicated by postoperative delirium.
She was admitted medically to a rural Irish hospital in November 2015 for management of a raised INR. During the admission she developed sudden onset left-sided weakness and altered levels of consciousness, as well as rigidity and one isolated temperature spike. The concern was raised that she may be or might have been suffering from neuroleptic malignant syndrome and her neuroleptics were stopped as a precaution (Table ). She was transferred to the intensive care unit (ICU) in the MMUH in Dublin with a suspicion of neuroleptic malignant syndrome or encephalopathy. Computed tomography (CT) brain imaging was normal at the time. As neuroleptic malignant syndrome was suspected, olanzapine was stopped. However, her creatinine kinase levels were normal as was her body temperature. Hence, neuroleptic malignant syndrome was deemed to be unlikely. An electroencephalogram during admission showed changes suspicious of encephalopathy and MRI imaging showed no acute abnormality. A working diagnosis of metabolic encephalopathy was established but extensive investigations yielded no cause for the encephalopathy.
Due to prolonged altered levels of consciousness and unexplained altered mental state, the Liaison Psychiatry service was consulted in January 2016.
On examination, she responded with a mouthed single word greeting, but made no other attempt at verbal interactions. She inconsistently followed the examiner with her gaze, but stared out of the window for most of the examination. On physical examination she presented with waxy resistance to passive movement and psychomotor retardation. The impression was that these features were most likely related to a catatonic exacerbation of her bipolar affective disorder, in the absence of an organic explanation. She was diagnosed as having bipolar I disorder with catatonia as per DSM-5 (Table ).
Delirium was raised as a differential diagnosis (Table ), but she had been reviewed in September 2015 by the Liaison service, when she was delirious after her valve replacement and her presentation was distinctly different on that occasion.
She was initially treated with intravenously administered lorazepam, but became drowsy, with a significant drop in Glasgow Coma Scale (GCS). As such the treatment was abandoned. Instead, olanzapine was cautiously reintroduced, which led to a significant improvement in her mental state within days. On follow-up review, she was mildly confused but engaged well at interview, and was euthymic with no evidence of thought disorder or movement disturbance. Subsequently she was discharged back to her own home. She was not reviewed at 6-month follow-up as she was living in a rural area and was followed up in her local service.
Of note, in 2017, the same patient was readmitted to the MMUH ICU, from the same peripheral hospital, in a very similar state to the presentation in November 2015. Again her neuroleptics had been stopped when she was acutely unwell and she developed typical traits of acute catatonia. She was trialled on lorazepam, which she did not tolerate and reinstitution of her neuroleptics brought no improvement. The therapy was then escalated to electroconvulsive therapy (ECT), to which she had a dramatic response and significant improvement of her mental state. |
pmc-6034265-3 | The third case is of a 68-year-old Irish woman who presented to the MMUH in April 2016 with acute laryngitis. She had a background of bipolar affective disorder which had been stable for the past 30 years on monotherapy with lithium. There had been a recent history of lithium toxicity secondary to a deterioration of her renal function, which had been managed at her local psychiatric hospital. After the episode, she had been restarted on a low dose of lithium as well as a low dose of valproate.
On presentation to the MMUH she was initially treated jointly by the ear, nose, and throat (ENT) team and medical team and was managed in an ICU environment due to respiratory compromise. She had no oral intake for multiple days. Once stabilized she was transferred to an acute medical ward but an acute onset confusional state with bizarre behavior was noted over a period of 2 days. Due to her psychiatric history the Liaison Psychiatry service was consulted. On review she was severely thought disordered and confused. She was only able to produce a word salad and showed echolalia. She had motor retardation, increased tone, negativism, and posturing on examination. The impression was that she was suffering from acute catatonia. Brain imaging did not reveal acute abnormalities. She was diagnosed as having bipolar I disorder with catatonia as per DSM-5 (Table ).
Advice was given to treat her with paliperidone. Her mental state improved slightly as a result, but she remained severely thought disordered and confused for 2 weeks. Eventually, lithium was cautiously reintroduced under close monitoring of her renal function. The reintroduction of lithium was well tolerated and she improved significantly over a 2-week period. At discharge she was no longer thought disordered, she was well orientated, and back to her fully independent baseline. She continues to live independently to date. |
pmc-6034340-1 | The male patient was the naturally conceived son of a 34-year-old gravida 2, para 0, miscarriage 1 mother. His healthy parents were consanguineous (first-degree cousins, of Algerian origin), without history of hypertension, calcification or cardiomyopathy. Prenatal concerns included a fetal hypertrophic cardiomyopathy (HCM) associated with a hydrops fetalis and a polyhydramnios, treated by one amnioreduction due to poor maternal tolerance. No other abnormality was noted on the prenatal ultrasound. Furthermore, antenatal investigations of fetal cardiomyopathy were negative or normal (enzyme disorder, maternal viral infection, normal fetal karyotype). Preterm birth was medically indicated because of an abnormal fetal heart rate: the newborn was delivered by urgent caesarean section at 29 weeks’ gestation. The newborn was eutrophic (birth weight 1330 g), umbilical cord pH was 7.21; his Apgar scores were 4 at 1 min, 8 at 5 min, 10 at 10 min. Resuscitative and neonatal appropriate care were provided.
At birth, he presented with hydrops fetalis (oedema and mild pericardial effusion); he was not dysmorphic; postnatal echocardiography confirmed the diagnosis of HCM without other heart anomalies and routine x-ray didn’t show bone abnormalities. The patient developed severe hypertension a few hours after birth (mean arterial pressure > 70 mmHg, normal range: 35–40 mmHg). This hypertension was refractory to a triple therapy (propranolol, nicardipine, clonidine). To assess this atypical hypertension, extensive blood and urinary tests (for endocrine disease and inborn error of metabolism) were performed, but yielded negative results. A renal artery Doppler ultrasound was performed and detected extensive calcifications of the renal arteries, the abdominal aorta and its major branches. To further elucidate the extent of the calcifications, a low-dose whole-body computed tomography was performed and detected diffuse calcifications of large- and medium-sized arteries (Fig. ). The brain Magnetic Resonance Imaging was normal at term equivalent age. In view of these findings we retrospectively reviewed the chest x-ray performed at day 1 of life: it is noteworthy that calcifications were already apparent (Fig. ). Prenatal ultrasound images were also retrospectively reviewed by several radiologist experts but no arterial calcifications were detected. These singularities were consistent with GACI: the patient’s genotype was analysed, and a mutation was found in the ENPP1 gene.
The treatment consisted of four courses of bisphosphonates administered by intravenous route on day 40 of life, on day 70 of life, on month 21 of life and at 2 years old: each course consisted of 2 or 3 disodium pamidronate infusions (0.50 mg/kg/infusion) delivered over 1 week. At 20 week-follow-up visit, 2 months after the two initial courses of bisphosphonates, the extent of the arterial calcifications was assessed by a whole body computed tomography and an almost complete resolution of the lesions was demonstrated (Fig. ). Concurrently, from 2 months-old to one-year-old, the baby developed transiently hypophosphatemia (serum phosphate level of 3.1 mg/dL (1.0 mmol/L); normal infant range = 4.6–7.1 mg/dL and 1.5–2.3 mmol/L) with inappropriate urinary phosphate excretion (fractional excretion of phosphate > 5%). All other relevant markers of phosphate homeostasis were within normal range (serum 1,25-(OH)2-vitamin D level, parathyroid hormone level, serum calcium level, serum alkaline phosphatase level). The infant had no bone deformities. The transient hypophosphatemia was corrected with oral phosphate supplementation and no vitamin D supplementation. Concerning cardiovascular outcome, at 2-year-old follow-up, hypertension was still treated with a triple combination of drugs: blood pressure levels were constantly above 140/80 mmHg. However, hypertrophic cardiomyopathy resolved with this treatment. The patient had normal growth and development. He had neither ocular nor cutaneous manifestations of Pseudoxanthoma Elasticum (PXE).
The investigation of a multi-gene panel was performed by high-throughput next-generation sequencing. Genomic DNA was processed with the TruSight One/Nextera hybrid capture method and coding regions with adjacent intronic regions of the two GACI-associated genes ENPP1 and ABCC6 were enriched by PCR (Illumina®). Subsequent analysis was conducted by massive parallel sequencing using the MiSeq Benchtop System (Illumina®). Quality criteria require a minimal coverage of 20× for at least 97% of target regions of the investigated genes: the minimal coverage of 20× achieved for all targeted regions was 98% with an average coverage of 172 sequences per base. For assessment of the variants, the dbSNP, ExAc, HGMD, LOVD and UniProt databases, as well as the prediction programs PredictSNP, PhDSNP, SNAP, SIFT, PolyPhen-1, PolyPhen-2, MutationTaster, SNAP, MAPP and Panther were used. Next-generation sequencing analysis of the patient’s ENPP1 gene revealed a homozygous mutation c.784A > G (p.Ser262Gly). There was no mutation in the ABCC6 gene. The parents consented to undergo genetic testing. The c.784A > G (p.Ser262Gly) mutation was also present, in the heterozygous state, in the consanguineous parents. The mutation c.784A > G (p.Ser262Gly) has so far not been described in the literature and mutation databases: there are no entries for the variation in the 1000 Genomes project and the Exome Aggregation Consortium, which indicates a very low global minor allele frequency (MAF). The prediction programs PredictSNP, PhDSNP, SNAP, SIFT, PolyPhen-1, PolyPhen-2, MutationTaster and SNAP all classify the variation as pathogenic whereas the prediction programs MAPP and Panther classify the variation as benign.
The combination of cardiovascular findings and extensive calcifications on imaging, together with the ENPP1 mutation, confirmed the diagnosis of GACI1 in the proband. |
pmc-6034342-1 | A 16-year-old white girl presented to our academic children’s hospital on postoperative day six with a chief complaint of shortness of breath and pleuritic chest pain. She had recently undergone an uncomplicated elective ACL reconstruction with a bone-patellar tendon-bone autograft and lateral meniscus repair with a single FAST-FIX suture (Smith & Nephew, Inc., Andover, MA, USA). The surgery was performed under a general anesthetic as well as a femoral canal block as per our usual protocol. There were no intraoperative complications. Total tourniquet time was 127 minutes at 250 mmHg. Her postoperative course was uncomplicated. She was discharged later that day as her pain was well controlled and she was cleared by physiotherapy. She was encouraged to be weight-bearing as tolerated while limiting her range-of-motion to 0° to 90° of knee flexion for 6 weeks, given her meniscal repair.
On the day of surgery, she weighed 65.3 kg and her height was 165.7 cm. Her past medical history was significant for acne which was controlled with tetracycline. She was on no other regular medication nor did she take a birth control pill. She was a non-tobacco smoker. She had no family history of anesthetic or hematological issues.
She presented to our Emergency Department on postoperative day six with progressively worsening pleuritic chest pain and shortness of breath. Her vital signs upon presentation were: temperature, 36.8 °C; pulse, 104 beats per minute (bpm); respiration rate, 20/minute; blood pressure, 135/76 mmHg; and O2 saturation of 96% on room air. Baseline laboratory work was drawn (Table ) and a computed tomography (CT)-PE study was performed. This revealed bilateral lower lobe emboli with moderate clot burden and an associated opacity in the left lung base in keeping with pulmonary hemorrhage/infarction (Fig. ). She was subsequently admitted and managed medically with a weight-based dose of Lovenox (enoxaparin) twice daily in consultation with Hematology. She was discharged home 3 days later and would successfully complete 3 months of anticoagulation therapy without any further complications. Anticoagulation treatment was stopped after 3 months as per Hematology.
At her 12-month follow-up, she was asymptomatic and found to have no concerning cardiorespiratory issues. Both an echocardiogram and a pulmonary function test were performed, and found to be within normal limits. Hematology decided not to rescan her in order to assess resolution of the PE given her clinical profile. They have investigated for some treatable causes of spontaneous or acquired thromboembolism (Table ). Results for lupus anticoagulant and anti-phospholipid syndrome as well as for paroxysmal nocturnal hemoglobinuria all returned negative. Admittedly, no further testing was performed to identify causes of inherited thrombophilia (that is, factor V Leiden, prothrombin G20210A, protein C, protein S, and/or antithrombin levels), following a thorough conversation with the family, as this would not have changed treatment pathway irrespective of results.
The hematological team did recommend to our patient that she did not use an estrogen-containing oral contraceptive pill, favoring a progesterone-only oral contraction or a progesterone-containing intrauterine device. From an orthopedic standpoint, she is ambulating independently and cleared for return to sport without any restrictions. |
pmc-6034759-1 | A 66-year-old African-American female was brought to the emergency room (ER) for confusion. Her past medical history is significant for polysubstance abuse (heroin, prescription opioids) with multiple prior emergency room visits for heroin overdose, bacterial endocarditis 30 years ago with remote epidural abscess, cervical cord compression from C3-C6 and myelopathy with residual bilateral upper extremity contractures and lower extremity weakness, hepatitis C and chronic obstructive pulmonary disease. According to the patient’s daughter, she appeared somnolent a day prior to the admission. On the day of admission, she seemed confused with short-term memory loss, unable to recognize the daughter’s face along with significant receptive aphasia, although she was alert and conversing. She was unable to perform the usual activities of daily living. Due to concern for stroke, she was brought to the ER for evaluation. She denied a headache, fever, malaise, night sweats, and loss of weight lately. She denied any chest pain, palpitations, loss of consciousness or seizure-like activity.
In the ER, she was afebrile with oxygen saturation of 100% on 4L of oxygen via nasal cannula, blood pressure was 157/96 mm Hg, heart rate of 92 beats per minute. Physical examination showed a middle-aged lady who was alert, oriented to name and place but not to time, along with mild receptive aphasia. Cranial nerves examination was unremarkable. Motor examination showed decreased bulk in bilateral upper extremities with moderate spasticity, tight contractures of the arms and forearms in flexed posture with some antigravity strength, bilateral lower extremity weakness with left side worse than right. Sensations were intact to light touch and pinprick in all the four extremities. Given the paucity of extremity strength, coordination and gait were difficult to assess. She scored 14 points on the National Institutes of Health Stroke scale assessment. Her initial laboratory work was significant for elevated liver transaminases (aspartate transaminase 1399 U/L (normal range 13-35 U/L) and alanine transaminase 894 U/L (normal range 7-38 U/L), elevated creatine kinase of 1790 U/L and was thought to be secondary to rhabdomyolysis. Serum magnesium and phosphorous were low at 1.5 mg/dL (normal range 1.7 -2.3 mg/dL) and 1.6 mg/dL (normal range 2.7-4.8 mg/dL) respectively. Urine toxicology was positive for cocaine. She had normal prothrombin time of 10.9 seconds (normal range 9.7-13.0 seconds), an international normalizing ratio (INR) of 1.1 (normal range 0.9-1.3) and activated partial thromboplastin time of 24.7 seconds (normal range 23.0-32.4 seconds). Computerized tomography (CT) of the brain showed heterogeneous low attenuation involving the entirety of bilateral cerebellar hemispheres with associated mass effect and partial effacement of the fourth ventricle suggestive of sub-acute infarction with areas of petechial hemorrhage along with encephalomalacia in the right insular and anterior operculum (Figure ).
There was no evidence of obstructive hydrocephalus. Due to the concern for posterior circulation large vessel occlusion, an emergent CT angiogram of the head and neck was performed which ruled out any dissection, significant stenosis or occlusion of intracranial or extracranial cerebral vasculature with areas of non-opacification of superior sagittal sinus and left transverse sinus. Both posterior inferior cerebellar arteries were widely patent (Figure ).
The differential diagnosis included multifocal infarction secondary to cocaine-related vasospasm, posterior reversible encephalopathy syndrome (PRES) and infective endocarditis with septic emboli (given prior history of heroin abuse). She was admitted to the neurointensive care unit for impending obstructive hydrocephalus and was started on hypertonic saline. Neurosurgery was consulted for possible external ventricular drain placement and posterior fossa decompression. She underwent magnetic resonance imaging (MRI) of the brain along with MR venogram which showed infarcts in the bilateral occipital lobes, both hippocampi and bilateral basal ganglia in addition to bilateral cerebellar hemispheres suspicious for an embolic phenomenon (Figure ).
A magnetic resonance venogram ruled out dural venous sinus thrombosis. Cerebral angiography was deferred given normal CT angiography. Transthoracic echocardiogram showed ejection fraction of 59% with no regional wall motion abnormalities. Two sets of blood cultures drawn on the day of admission showed no growth. Her ultra-sensitive C-reactive protein (CRP) was elevated at 10.4 mg/L (reference range <3.1 mg/L) but erythrocyte sedimentation rate (ESR) was normal at 2 mm/hr (reference range 0-20 mm/hr). Telemetry during the course of hospitalization was unremarkable for arrhythmias. Her glycated hemoglobin (HbA1c) was 5.4% (normal range 4.3%-5.6%), low-density lipoprotein (LDL) was 105 mg/dL (normal range 60-129 mg/dL). Acute hepatitis viral panel showed positive hepatitis C virus (HCV) antibody with elevated Hepatitis C virus ribonucleic acid (HCV RNA) by polymerase chain reaction (PCR) 4,838,806 IU/mL. She was diagnosed with cocaine-induced bilateral posterior inferior cerebellar artery and hippocampal infarction with multifocal punctate infarcts in the bilateral posterior cerebral artery and basal ganglia secondary to severe vasoconstriction. PRES was a consideration given the significant involvement of posterior circulation and mild encephalopathy, but she did not have high blood pressure during hospitalization, denied headache, vision issues and no pattern of radiographic vasogenic edema, but rather multifocal infarcts causing mild aphasia and encephalopathy. Due to negative blood cultures, normal echocardiogram and lack of fever and other constitutional symptoms, we ruled out infective endocarditis.
Her mental status improved during hospitalization and she was discharged to a skilled nursing facility after seven days with persisting memory problems and unable to recognize faces. Her modified Rankin scale at the time of discharge was 4, requiring assistance with most of her activities of daily living and unable to walk. She was lost to follow up. |
pmc-6034760-1 | A 35-year-old Caucasian male presented during February 2012 with a three-month history of progressive lower back pain radiating to the left leg. Dorsal spine magnetic resonance imaging (MRI) revealed a mass involving the left ilium, sacrum, and left sacroiliac joint. It was also invading the S1-S2 left neural foramen and superior sciatic notch (Figure ). Biopsy of the mass showed a small round blue cell malignant neoplasm, having a uniform site of morphology with a lobulated growth pattern with some of the cells having limited amounts of amphophilic cytoplasm. There was a strong immune positivity for CD99 but negative for desmin CD 163 and CD68. He was diagnosed with primary localized ES. The patient received neoadjuvant chemotherapy and adjuvant radiation therapy according to the VIVA (vincristine + ifosfamide + doxorubicin + actinomycin D) regimen. He completed radiotherapy to the primary site in August 2012 with concurrent ifosfamide and etoposide. All planned treatment was completed in January 2013. The patient was under close follow-up, and in May of 2014, he presented with multiple lung and two pleural lesions. Biopsy confirmed the lesions to be a relapse of ES with metastasis to lungs (Figures , ). In addition, pleural fluid immunohistochemical stains demonstrated the neoplastic cells to be positive for CD99 and negative for MAK-6, synaptophysin, neuron-specific enolase (NSE), and CD56, consistent with metastatic ES. The patient received five cycles of topotecan and cyclophosphamide. A follow-up computed tomography (CT) of the chest in July 2014, before cycle 3, showed interval decrease in the metastatic lesions, consistent with chemosensitive disease. A positron emission tomography/computed tomography (PET/CT) scan during August 2014, after five cycles of topotecan/cyclophosphamide, showed stable metastatic disease in the form of pulmonary nodules and pleural involvement. Autologous stem cells were collected during a single leukapheresis session before the first high-dose chemotherapy (HDCT). The patient received high-dose chemotherapy in October of 2014 (busulfan, 0.8 mg/kg IV (intravenous) q six hours x 16 doses; melphalan, 140 mg/m2) and received CD34+ cells 4.24 x 10 e6/kg infused as an autologous stem cell rescue. The second round of planned consolidative high-dose chemotherapy was given during July 2015 (etoposide/melphalan regimen - etoposide, IV 400 mg/m2, total three doses (Days 2, 3, 4) and melphalan, IV 100 mg/m2, one dose (on Day 1)) followed by autologous peripheral blood stem cell rescue. During the first cycle of chemotherapy, the patient developed mucositis, neutropenic fever, and diarrhea secondary to Clostridium difficile colitis. After the second cycle of HDCT, the patient developed mucositis and transient elevation of liver function tests but these abnormalities resolved over a few weeks.
HDCT achieved 12 months of progression-free survival (PFS); however, on a subsequent PET scan performed during October 2015, the patient showed disease progression. An increase in the size of the metastatic lesions was noted, along with new areas of involvement of malignancy. The patient subsequently received six cycles of pembrolizumab, an antibody against programmed cell death 1 (PD-1) receptor. Subsequent imaging studies showed a progression of the disease, and the patient subsequently received cyclophosphamide, vincristine, dactinomycin (alternating with ifosfamide), and etoposide during March of 2016. The patient developed significant toxicities, bone marrow suppression, and in the presence of progressive disease during June 2016, he chose hospice care (Figure ). |
pmc-6034761-1 | An 81-year-old male presented for consideration of vertebral augmentation due to diagnosis of stage IV, metastatic prostate adenocarcinoma, and worsening back pain. Lupron therapy was initiated at diagnosis four months prior. Docetaxel treatment was planned for six cycles but was subsequently stopped after the first cycle secondary to side effects. No radiation therapy was previously given. PSA level was 120.73 at diagnosis and 0.6 before radiofrequency ablation.
At the first appointment, the patient reported mild back pain and required a walker but was able to ambulate without difficulty. He did have pain upon palpation of the thoracolumbar junctional level. He did not have any neurologic deficit at presentation. Computed tomography (CT) scans showed 40% compression deformity of T12. Magnetic resonance imaging (MRI) showed pathologic involvement of T12 and L1 and metastatic involvement of the epidural component, resulting in 40% spinal canal stenosis (Figure ). At this time, vertebral augmentation was recommended although it was believed the epidural component would not be addressed and Radiation Oncology would need to be consulted. In a short period of two months, the patient’s condition deteriorated where he was wheelchair bound due to severe pain, not controlled with NSAIDS or opioids. In addition, repeat studies showed further tumor infiltration involving T11, prompting augmentation of T11, in addition to T12 and L1.
The procedure was performed under monitored anesthesia care (MAC) and fluoroscopic guided imaging. Under this image guidance, 10-gauge introducer needles were advanced into the T11, T12, and L1 vertebral levels using a bilateral transpedicular approach (Figure ). A drill and osteotome were used to create cavities at the anterior aspect of the vertebral bodies. Bilateral 17-gauge bipolar radiofrequency probes were advanced into the vertebral cavities and simultaneous application of radiofrequency energy was performed as part of the protocol for volumetric ablation of the vertebral bodies. These were done in serial at T11, T12, and L1 vertebral bodies for approximately 15 minutes for each level. Lastly, methylmethacrylate was injected into the vertebral bodies of T11, T12, and L1 (Figure ) for vertebral stability. No complications occurred during the surgery and the patient was discharged the same day.
The patient reported no pain at the three-week follow-up and he was able to ambulate without assistance and continued to increase daily activities. He also no longer required any pain medication. He continued to be pain-free at the eight-week follow-up and repeat MRI showed stable vertebral changes and complete resolution of epidural disease at the T12 and L1 level (Figure ). At nine-months post-op, the patient still had no pain and returned back to his normal activities. |
pmc-6034762-1 | A 32-year-old pre-menopausal woman presented after a routine pap smear revealed a high-grade squamous intraepithelial lesion (HSIL). Subsequent cold knife cone revealed an infiltrating poorly differentiated squamous cell carcinoma with lymphovascular invasion. A pelvic MRI with and without contrast showed a non-enhancing cervical lesion, 3.5 x 3.2 x 4.6 cm in size. The mass was T1 isointense and T2 heterogeneously intense with evidence of left parametrial extension that was palpable on bimanual exam. MRI and positron emission tomography (PET)/CT revealed no pelvic lymphadenopathy or definitive metastatic disease. She was staged as FIGO IIB and recommended to undergo concurrent chemoradiation, but ultimately was unable to receive chemotherapy due to an atypical mycobacteria pulmonary infection. She received 45 Gray (Gy) in 25 fractions to the PTV, which encompassed the GTV, cervix, parametrium, uterus, upper vagina and pelvic lymph nodes. The external bean treatment was delivered using IMRT on a Tri-Cobalt-60 MRI-guided radiotherapy system (ViewRay MRIdian, Cleveland, Ohio, United States). She was advised to have comfortable bladder filling for treatment. She tolerated external beam radiation with moderate fatigue endorsed toward the end of her treatment course and without gastrointestinal, genitourinary, or integumentary toxicities.
She went on to receive high-dose-rate (HDR) brachytherapy with four intracavitary implants. On the day of the first implant, a Smit sleeve was inserted to facilitate subsequent tandem insertion. An MR-compatible, intra-uterine tandem and two 25 mm ovoids were used for each implant. Ultrasound was utilized intraoperatively to assure proper placement of the applicators. A CT scan was performed for treatment planning which was followed by an MRI study on the MRIdian system for tumor visualization (Figure ). The CT and MRI were then registered and transferred to the MIM (MIM Software Inc., Cleveland, Ohio, United States) for target delineation. After contouring, the structure set was transferred to Oncentra (Elekta, Stockholm, Sweden) for treatment planning. For each implant, a dose of 7 Gy was prescribed to ensure 90% coverage of the HR-CTV. The patient tolerated brachytherapy without acute side effects. As of her last follow-up, 12 months after completion of radiation, she remains without evidence of disease and the only treatment-related side effect is early menopause.
Under an institutional review board (IRB)-approved protocol, each of the patient’s 25 daily setup MRIs were retrospectively contoured to include the GTV, bladder, uterus, and rectum. The daily MRIs were registered with the planning MRI to assess organ motion. GTV motion was assessed via GTV displacements. GTV and organ volumes along with GTV and uterus displacements were determined in order to evaluate the relationship between GTV motion and bladder and rectal volumes. As seen in Figure , the GTV volume dramatically decreased throughout the course of treatment. In parallel, the amount of GTV displacement increased throughout the treatment course and was most apparent in the caudal direction. In this case, the variation of bladder and rectal filling did not correlate strongly with GTV and uterus displacement. |
pmc-6035123-1 | A 64-year-old man underwent LDLT from his daughter in May 2009 for acute fulminant hepatitis B. Both the recipient and donor had prior infection with EBV. The initial immunosuppression consisted of methylprednisolone and tacrolimus, with induction therapy using basiliximab. The trough level of tacrolimus was adjusted within the range of 3–4 ng/ml. Thereafter, he received tacrolimus (3 mg/day) and mycophenolate mofetil (500 mg/day), which kept the graft function in good condition. He had no history of immunological rejection in post-operative course until 65 months following LDLT, when he noted fever, pain in the left epigastrium, and nausea. He underwent computed tomography (CT) as a follow-up just 1 year before the onset of this symptom, but no abnormal findings were found in particular. CT revealed systemic lymphadenopathy, mainly in the abdomen, mediastinum, and bilateral cervical lymph nodes. In the splenic hilum, there was a large lymphadenopathy that compressed the stomach (Fig. ).
Upper gastrointestinal endoscopy revealed that a part of the gastric wall was compressed by the large lymphadenopathy in the splenic hilum on CT. We performed a biopsy from the lesion of the stomach; however, the result was inflammatory mucosa only, and we could not find a definitive diagnosis. Fluorodeoxyglucose positron emission tomography (FDG-PET) also showed systemic uptake corresponding to the area of lymphadenopathy on CT (Fig. ). His EBV viral load in the blood was undetectable. Biopsy from the cervical lymph node showed diffuse distortion of architecture, with hyperplasia of large and pleomorphic atypical lymphoid cells (Fig. ).
Flow cytometry for abnormal B cell populations revealed the following phenotypes: CD20+, CD10+, CD3−, CD56−, CD4−, and CD30−. Antibodies used for immunohistochemistry showed CD20+, CD10+, CD3−, CD5−, CD45+, CD56−, CD79a+, bcl2−, and bcl6+ (weak) (Fig. and Table ). EBV-encoded ribonucleic acid in situ hybridization (EBER-ISH) was negative in the tumor cells (Fig. ). Chromosome analysis demonstrated 47,X,−Y,+X,add(3)(q27),+ 5,del(6)(p23),add(10)(q26). Polymerase chain reaction analysis showed rearrangement of the IgH gene. The histopathological diagnosis was follicular lymphoma (FL) grade 3B, with split signals of BCL6 gene defined by fluorescence in situ hybridization (FISH) (Fig. ). From these findings, he was diagnosed with EBV-negative monomorphic B-cell PTLD.
The clinical course with the progress of serum lactate dehydrogenase and soluble interleukin-2 receptor is shown in Fig. . His immunosuppressive therapy was reduced. His target trough level of tacrolimus was adjusted to ≤ 4 ng/ml at the onset of PTLD, which is the target trough level during the treatment of PTLD at our institution. Therefore, we elected to discontinue mycophenolate mofetil without changing the dose of tacrolimus. Moreover, he received prednisolone, but there was minimal response. Thereafter, he received rituximab therapy; unfortunately, this treatment was slightly effective and the splenic hilar lymph node remained unchanged in size. Subsequently, he received radioimmunotherapy with yttrium-90-ibritumomab tiuxetan (90Y-IT). The agent 90Y-IT contains the anti-CD20 monoclonal antibody ibritumomab covalently bonded to the chelating agent tiuxetan and radiolabeled with 90Y. Three months after the therapy, the systemic lymphadenopathy resolved almost completely, except for the splenic hilar lesion, which seemed to progress uncontrollably and penetrate the stomach (Fig. ).
The patient underwent resection of the pancreatic tail with splenectomy and partial gastrectomy (Fig. ). The splenic hilar lesion was exposed in the lumen of the stomach (Fig. ). It was enlarged and compressed its surroundings, penetrating the stomach (Fig. ). Morphologically, the specimen revealed few large dyskaryotic or multinucleated atypical lymphoid cells infiltrating the stomach wall, pancreas, and splenic artery and vein (Fig. ). The atypical lymphoid cells were suggested to be Hodgkin/Reed–Sternberg cells. Antibodies used for immunohistochemistry showed CD20−, CD3−, CD5−, CD45+, CD56−, and CD79a+ and weak positivities for both CD30 and CD10 (Fig. and Table ). To reveal the relationship between the Hodgkin cells and the underlying monomorphic PTLD, we performed a double labeling detection method that combined FISH for breakpoint in BCL6 and immunohistochemical staining for CD30 on the Hodgkin cells, revealing rearrangement of BCL6 (Fig. ). EBER-ISH showed negativity in the tumor cells (Fig. ). His preoperative EBV viral load in the blood was also undetectable. Therefore, we diagnosed EBV-negative cHL-type PTLD.
Six months after the surgery, surveillance CT showed no recurrence of PTLD; the patient then resumed tacrolimus (3 mg/day).
PTLD is regarded as distinct from hematological malignancies, which develop in non-transplant recipients []. PTLD has various origins; approximately 85% are B cell proliferations, 14% are T cell proliferations, and the remaining 1% are NK cell or plasmacyte proliferations [].
The main risk factors for PTLD are age, EBV status, use of immunosuppressive agents, and the type of organ transplanted []. Most adults with PTLD are EBV-negative, whereas children with PTLD tend to be EBV-positive []. As a patient receives higher doses of immunosuppressive agents, the frequency of developing PTLD increases and the time to onset of PTLD shortens []. In our patient, therapeutic drug monitoring kept the levels of immunosuppressive agents in the appropriate range. There is a difference in the prevalence of PTLD according to the type of organ that is transplanted; the small intestine is the most affected (20%), followed by the lungs at 4–10%, heart at 1–6%, liver and kidneys at 1–3%, and other organs at an average of 10% []. At our institution, we encountered 95 pediatric cases (< 18 years old) and 75 adult cases who underwent LDLT between July 1991 and December 2017. Among these, seven pediatric patients (7%) and two adult cases (3%) developed PTLD.
PTLD has a strong relationship with EBV, particularly in B cell proliferations []. Krasuska-Sławińska et al. reported that EBV has been identified in 90% of B cell-proliferating PTLDs []. In general, the time of development of EBV-negative PTLD occurs later than that of EBV-positive PTLD []. These features were seen in our case. EBV-negative PTLD remains lesser elucidated than EBV-positive PTLD. Among the nine PTLD cases that we have encountered at our institution, all seven pediatric PTLDs were EBV-positive, whereas both adult cases were EBV-negative. In general, the first choice of treatment for PTLD is the reduction of immunosuppression. Rituximab is theoretically effective for CD20-positive PTLD but is only effective in approximately half of these patients []. In our case, considering that this patient was older and that the type of PTLD would be enough response by steroids, we did not initially use rituximab. However, because it was not possible to attain the expected response, we used rituximab. Recently, 90Y-IT has been reported to be effective in PTLD patients who were resistant to rituximab [] as well as in patients with CD20-positive cHL []. In our case, 90Y-IT seemed to be effective for monomorphic PTLD but ineffective for cHL-type PTLD because of the negativity to CD20. Surgical therapy can also be effective and provide the correct diagnosis for localized PTLD, like in our cases []. His target trough of tacrolimus has been adjusted at a low level of ≤4 ng/ml both before and during PTLD []. Moreover, because he did not show recurrence and rejection findings after surgery, there was no need to change the target trough, and thus, he was observed at the same dose of tacrolimus. For data pertaining to adjuvant chemotherapy, we searched PubMed and found no studies that reported on using adjuvant chemotherapy for PTLD after curative surgery. Further cases and studies are needed to reveal the effectiveness of adjuvant chemotherapy for PTLD after surgery.
Our case exhibited various histopathological types of PTLD. It was difficult to determine whether the cHL-type PTLD developed after the remission of monomorphic B cell PTLD; the two types of PTLD might have coexisted from the beginning. There are reports of two pediatric liver transplant patients [–] and one adult patient [] who developed cHL-type PTLD subsequent to another type of PTLD (Table ); all cases developed EBV-positive cHL-type PTLD after EBV-positive polymorphic B-cell PTLD [, , ]. Our case was very interesting due to simultaneous presentation of EBV-negative cHL-type PTLD and EBV-negative monomorphic B-cell PTLD.
In our case, the monomorphic PTLD showed rearrangement of BCL6 by FISH (Fig. ), whereas the cHL-type PTLD showed rearrangement of BCL6 during the double labeling detection method that combined FISH and immunohistochemical staining (Fig. ). From these results, we concluded that these two PTLDs might have a common origin. Several reports have shown that cHL and FL are derived from a shared germinal center B cell clone []. Nakamura et al. reported that FL converted to cHL by proving a rearrangement of BCL2 []. A rearrangement of BCL6 is mainly observed in FL or diffuse large B cell lymphoma and is rarely observed in cHL. With regard to PTLD cases, Poirel et al. reported three cases of monomorphic type and one case of cHL type with rearrangement of BCL6 []. Five percent of patients who developed EBV-related PTLD developed another EBV-related PTLD, but there are no reports that can prove a causal relationship between the two types of PTLD []. To the best of our knowledge, there has been no report of EBV-negative monomorphic B cell PTLD converting to EBV-negative cHL-type PTLD by a rearrangement of BCL6 in the same patient following SOT. |
pmc-6035394-1 | A 35-year-old man of Fulani origin in the Northern region of Cameroon, previously healthy, presented to our surgical ward with severe left submandibular non-fluctuant swelling, which was erythematous and warm to palpation. Furthermore, mild trismus and sublingual edema were noted. Otherwise, physical and neurological examinations were unremarkable. He had a sinus tachycardia of 110 beats/minute, fever 39 °C, and blood pressure 140/85 mmHg. A chest radiograph on admission was normal. He had a 1-month history of a left mandibular second and third molar teeth untreated infection (periodontitis). He was urgently transferred to our operating room (OR) where he underwent surgical drainage of the left submandibular abscess; he was then admitted to our intensive care unit (ICU) while sedated, mechanically ventilated, hemodynamically stable, and febrile (38.7 °C). Laboratory tests were noted for extensive leukocytosis (30,000 cells/ul). Immediately on ICU admission, broad-spectrum parenteral antibiotic therapy (intravenously administered ceftriaxone 1 gm 12 hourly and intravenously administered metronidazole 500 mg 8 hourly) was initiated. The following day, extensive bilateral submandibular abscess collection (left >> right) and multiple cervical lymphadenopathy were shown on an urgently performed superficial ultrasonography scan and he was transferred to our OR again for exploration of deep neck abscess. He was co-managed by the dental surgery team. On the second day of admission, he had extraction of the left mandibular second and third molar teeth by the dental surgeon. A large amount of purulent fluid was drained; 3 days later cultures were positive for mixed aerobic (Gram-positive cocci, commonly streptococci) and anaerobic (Bacteroides species essentially Peptostreptococcus species) bacteria. He remained febrile (39.3 °C) with a white blood cell (WBC) count of 24.5, although hemodynamically stable. An additional chest radiograph and ultrasound scan of the soft tissue neck and chest 2 days later showed minimal collection in the anterior superior mediastina space and significant abscess in both pleural spaces (empyema thoracis), which was far greater in his left hemithorax. An urgent left closed thoracostomy tube drainage (CTTD) was performed and connected to pleurovac and suction. A large amount (approximately 800 cc) of purulent fluid, pH 7.18, was drained from his left pleural space; he was transferred back to the ICU. Bacterial culture samples of serous fluid were positive again for mixed microbial flora as above. He continued intravenously administered antibiotics therapy with ceftriaxone, ciprofloxacin, and metronidazole only due to bacterial susceptibility of isolated growth of polymicrobial flora. Figure is a post-incision and drainage photograph of this patient showing multiple neck wounds and sinuses. Figure is a plain chest radiograph showing the left thoracic empyema. During the next 4 days he went to OR two additional times for surgical debridement, drainage, and washout of neck wounds. He continued the neck wound washout and antibiotic therapy over the next 3 weeks. He was discharged from the unit 4 weeks after admission. |
pmc-6035394-2 | A 32-year-old woman of Fulani origin in the Northern region of Cameroon presented to our surgical ward with a 5-day history of a painful right submandibular swelling with involvement of the right side of her neck and upper anterior chest wall. There was associated right upper quadrant abdominal pain. These symptoms were preceded by 1-week history of right second and third mandibular teeth infection which was left untreated. She was newly diagnosed with retroviral infection but yet to commence highly active antiretroviral treatment (HAART) before admission. She had right submandibular fluctuant and tender swelling, which was warm to palpation. Further physical and neurological examinations were unremarkable. She had tachypnea 32 breaths/minute, tachycardia 140 beats/minute, fever 38.5 °C, and blood pressure 120/70 mmHg. An ultrasound scan of the submandibular and neck swelling showed right submandibular turbid collection with inflamed muscles. A chest radiograph revealed blunting of the right costophrenic angle. Thoracocentesis revealed a pleural fluid analysis of marked leukocytosis 57,000 cells/ul, predominantly Gram-positive cocci.
She was urgently transferred to our OR where she underwent surgical drainage of the right submandibular abscess and right CTTD connected to pleurovac and suction; she was then admitted to our ICU while sedated, ventilated on oxygen by facemask, hemodynamically stable, and febrile (38.7 °C). A laboratory test was notable for extensive leukocytosis (17,000 cells/ul). Immediately on ICU admission broad-spectrum parenteral antibiotic therapy (intravenously administered ceftriaxone 1 gm 12 hourly and intravenously administered metronidazole 500 mg 8 hourly) was initiated. On the following day, she underwent extensive washout of abscess spaces of the submandibular region and neck. The culture of the submandibular abscess isolated viridans streptococci after 72 hours and the bacteria of the isolated growth were susceptible to Augmentin and doxycycline. Figure is a pre-incision and drainage photograph of this patient showing right submandibular abscess. Figure is a plain chest radiograph and computed tomography (CT) slides showing the left thoracic empyema. Her postoperative period was essentially unremarkable with our patient showing significant relief of symptoms. On the fifth day of admission she developed sudden onset of cardiac arrest from which she could not be resuscitated and was certified dead approximately 1 hour later. |
pmc-6035407-1 | We describe the case of a 34-year-old gravida II para l woman, with a gestational age of 26 + 3 weeks at admission, who had a relatively healthy 4-year-old child with her 40-year-old husband of non-consanguineous marriage. She had been on injectable contraception for 2 years and had regular menses for 6 months before the pregnancy. She had antenatal care at a local health center and was vaccinated with tetanus toxoid once and supplemented with iron for 3 months. She was screened for retroviral infection, hepatitis, and syphilis and it was documented nonreactive. She had no anatomic scan at early gestation. She came to Felege Hiwot Referral Hospital with the chief complaint of severe and persistent headache of a day’s duration which was occipital in location associated with blurred vision and generalized body swelling of 1 week’s duration. She had no other danger signs in pregnancy. Her past gynecologic history, medical history, and surgical history were uneventful. She is Amhara by ethnicity. She had no known family history of hereditary or chromosomal disorders.
Her blood pressure at admission was 180/120 mmHg and pulse rate was 84 beats per minute; her respiratory rate was 22 breaths per minute and she was afebrile. She had pink conjunctiva and non icteric sclera, 24 weeks-sized gravid uterus, no abdominal tenderness, no organomegaly, no sign of fluid collection in her abdomen, and the fetal heart beat was positive. She had no vaginal bleeding or discharge. She had pedal and pretibial edema. She was conscious and oriented to person, place, and time. Her deep tendon reflex was +2 and her motor and sensory examinations showed no motor or sensory problems. Other parts of systemic examinations were normal.
Her hypertension was controlled with intravenously administered hydralazine 5 mg two doses at our emergency department. In her complete blood count her white blood cells were 7300 cells/micL, hemoglobin of 13.4 g/dl, and platelet count was 169,000 cells/micL. Urine protein dipstick was +2, and liver and renal function tests were done: serum glutamic pyruvic transaminase (SGPT) 89 IU/L (elevated), serum glutamic oxaloacetic transaminase (SGOT) 102 IU/L (elevated), alkaline phosphatase (ALP) 229 IU/L, and lactate dehydrogenase (LDH) 288 IU/L. Total bilirubin was 0.24 mg/dl, albumin was 3.49 g/dl, blood urea and nitrogen was 12 mg/dl, serum creatinine was 0.69 mg/dl, and oral glucose tolerance test was in the normal range. Obstetric ultrasound showed a singleton, alive, intrauterine pregnancy with average gestational age of 26 weeks, there was a single large ventricle with partially formed midline structure (see Fig. ), amniotic fluid index was 13.4 cm, placenta was located anteriorly at the body of the uterus, and the presentation was breech; the fetus had normal four chambers of heart with normal outflow tract.
After blood pressure was controlled (it took 2 hours), she was admitted with the diagnosis of late second trimester pregnancy and preeclampsia with severity feature plus semilobar HPE. Seizure prophylaxis for preeclampsia was given (magnesium sulfate according to World Health Organization guideline), methyldopa 500 mg orally every 8 hours was added, and she was counselled about options of management; the high incidence of associated anomalies, severe morbidities of survivors, and poor prognosis were discussed. Termination was decided and done with misoprostol 100 microgram every 3 hours at the third dose with outcome of 1.1 kg male, alive neonate. On examination of the neonate, there was cebocephaly, hypotelorism, single patent nostril which enabled nasogastric tube 6F, micropenis (8 mm), and unilateral right hand polydactyly with agenesis of middle phalanges of the fifth finger. There was rigidity involving all extremities which resisted extension and flexion (see Figs. , and ).
After basic neonatal care was given (cord tied, airway cleaned, and newborn dried), he was transferred to our neonatal intensive care unit (NICU) but he died 20 minutes after admission to NICU. Immediate cause of death was not known. Following his death, further investigations were not possible for cultural reasons. At third postpartum day, maternal blood pressure was 130/90 mmHg, pulse rate was 78 beats per minute, and respiratory rate was 20 breaths per minute. Her complete blood count showed white blood cells of 12,000 cells/micL, hemoglobin was 11 g/dl, and platelet count was 122,000 cells/micL. Liver function tests showed SGPT of 35 IU/L, SGOT of 12 IU/L, ALP of 359 IU/L, and LDH of 254 IU/L; total bilirubin was 0.56 mg/dl, blood urea and nitrogen was 22 mg/dl, and serum creatinine was 0.8 mg/dl. After she was counselled to have preconception care and prenatal screening in next pregnancy, she was sent home relatively healthy. She was well at postpartum visits and methyldopa was discontinued at seventh postpartum day. |
pmc-6035426-1 | A 27-year-old non-HIV-infected female was admitted to our hospital because of a headache and the worsening of pre-existing visual disturbance. Her medical history comprised tuberculous meningitis treated with anti-TB drugs (2-month treatment with isoniazid, 300 mg/day; rifampin, 450 mg/day; ethambutol, 1000 mg/day; and pyrazinamide, 1500 mg/day, followed by a 10-month treatment with isoniazid and rifampin) with adjunctive corticosteroid 10 years ago. The Mycobacterium tuberculosis culture was susceptible to all anti-TB drugs, and the patient attained bacteriological remission with a sequela of visual impairment (bilateral counting finger) because of hydrocephalus that was treated with the placement of a ventriculoperitoneal (VP) shunt. Although asymptomatic remnant tuberculomas were reported primarily in the bilateral ambient cistern after the completion of the anti-TB treatment (Fig. ), her symptoms remained stable, and she visited a neurosurgical clinic regularly to check the patency of the VP shunt.
Upon admission, the patient was alert and afebrile, and the neurological examination revealed only the worsening of pre-existing visual disturbance from counting finger to light perception without meningeal irritation. The blood analysis revealed a slight elevation in CRP levels (0.37 mg/dL), and the interferon-γ release assay (T-SPOT) was positive. A chest X-ray revealed no abnormal lesions suggestive of pulmonary tuberculosis. In addition, the cerebrospinal fluid (CSF) testing revealed elevated protein levels (570 mg/dL), no pleocytosis (6/μL), and normal glucose levels (60 mg/dL), with a standard opening pressure (130 mmH2O). Notably, antigens of Cryptococcus, Toxoplasma, and Aspergillus in the CSF and the serum antibody against Taenia solium were all negative, and no elevation of soluble interleukin-2 receptor and the angiotensin-converting enzyme was observed in the CSF. Furthermore, a head CT revealed new emerging tuberculomas in the left sylvian fissure, which was accompanied by a low-density area in the left temporal lobe (Fig. ). While a contrast-enhanced axial T1-weighted brain magnetic resonance imaging (MRI) scan revealed tuberculomas with a diffuse hypointense area around the left sylvian fissure, brain fluid-attenuated inversion recovery (FLAIR) imaging indicated a hyperintense area around the tuberculomas in the left sylvian fissure, suggesting edematous changes (Fig. and ). Repeated mycobacterial culture and real-time polymerase chain reaction (PCR) in the CSF did not indicate the existence of the reactivation of CNS tuberculosis. During these 2 weeks of examination, the symptoms did not worsen without specific treatment. Based on these findings, we diagnosed these lesions as late-onset post-treatment PRs. Accordingly, we prescribed the oral administration of corticosteroids (prednisolone, 30 mg/day, 0.6 mg/kg/day), which were gradually tapered off. After 3 months of the corticosteroid treatment, the tuberculomas disappeared, including those previously present in the bilateral ambient cistern, and brain edematous changes around the left sylvian fissure vanished (Fig. and ). Four months after the onset of PRs, we reduced the dosage of prednisolone to 5 mg/day; however, because a tuberculoma reappeared in the same place of the left sylvian fissure, we increased the dosage of oral prednisolone to 20 mg/day. This time, we carefully tapered off prednisolone over 1 year, and the corticosteroid treatment erased the residual tuberculoma again with no recurrence of tuberculous meningitis. The patient’s symptoms remained stable during 1 year of follow-up without additional anti-TB drugs. |
pmc-6035443-1 | Our patient is a 62-year-old male with multiple comorbidities including morbid obesity, coronary artery disease, lipodermatosclerosis of the lower extremities, chronic peripheral venous insufficiency, and prostate cancer (Gleason 4+5) on long-term androgen deprivation therapy. He was previously treated with docetaxel for pelvic lymph node metastases. The patient also had a small renal tumor for which he was followed with imaging. He had a distant history of varicose vein ligation. While undergoing surveillance imaging to evaluate for spread of his prostate cancer, an incidental pulmonary embolism was discovered. He was started on enoxaparin 120 mg by subcutaneous injection twice daily. Within several days the patient noticed several small black blisters on his hands that resolved spontaneously. These were not reported by the patient or observed by any practitioner during routine clinic visits. The timeline of development of hemorrhagic skin lesions of this patient is outlined in Fig. . Four months after starting anticoagulation with enoxaparin, he presented with several large hemorrhagic bullae on his calves (Figs. and ). His coagulation values and his platelet count were within normal range. The diagnosis of bullous hemorrhagic dermatosis (BHD) was made by visual inspection. Therefore, no biopsy was performed. Enoxaparin was discontinued and apixaban was started as alternative anticoagulation. The lesions healed in 3 weeks with intensive outpatient wound care and have not recurred to date while on apixaban. |
pmc-6035448-1 | A 7-year-old boy was referred to our hospital for an elevated BLL (> 60 μg/dL) discovered during routine screening procedures. On admission, a recheck of the BLL, tested by Atomic Absorption Spectrometry (AAS), showed the level to be 91 μg/dL. Thus the diagnosis of lead poisoning was confirmed.
Two months before admission, he started to feel dizzy and developed headaches. Symptoms progressed to poor appetite, mouth-bitterness, repeated vomiting and abdominal pain for more than a month. The abdominal pain was intermittent, without an obvious precipitant and generally lasted for 10 min with spontaneous resolution. Subsequently, intense joint pain and fatigue occurred causing him to be unable to walk by himself.
Before admission, he had been hospitalized twice elsewhere. At his first presentation 2 months earlier, laboratory examinations found elevated serum liver enzymes: alanine transaminase (ALT) 145 U/L and aspartate aminotransferase (AST) 78 U/L. He also had anemia with a hemoglobin (Hb) level of 96 g/L and red blood cell (RBC) count of 3.67 × 1012/L). Superficial gastritis and bile reflux were found by endoscopy. An upper abdominal CT angiography showed “a general decrease in liver density; possible superior mesenteric artery syndrome”. A descriptive diagnosis of “chronic superficial gastritis, possible superior mesenteric artery syndrome, and abnormal liver function tests” was made. He was treated with omeprazole and sucralfate for 2 weeks which was accompanied by relief of his symptoms. He was discharged from the hospital without an identified etiology.
Ten days after discharge, he was admitted to another hospital for intermittent vomiting and severe abdominal pain. Liver function tests, electroencephalogram and abdominal ultrasonography were normal. An incidental BLL test was performed (this hospital tests lead routinely) and reported as elevated. He was referred to our hospital for further evaluation and treatment.
On admission to our hospital, his examination found a soft, non-tender abdomen without rebound tenderness. Both liver and spleen were impalpable. Neurologic examination was normal. Abnormally increased pigmentation of the gums (“lead line”) was observed (Fig. ). No radiopaque point masses were identified on anteroposterior abdominal radiography. Anteroposterior radiograph of the knees (Fig. ) showed increased linear radio-density at the distal femoral and proximal tibial and fibular metaphyses. Serum chemistries were normal. The RBC count was 3.68 × 1012/L (reference range 4.0–5.5 × 1012/L) and the Hb level was 101 g/L (reference range 120-150 g/L), consistent with anemia.
A more detailed history about sources of lead exposure was taken. He had been using a red-colored folk medicine (Fig. ), spraying it in the nostrils two to four times a day during the week prior to consulting a doctor. The nasal spray liquid was obtained from a traditional Chinese medicine (TCM) practitioner (Shenzhen, Guangdong, China). Analysis of the liquid revealed a lead concentration of 148,000 mg/kg (14.8%) as measured by ICP-MS.
Chelation therapy combined orally administered dimercaptosuccinic acid (DMSA) (350 mg/m2 body surface area, q8h) and intravenous calcium disodium edetate (CaNa2EDTA) (1 g/m2 body surface area, continuous infusion using a micro-pump over 24 h) for 5 days. Glutathione (600 mg, intravenous drip, once a day) was added as an antioxidant. All the symptoms resolved; and the BLL declined to 21 μg/dL after chelation therapy was completed. |
pmc-6035448-2 | An 8-year-old girl with rhinitis had consulted the same TCM practitioner as in case 1 and was prescribed the same nasal spray liquid to be used twice a day for 10 days. She developed severe abdominal colic, vomiting, constipation and felt fatigued. Her venous BLL was 91 μg/dL. The nasal spray contained 223,000 mg/kg (22.3%) lead. Abnormal laboratory test results included: creatine kinase 747 U/L (reference range 30–135 U/L), creatine kinase isoenzyme MB 14.8 ng/mL (reference range 0–6.8 ng/mL); AST 119 U/L (reference range 8–38 U/L), ALT 390 U/L (reference range 0-75 U/L). Radiography of the abdomen revealed shadow of stool and gas as well as points of increased density (Fig. ).
It could not be determined whether the radiopaque particles seen on the abdominal x-ray contained lead or not. Since chelating agents may increase gut lead absorption, folium sennae was administered as a cathartic to eliminate lead from the intestine prior to initiating chelation. The chelation regimen was identical with case 1. However, after 2 days of therapy the white blood cell count fell to 2.11× 109/L (normal range: 4.0–10.0× 109/L). This may be attributed to DMSA, which was withheld subsequently. Chelation continued with an intravenous infusion of CaNa2EDTA to achieve a BLL of 36 μg/dL at the end of 5 days. |
pmc-6035448-3 | A 5-year-old boy had consulted the same TCM practitioner as in Cases 1 and 2 and was prescribed the same medication to be used twice a day for 7 days. Severe abdominal pain and vomiting followed. Prior to transfer to our hospital his initial laboratory data showed markedly elevated liver enzyme levels (ALT 1083 U/L, AST 972 U/L). No abnormality was found on abdominal x-ray. On examination, his liver was palpable 4 cm below the right costal margin. The BLL on admission was 105 μg/dL. The nasal spray contained 33.4% lead.
Chelation therapy was initially withheld because of the severely altered liver enzyme results as both drugs are potentially hepatotoxic. Initially, he received treatment aimed at improving liver function with glutathione and disodium glycyrrhetate (Table ) and continued to receive these medications during the whole course of treatment. As his liver function tests improved, his BLL went down concomitantly, prior to chelation (Table , BLL of Day 1: 105 μg /dL; Day 4: 96 μg/dL; Day 8: 80 μg/dL) at which point chelation treatment was initiated with DMSA and CaNa2EDTA infusion (same usage as Case 1). The post-chelation lead level for this child was 34 μg/dL. No adverse events happened to all the three children. |
pmc-6035790-1 | A 53-year-old woman had symptoms of bloody stool, repeated constipation, defecation habits change, and weight loss for 1 year (Table ). She was diagnosed with locally advanced rectal cancer in Jilin University Second Hospital, Jilin, China. The tumor was located 2 cm from the anus merge, and its size was 4 cm × 3 cm. The rectum wall was circularly covered around half area, and the pathology was adenocarcinoma by the colonoscopy biopsy. By the pelvic magnetic resonance imaging (MRI), lymph node metastasis was found and there is no distal metastasis detected by the chest X-ray, abdominal computed tomography (CT) scan, and hepatobiliary ultrasound. Particularly, anorectal manometry was required to be as an index measuring its preoperative anal function (Fig. ).
The carcinoembryonic antigen (CEA) level of this patient was 1 ng/ml, and CA19–9 level was 10.5 U/ml. She had no family history and other systemic diseases. After she signed the consent form, Lap ISR combined with IORT using low-energy X-rays and prophylactic ileostomy were performed on December 05, 2015, without preoperative chemotherapy or radiotherapy. |
pmc-6035803-1 | A 54-year-old, right-handed unemployed Moroccan Berber woman from an urban area reported a personal medical history of intermittent epigastric pain without a history of diabetes or chronic disease, nor any special psychosocial background, and with a familial history of allergic rhinitis. She presented with a 10-year history of progressively intense pain, cold sensitivity, and severe tenderness to palpation of the pulp of her left little finger, with no gross abnormalities of her fingers, and no previous trauma history. The pain increased when her digit was exposed to cold. Furthermore, the tip was excessively sensitive to touch, and her pain increased at night. She had seen a primary care doctor, with no definitive diagnosis. Moreover, she reported occasional intake of omeprazole for intermittent abdominal pain. She had no history of active or passive tobacco smoking or alcohol intake. She also had no past intervention. She was referred to our department for surgical excision with histopathological examination.
A clinical examination showed a well-oriented, apyretic, and eupneic patient, with normal cardiac frequency and regular blood pressure, presenting with a painful subcutaneous nodule of approximately 1.5 cm, of firm consistency and pinkish red coloration, streaked with multiple telangiectasias on the pulp of the distal phalanx of her left little finger (Fig. ). Polarized contact dermoscopy induced peripheral clearing of the reddish color, disclosing a yellow to white background, with multiple telangiectasias on the surface (Fig. ). A neurological examination showed no signs of paresthesia or hypoesthesia in the area of the tumor, nor at a distance, with a preserved muscular and neurological function; a general examination showed no other abnormality.
The differential diagnosis at the time of examination included glomus tumor, schwannoma, mucoid cyst, and neurofibroma. An X-ray study was done for her left hand. No bony lesions were identified by radiographic studies (Fig. ). She did not benefit from ultrasonographic imaging or magnetic resonance imaging (MRI) because the diagnosis of glomus tumor was highly probable.
Surgical intervention was performed. A paramedian volar incision was made of the pulp of the distal phalanx of her left little finger. The mass was well circumscribed and removed with blunt dissection and sent to pathology (Fig. ). It was a red soft tissue nodule of 1.5 cm in diameter and had no stalk or adherences to a joint. It was removed completely and dermoscopy of the excised tumor was performed showing yellow structureless areas surrounded by linear vessels. A histological examination confirmed a glomus tumor showing a tumor proliferation arranged around many narrow vascular clefts that circumscribed flattened endothelial cells. These vessels were surrounded by several superimposed layers of ovoid cells with round, regular nuclei and moderately acidophilic cytoplasm with imprecise boundaries (Fig. ). In places, these elements deviated from the vascular walls and spread irregularly, sometimes isolated or in small clusters, within a fibromyxoid stroma strewed with lymphocytes and some plasma cells. Her symptoms improved on removal of tumor and she healed without complication. At follow-up visits, she presented no signs of recurrence with complete healing of the pain within 1 year. |
pmc-6035805-1 | A 54-year-old postmenopausal woman from Casablanca (Morocco), presenting episodic abdominal complaints, was referred to our outpatient institution for laboratory testing as part of a routine checkup in May 2011. Her medical history was significant for extensive endometriosis confirmed by the laparoscopy procedure and infertility. The patient had surgery followed by hormonal therapy in 2003. At initial blood testing, the results indicated normal cell count with a hemoglobin of 14 gm/dl, a total leukocyte count of 8,200/mm, and a platelet count of 3,44,000/mm. Serum urea, creatinine, bilirubin, transaminases, total cholesterol, triglycerides, calcium, fasting blood glucose, C-reactive protein, gamma-glutamyl transpeptidase, alkaline phosphatase levels, and other electrolytes were normal (). However, the serum amylase level was 198 IU/L (reference range 30–110), and serum lipase increased to reach 1461 IU/L which is fivefold over the upper limit (reference range 27–280). Blood pancreatic isoamylase values were also abnormal. As the abnormal pancreatic enzyme secretions usually denote pancreatic pathology, a deep screening for pancreatic alterations was done to find out the possible causes of the elevated levels of lipase and amylase. Serological tests for hepatitis A, B, and C viruses and human immunodeficiency virus (HIV) were negative. The tumor markers (carcinoembryonic antigen, carbohydrate antigen 19-9, alpha-fetal protein, and carbohydrate antigen 125) showed normal ranges (). To exclude the common sources of hyperlipasemia and hyperamylasemia such as macroamylasemia, autoimmune diseases like systemic lupus erythematosus, celiac disease, and inflammatory bowel, investigations were done and all were absolutely normal. Abdominal ultrasonography and magnetic resonance imaging scans were performed in 2011 and showed normal pancreas, liver, and biliary tree (). The patient denied weight loss, diarrhea, vomiting, cigarette smoking, or alcohol drinking. The biochemical screening of pancreatic hyperenzymemia was negative in the patient's family members. Given the interest of this case, we decided to follow-up the patient with blood and imaging tests as long as possible. Five years after the first medical consultation, the patient remained asymptomatic although the serum lipase and amylase values fluctuated widely. The amylase and lipase median values were 205.5 and 831.5 U/L, respectively (). Interestingly, the lipase concentration never exceeded the value of 2,000 U/L. In the light of these observations, we hypothesized that hyperenzymemia in this situation may be considered as a true Gullo's syndrome case. |
pmc-6035807-1 | A 53-year-old female with no previous history visited a local hospital due to right wrist pain and swelling caused by falling. She was diagnosed with a distal fracture of the right radius, underwent splinting, and returned home. When she visited the local hospital again 2 days after the injury, blister formation on the right forearm was observed, and she was referred to our hospital. The blister was observed along the splint application area () and was considered to be due to the heat and stuffiness of the splint. Plain X-ray examination revealed a distal radius fracture accompanied by dorsal displacement of the distal bone fragment (AO classification: type A2) (Figures and ). Based on the skin condition, we considered conservative treatment by external fixation using a splint or cast to be difficult, and surgery after improvement of the skin state would be more invasive due to bone union and, therefore, planned minimally invasive locking plate osteosynthesis.
As we previously reported, surgery was performed using the Henry approach through a 10 mm incision starting from 15 mm proximal to the radial styloid process at 9 days after injury []. In this patient, there was no skin lesion at this incision site, which allowed this surgical technique (). After reduction of the distal bone fragment using a Kirschner wire, osteosynthesis was performed using a volar locking plate (Acu-Loc 2 proximal plate standard, Nihon Medical Next, Osaka Japan) (Figures and ). After the operation, a favorable alignment was obtained (Figures and ). The wrist was immobilized postoperatively in a bulky dressing without an arm splint until the tissue swelling had decreased.
All muscles, vessels, and nerves of the anterior compartment—except the radial artery—were retracted ulnarly. The pronator quadratus was incised transversely at its distal portion and dissected off the periosteum using a periosteal elevator preserving its ulnar and radial insertions. Therefore, active finger motion was encouraged immediately after the operation and wrist mobilization was started as soon, and as much, as pain allowed. Moreover, this approach can avoid the median nerve damages during the surgery.
Six months after the operation, favorable union of the radius was obtained. The wrist range of motion was as follows: flexion, 70°; extension, 65°; pronation, 85°; and supination, 85°. The visual analogue scale (VAS) was 1/10, and the quick disabilities of the arm, shoulder, and hand (Q-DASH) score was 20.45/100. The Mayo wrist score was 85/100 (excellent). The state of the forearm skin and surgical wound favorably improved (), and she returned to her preinjury job. |
pmc-6035812-1 | We report a case of a 62-year-old male who developed DRESS syndrome after seven weeks of antibiotic treatment with vancomycin. He initially underwent instrumented thoracic spinal fusion (T1–7) due to cord compression from a metastatic T4 lesion from renal cell carcinoma and developed a postoperative deep spinal infection. He underwent multiple washouts and vacuum-assisted closure over a period of twelve weeks, with various antimicrobial regimes, initially receiving seven weeks of vancomycin as well as a shorter duration of ciprofloxacin. He developed a maculopapular morbilliform rash, () initially on the right arm and scalp, before spreading to cover the entire head, trunk, and upper legs () which progressed to become exfoliative and was intensely pruritic and painful (). This was accompanied by a fever and eosinophil count of 9.77 × 10−9/L at the highest, occurring simultaneously with the development of the rash, and which remained elevated over the course of a month of regular blood tests. Other haematological abnormalities were also present, with a rise in both lymphocytes and neutrophils. Vancomycin was discontinued immediately, and other causes for these results were excluded, with negative blood cultures, CMV, EBV, ANA, and hepatitis B, hepatitis C, and HIV titres. There was no clinically apparent lymphadenopathy; however, a CT scan performed after the onset of symptoms showed new prominent right hilar lymph nodes, although this may have been due to metastatic cancer and not DRESS syndrome. Skin biopsy showed superficial perivascular lymphocytic infiltrate and rare eosinophils, consistent with a morbilliform drug rash. Ciprofloxacin was felt to be unlikely to be the cause of his DRESS, as he had been prescribed the drug several times in the past, as well as having a shorter duration of treatment which would not fit with the typical timeframe for DRESS, so this was continued to treat his infection.
The patient initially received a single dose of intravenous high-dose hydrocortisone, but due to the severity of infection and the risk of immunosuppression, he was subsequently treated exclusively with topical steroids, emollients, and antihistamines (). No liver or renal function abnormalities were noted during this time; however his eosinophils remained raised as described. He developed acute chest pain and shortness of breath four weeks after the initial rash, with new onset fast atrial fibrillation and negative troponin and creatinine kinase. A CT scan demonstrated bilateral pleural effusions, as well as progression of lung and rib metastases. An echocardiogram showed mild left ventricular and right ventricular impairment and a rim of pericardial fluid. Unfortunately, within three months of initial surgery, the metastatic spinal load increased causing further cord injury and paraplegia. Further surgical intervention was deemed inappropriate at this point, and the patient was discharged to the community palliative care team. |
pmc-6035818-1 | A 53-year-old, blood group B, Rh(D)-positive female with history of metastatic adenocarcinoma status after chemotherapy treatment presented to the emergency department with abdominal pain, vomiting, and fever. She was admitted for a possible small bowel obstruction and sepsis. Upon her admission, her complete blood count (CBC) revealed a normal platelet count of 186 × 103 (reference range: 130–400 × 103/mm3), low white blood cell count of 2.7 × 103 cells/mm3 (reference range: 4.4–11 × 103 cells/mm3), normal hemoglobin level of 15.9 g/dL (reference range: 12–16 g/dL), and a normal hematocrit of 46.6% (reference range: 37–47%). Red blood cell antibody screen was negative. Over the next few days, the patient's platelet count continually decreased, reaching a critical low level of 10 × 103 cells/mm3 one week after admission. She received a single unit of apheresis platelets from a group A, Rh(D)-positive donor. The unit initially contained 3.7 × 1011 platelets per milliliter (mL) within total volume of 270 mL. The platelet product was suspended in Anticoagulant Citrate Dextrose Solution, Solution A (ACD-A) and transfused on storage day 5. The red blood cell visual count was reported negative. Approximately ten minutes after the transfusion was started, the patient began to complain of severe lower back pain. The pain was described as 10/10, sharp and stabbing. No other signs or symptoms were reported, including no fever or blood pressure changes. The patient received 135 mL (50%) of the platelet product. The primary provider care team was notified, and the patient observed while receiving normal saline at 100 mL/hour. No other treatment was initiated. The patient reports the pain 1 1/2 hours later to be 5/10 and “much better” 2 hours after transfusion. A transfusion reaction work-up was initiated, which revealed a posttransfusion blood sample with visible hemolysis and 1+ positive direct antiglobulin test (C3b and C3d positive; IgG negative). No eluate was performed due to absence of detectable IgG on the posttransfusion DAT sample. Pretransfusion blood sample showed no hemolysis, and pretransfusion DAT was negative. Subsequent Gram stain of the platelet unit revealed no organisms seen. Aerobic and anaerobic cultures of the platelet unit after transfusion showed no growth at 5 days. Initial cultures performed by the blood center also reported no growth at 5 days. No medications or fluids were infused with the product. The results of the work-up were consistent with an acute hemolytic transfusion reaction related to an apheresis platelet unit from a group A, Rh(D)-positive donor to a group B, Rh(D)-positive recipient. The antibodies (anti-B) from the platelet unit were titered and found to be high-titer at 512 (). Titer performed following the College of American Pathologists (CAP) tube method “uniform procedure” utilizing 0.9% normal saline (NaCl). The hemoglobin 14 hours prior to transfusion was 8.9 g/dL. The hemoglobin 1 hour 15 minutes after transfusion was 7.4 g/dL. From a transfusion medicine standpoint, the patient remained stable throughout the remainder of her hospital admission. The platelet product was donated by an 18-year-old male and was part of a double platelet unit donation. The companion platelet aliquot was transfused at another institution to a group compatible recipient without reported adverse event. The donor had previously donated a double red cell product with no known associated adverse event. Multiple attempts to contact donor after reaction did not receive a response. History of probiotic use was not reported by the donor or asked at time of donation. Status of probiotic use could not be confirmed after donation. |
pmc-6035819-1 | A 32-year-old, gravida 8, para 5, aborta 2 monochorionic diamniotic twin pregnancy (MCDA) was referred as a high-risk pregnancy to our fetal assessment clinic at 25 weeks of gestation for detailed fetal ultrasonography (USG).
The woman had no history of any past medical illness. All her previous pregnancies had resulted in healthy live births at term by vaginal delivery. This pregnancy had been conceived naturally, and she had not been detected to be hypertensive or diabetic. Furthermore, there was no history of consanguinity with her husband.
The USG revealed an MCDA twin pregnancy with discordant growth (28%) of the twins. One fetus was detected to have left hypoplastic heart, with a single umbilical artery, the absence of the kidney and bladder, and lower extremity deformities with an absence of feet. The other fetus was observed to have a normal growth and biophysical profile. The placentas appeared fused in the posterior uterine wall with barely detectable dividing membranes. The amniotic fluid index was grossly normal for both fetuses. Fetal echocardiography was performed and revealed hypoplastic left heart, transposition of the great arteries (TGA), and pulmonary atresia.
The patient and her husband were informed regarding the poor prognosis of the anomalous twin fetus. At 36 weeks of gestation, after antenatal steroid coverage, an elective caesarean section was performed for the patient in view of the MCDA twin with malposition of the first twin in accordance with the hospital protocol.
The neonate was born with severe growth restriction with a weight of 1300 gm and cyanosed with a poor Apgar score. As a result, soon after the delivery, it was shifted to the neonatal intensive care unit (NICU). Physical examination of the baby revealed microcephaly (head circumference 29 cm), low-set ears, right hand with polysyndactyly, and complete fusion of the entire lower extremities with an absence of feet (Figures and ). There was no anal opening, and no external genital organs could be identified. The infant was clinically diagnosed as a case of sirenomelia.
Radiologic examinations revealed vertebral deformity at the midthoracic spine, partial sacral agenesis, bilateral hypoplastic iliac bones, complete fusion of both femurs (), fused proximal tibia, and an absence of fibula and feet, which was consistent with type VI (sympus apus) sirenomelia []. The oesophageal atresia and distended bowel loops present could be secondary to anal atresia.
Neonatal echocardiography was performed, and the diagnosis of hypoplastic left heart syndrome, TGA, and pulmonary atresia was made. USG abdomen and pelvis demonstrated an absence of the bladder and both kidneys with other normal abdominal organs. Chromosomal analysis confirmed the female karyotype 46,XX. The neonate survived for 3 days under human care. An autopsy was not performed due to cultural and social constraints. The other twin was healthy, weighing 2500 gm, and was discharged in good condition after routine preterm care. |
pmc-6035825-1 | A 29-year-old, right-handed, 6-month pregnant woman presented to another hospital's emergency service with a history of falling on her outstretched left hand. X-ray was not administered to her due to pregnancy. She was treated with a long-arm splint. After 6 weeks, she was admitted to our outpatient clinic with pain and swelling around the elbow. Her elbow flexion-extension range was measured as 60/100 and supination-pronation was measured as 45/45. The elbow varus-valgus stress tests were normal. There was no neurovascular deficit. Plain X-rays were taken by using a lead shield to protect the fetus. Radiographs showed a Bryan and Morrey type I osteochondral capitellar fracture that displaced anterosuperiorly (). There was no concomitant injury in the forearm, radius, or distal radioulnar joint. A computerized tomography (CT) scan was not performed due to her pregnancy. Open reduction and internal fixation was planned for the patient. The patient was consulted with an obstetrician preoperatively. An informed patient's consent was obtained for emergency caesarean delivery in case of acute decompensation of the fetus during surgery. The fetal heart was monitored during surgery and operation was completed without any complications. RIVA (regional intravenous anaesthesia) was applied. The patient was operated in a supine position under tourniquet control. Using the posterolateral approach as described by Kocher, the fracture was fixed. Headless cannulated compression screws (3.0 mm Barouk screws, DePuy, Lyon, France) were used for fixation. A flouroscopy was taken at the end of the surgery, using a lead shield to protect the uterus. The elbow was immobilized using a posterior long-arm splint for 3 days to prevent swelling. This was followed by a progressive elbow mobilization program guided by a physiotherapist. She was called for clinic control at 1, 2, 3, 6, and 12 months. She gave birth to a healthy baby three months after the operation. At this time, the fracture site was considered to be healed based on radiographic appearance of the fracture and absence of any pain on movement during the clinical evaluation (). She attained 135 degrees of flexion and full extension (0–135). Supination-pronation was measured at 180 degrees three months after surgery with no further complications. Her clinical examination revealed a perfect result with a full pain-free range of motion at the 6-month () and 12-month follow-ups. Informed consent was obtained from the patient for the publication of this report, related pictures, and radiographies. |
pmc-6035826-1 | A 10-week-old girl was the product of a full-term pregnancy which was complicated by maternal hypertension. No significant past medical or family history was present. The patient presented with a one day history of multiple episodes of nonbilious emesis, hematochezia, lethargy, and fussiness. On physical examination, the abdomen was diffusely tender to palpation with mild distension, and bowel sounds were audible. No cutaneous vascular lesions were noted at the time.
An abdominal ultrasound showed an apparent colocolic intussusception with no substantial interloop fluid collection, and color Doppler flow was demonstrated within the walls of the intussuscipiens and intussusceptum. A reduction of the intussusception was attempted with air contrast enema, and the intussusception was initially present at the rectosigmoid junction. With pressure maintained at less than 120 mm·Hg, the intussusception was reduced to the proximal descending colon, but the patient developed free intraperitoneal air apparent by fluoroscopy. During laparoscopic exploration, an intussusception was identified at the level of the distal descending colon, and fibrinous exudate was found along the descending colon, consistent with a perforation site. A segmental colonic resection with anastomosis was performed.
The resected segment of large intestine showed a telescoped segment of intestine. The intestinal wall measured from 0.2 to 0.5 cm. A minute gross perforation was identified; however, there was no evidence of a gross vascular malformation. Microscopic examination showed mucosal ischemic changes with vascular congestion. A vasoformative anomaly was present in the submucosa with involvement of the overlying mucosa where the lamina propria was occupied by a dense network of capillary-sized vascular spaces (). Immunohistochemistry revealed that the endothelium was positive for vimentin, CD31 (), CD34, GLUT-1 (), and nonreactive for D2-40 (). |
pmc-6035830-1 | The current disease story of our 68-year-old Caucasian male started two years ago, when this patient observed a hard lesion in the anal canal, which, in the first months since he observed it, was neither officially diagnosed nor treated. Ten months after that, he underwent cholecystectomy due to cholelithiasis, and the perioperative diagnostic examination revealed some suspicious lesions in the anal canal and some enlarged pelvic lymph nodes. His comorbidities included only gout, and his Karnofsky performance status was 90. He has never been treated with radiotherapy, and he had a second-degree family history for malignancies. At that time, a digital rectal examination identified mild prostatomegaly, with a palpable suspicious nodule in the left prostate lobe, without any signs of the extracapsular extension. Also, a large ulcerated mass was palpable at the dorsal anal canal wall, without lymphadenopathies in the inguinal region. Subsequent colonoscopy confirmed the presence of a suspicious lesion on the posterior anal canal, extending above up to the anorectic junction. A magnetic resonance imaging (MRI) for the regional staging confirmed an infiltrative lesion of the anal canal with a maximum diameter of 3.2 cm, with a high-grade suspicion of sphincter infiltration, and two enlarged lymphadenopathies in the mesorectum and in the right internal iliac region, respectively, both with a maximal diameter of 2 cm, which is suspicious of tumour metastases. Further, an area with a maximal diameter of 2 cm within the left prostate basis having a restricted diffusion with a lower apparent diffusion coefficient (ADC) than that in the surrounding healthy prostate tissue, which appeared hypointense on ADC maps but hyperintense on the diffusion-weighted maps, was also described. Afterwards, biopsies confirmed a poorly differentiated squamous cell carcinoma of the anal canal, as well as a moderately differentiated prostate adenocarcinoma, with a Gleason score of 7 (3 + 4) in both lobes. He resulted human immunodeficiency virus- (HIV-) negative, and his carcinoembryonic antigen (CEA) and prostate-specific antigen (PSA) were 2.09 U/L (reference range = 0–5.0 U/L) and 0.74 ng/mL (reference range = 0–4.0 ng/mL), respectively. Additional staging with the whole-body computed tomography (CT) scans excluded the presence of any distant metastases. Therefore, our patient had a synchronous intermediate-risk stage IIB cT2cN0M0 prostate adenocarcinoma and stage IIIB cT2N2M0 anal canal squamous cell carcinoma.
A multidisciplinary team which included surgical, medical, and radiation oncologists evaluated this case and passed a decision to submit the patient to the definitive concurrent radio-chemotherapy.
Regarding this clinical entity, the current papers are “blind,” and at this moment, they can provide us only with one case report on early-stage I metachronous small anal canal squamous cell carcinoma (cT1N0) and intermediate-risk prostate cancer (cT1cN0) that was published in 2011 []. Due to the relative lack of experience in the intensity-modulated radiation therapy (IMRT) at the time, which should be a more appropriate radiotherapy technique for managing this kind of conditions, the authors planned a conventional 3D conformal radiotherapy by using a wide anterior-posterior/posterior-anterior field arrangement with concurrent mitomycin C (12 mg/m2 i.v. bolus on day 1) and 5-fluorouracil (1000 mg/m2 on days 1–4 (week 1) and 29–32 (week 5) by continuous 24 h i.v. infusion). Due to the use of this technique, the prescribed doses for the anal canal carcinoma and the prostate carcinoma were 50.4 Gy and 73.8 Gy, respectively. That treatment has been well tolerated, without any significant gastrointestinal or genitourinary toxicity, except for one-week treatment break, due to the moist desquamation in the bilateral inguinal and intergluteal areas. The last restaging eighteen months later showed no evidence of the disease or of the cancer whatsoever. |
pmc-6035833-1 | A 55-year-old Caucasian male with past medical history of cerebral palsy (CP) presented with nausea, vomiting, thirty-pound weight loss, and worsening bilateral lower extremity weakness for one month. A computerized tomography (CT) angiogram of the brain revealed a suprasellar mass facilitating transfer to our institution for further management. Magnetic resonance imaging (MRI) of the brain indicated abnormal enhancement along the ependymal margin of the frontal horns of the bilateral lateral ventricles with four distinct abnormal enhancing mass lesions in the hypothalamus (11 × 12 × 13 mm), pineal gland (8 × 8 × 9 mm), the trigon of the right lateral ventricle (5 × 5 × 4 mm), and the foramen of Magendie (7 × 6 × 9 mm) which demonstrated restriction diffusion indicating hypercellularity ().
An endoscopic biopsy of the third ventricle floor lesion was performed with pathology revealing sheets of intermediate size monotonous lymphoid cells displaying high nuclear-to-cytoplasmic ratio with dispersed chromatin and indistinct nucleoli. Numerous apoptotic cells and mitotic figures with foci of necrosis were observed. The tumor cells displayed CD 20 with coexpression of CD 10 and were negative for BCL 2, BCL 6, CD 3, and CD 5. EBER in situ hybridization was also negative. Fluorescent in situ hybridization was positive for [, ] (MYC/IHG) fusion in 97% of the cells and loss of BCL2 in 96%. These results appeared to be consistent with Burkitt lymphoma.
Staging workup was obtained which only revealed concern for extra cranial disease present at T12-L1 and L2-L3 consistent with subarachnoid nodular pial metastases on MRI of the lumbosacral spine. PET/CT disclosed no evidence of extra-CNS disease. A lumbar puncture and bone marrow biopsy were performed and found to be negative for disease. In the absence of extra-CNS disease, the patient was diagnosed with PCNSBL.
Patient was started on IV HD-MTX (3.5 grams per meter squared) and cytarabine (2 grams per meter squared) per Ferreri regimen [] with the addition of IT MTX/cytarabine every 21 days for four cycles. Dose was reduced by 25% for cycle three due to persistent cytopenias and toxicity including renal dysfunction with delayed MTX clearance. A repeat brain MRI was obtained after 6 months which indicated complete remission with no evidence of disease ().
Four months later, the patient began having generalized weakness with visual disturbances and headaches. A repeat brain MRI at that time revealed interval development of markedly abnormal signal in the pons extending to the midbrain and dorsal medulla. Repeat cerebral spinal fluid (CSF) analysis showed rare atypical lymphocytes consistent with relapse of lymphoma. He underwent WBRT, receiving 30 Gray (Gy) over 17 fractions with an additional boost to the midbrain lesion with 9 Gy in 8 fractions. Currently, he is disease-free at 30 months s/p diagnosis. |
pmc-6035840-1 | An 8-year-old girl was referred for investigation of hyperthyrotropinemia (increased TSH) and cushingoid features probably due to oral prednisolone intake prescribed for the management of her AD. The patient was prepubertal and overweight, with a body mass index (BMI) of 26.56 kg/m2. Already by the age of 6 months, our patient was diagnosed with mild atopic dermatitis which was successfully managed with emollients. As a toddler, atopic dermatitis relapses became more frequent, but the episodes were still easy to control with topical emollients and corticosteroids. Complete and thorough assessment for common allergens was negative. During breastfeeding, for almost 6 months, Vit D supplementation was not provided. After the age of 5 years though, her dermatitis worsened, and the lesions spread all over her body (SCORAD 70) []. Intense scratching of the patient, which was very difficult to alleviate with the topical use of antibiotics, corticosteroids, and oral antihistamines, caused sleep disturbances and a huge frustration to the family (). All available treatments and protocols using local and systemic drug therapeutic options including calcineurin inhibitors and methotrexate had failed to control the disease. Narrowband ultraviolet B phototherapy, normally not recommended for <18 yrs of age, had not been offered, tried, or discussed by her treating dermatologists. Regarding family history of other atopic conditions, the older sister was diagnosed with mild atopic dermatitis. The mother claimed latex allergy, and the father mentioned he had unclear food allergy in his childhood. Nobody else in the family suffered from asthma or allergic rhinitis.
Upon review, we suggested a new measurement of TSH and several other laboratory tests including Vit D levels ().
We treated her with 0.5 mcg × 3/day calcitriol and 4000 IU/day cholecalciferol p.o. During treatment, no other medications except for local use of moisturizers were allowed. After two months, the patient's AD had improved significantly, and both the pruritus and the extent of the eczematous lesions were reduced. Then, we increased the dose to 1 mcg × 3/day calcitriol maintaining the cholecalciferol dose. At 6 months, there was almost healing of her skin with only mild existing dryness, no itch, and absent inflammation (SCORAD 10) () with signs of postinflammatory pigmentation over her skin as well. Her quality of life had dramatically improved, and from complete depression such as dressed always veiled, experiencing constant fatigue, feelings of helplessness, pessimism, and hopelessness, sleeping too much and overeating, loss of interest in things once pleasurable, and persistent sad or “empty” feelings, she was joyful again resuming all sports and outdoor activities she ever dreamed of. We continued the same treatment controlling every 6 months the biochemical parameters related to calcium metabolism, and more specifically calcium (Ca), phosphorus (P), parathormone (PTH) in serum, and Ca and Ca/creatinine (Cr) in 24-hour urine samples. For one year, the values were within the normal range, and no adverse effects were detected. At 12 months, while on calcitriol 1 mcg x 3/day, we detected an increased Ca/Cr ratio in the urine for her age, and we decided to switch from calcitriol therapy to the “equivalent” as far as VDR activation is concerned with paricalcitol dose, that is, 2 mcg × 3/day. Written informed consent was obtained for the off-label use of paricalcitol. Clinically, the patient remained stable () practically free of AD but hypercalciuria resolved completely (). Paricalcitol treatment is being continued for 2.5 years now with a 6-month follow-up. In the future, we will reassess a possible discontinuation of the treatment which for now is out of the question by the patient herself considering the stress, the psychological pressure, and the physical “disability” she experienced due to the disease in the past, expressing fear in any relevant discussion. |
pmc-6035845-1 | A one-year-old Thai boy was referred to Phramongkutklao Hospital due to subacute fever, abdominal distension, mucous diarrhea, and failure to thrive. He was born at term with uneventful pregnancy, and he is the first child of nonconsanguineous parents. There was no history of autoimmune or primary immunodeficiency disorders in the family. Intradermal BCG vaccination was inoculated at the left buttock without any reaction within 3 months of life, and intramuscular vitamin K was routinely given after birth. He was exclusively breastfed. At 3 months of age, he developed frequent vomiting and irritability. Physical examination revealed enlarged and tense anterior fontanelle. CT brain showed hyperdensity lesion size of 1.5 × 1.8 cm at left temporal lobe with perilesional edema () which was confirmed to be intracerebral hemorrhage. All hematologic, coagulation studies and biochemical laboratory tests () were consistent with deficiency of vitamin K dependent clotting factors. The cause of vitamin K deficiency in this patient was presumed to be caused by malabsorption mechanism. Therefore, intravenous vitamin K was given for 3 days at initial presentation, and the coagulogram data was corrected within 24 hours. One week later, the patient developed steatorrhea. Fat malabsorption was suspected as the levels of fat-soluble vitamins were evaluated (). Cystic fibrosis was excluded by the negative sweat chloride test. At 4 months of age, perianal abscess was detected and treated with amoxicillin/clavulanic acid for 7 days without surgical drainage. However, subsequent pus culture was not performed. At the age of 6 months, lymphadenopathy of 3 cm in size at the left groin was detected. Fine needle aspiration was accordingly performed, and pus culture was found to be positive for BCG and the tuberculin skin test was positive at 15 × 20 mm. Chest X-ray revealed no pulmonary infiltration. The patient was diagnosed with BCG lymphadenitis and was treated with isoniazid and rifampicin. Interestingly, there was no history of tuberculosis contact in the family. Nevertheless, the patient did lose to follow-up which resulted in the delay of definite diagnosis in this patient.
On physical examination at age of 1 year, his weight was 7.8 kg (<3rd percentile) and height was 69.5 cm (<3rd percentile). Abdominal distension, moderate hepatosplenomegaly, and ascites were detected. Left inguinal lymph node was still palpated with 1.5 cm in size. The site of BCG vaccination showed no induration. Physical examinations were unremarkable. Hematologic and biochemical laboratory tests were described (), and chest radiography showed consolidation at the left upper lobe (). Abdominal CT showed generalized ascites with evidence of hepatosplenomegaly. Abdominal paracentesis was performed, and the results were described (). Ascitic fluid adenosine deaminase (ADA) was performed because of suspicious of mycobacterial infection, and the result was compatibly high. The ascitic fluid PCR was positive for BCG. Disseminated BCG infection was diagnosed in our patient. IgG was slightly elevated (1,236 mg/dL) while IgM and IgA levels were normal (). Lymphocyte subset analyses revealed normal T-cell and B-cell counts. The neutrophil dihydrorhodamine (DHR) test revealed no fluorescence detection after granulocyte stimulation. The stimulation index (SI) was 1.21 which was compatible with XL-CGD ().
Finally, the patient was diagnosed with XL-CGD accompanied with disseminated BCG infection. This clarified the clinical of fat malabsorption leading to vitamin K deficiency since 3 months of age. Treatment was started with antituberculosis including isoniazid, rifampin, pyrazinamide, ethambutol, and amikacin. Itraconazole and co-trimoxazole were given as the prophylactic treatment. The clinical of ascites and steatorrhea was improved after 2 weeks of treatment. The DHR assay was also performed on the mother and revealed bimodal distribution compatible with the XL-CGD carrier (). Allogeneic hematopoietic stem cell transplantation (HCT) is therefore planned as the curative treatment since the mother is six months pregnant.
After informed consent was obtained from the patient's parents, genomic DNA was extracted from peripheral blood leukocytes using the commercial available kit as per the manufacturer's protocol. Thirteen coding exons and exon-intron boundaries of the CYBB gene were amplified by PCR using specific of primers for each exon as previously described []. The PCR products were purified and directly sequenced in both forward and reverse directions. The reference sequences were NM_00397.3 for CYBB cDNA and NP_000388.2 for gp19-phox protein. |
pmc-6035848-1 | A 61-year-old woman with diabetes mellitus, hypertension, and hyperlipidemia initially presented with unstable angina; a regadenoson stress nuclear myocardial perfusion imaging (MPI) revealed anterolateral wall ischemia. Subsequent coronary angiography demonstrated severe stenoses of the left anterior descending (LAD) artery, left circumflex artery, and right coronary artery. She underwent CABG with a left internal mammary artery (LIMA) graft to the LAD artery and saphenous venous grafts (SVG) to the right posterior descending (RPDA) and obtuse marginal 4 (OM4) arteries. After CABG, the patient was doing well on guideline-directed medical therapy. Three years later, she presented with acute onset of chest pain suggestive of unstable angina. On admission, her heart rate was 80/min, blood pressure was 140/86 mmHg, and her physical exam was unremarkable. |
pmc-6036243-1 | As summarized in Figure , 7 years after a revision arthroplasty of the left hip joint, a 64-year-old woman presented with increasing pain in her left hip (admitted on day 0). No sinus tract was apparent, but a 4 cm × 3 cm skin burst with crusting was observed in the anteromedial region of the left knee (unknown cause), and the surrounding skin was red and hot, with a few pustules. Bacteria isolated from the cultured aspirate (obtained from left hip joint cavity, purulent and bloody fluid) were identified as “S. aureus” (strain XNO62) by routine biochemical tests and 16S rRNA gene analysis. Surgical debridement was proposed, but the patient refused. Periodic dressing changes for the knee, irrigation with amikacin, injection with vancomycin for the hip joint, and systemic applications of amikacin and vancomycin were performed. The patient was discharged (day 7) after clinical symptoms improved and infection indicators declined.
On day 89, the patient was readmitted with pain and swelling in the left hip. A sinus tract and effusion of canary yellow liquid were seen in the previous operative incision. Amikacin and vancomycin were administrated intravenously and radical debridement of the hip joint was performed. Although joint fluid and tissues were sampled several times, no pathogen was found until day 100, when another “S. aureus” strain (strain XNO106) was isolated from the periarticular tissue in left hip during an operation. Strain XNO106 formed pinpointed colonies compared to strain XNO62, suggestive of slow growth. The patient was discharged on day 115 after clinical symptoms improved and infection indicators declined. The patient continued to have frequent relapses of pain and infection of the hip afterward. For dynamic changes of laboratory data, values of white blood cell (WBC) count, neutrophilic granulocyte percentage (Neu%), C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were all high in the initial infection (caused by strain XNO62). In the second hospitalization, the WBC, Neu%, and CRP values were all relatively lower (increased WBC and Neu% values at day 100 might be caused by the operation), but ESR value was always high throughout the course of the infection (the patient had no hematologic disorder that may cause high ESR value). |
pmc-6036671-1 | A previously healthy 3-year-old boy showed limping with the left leg pain, and then fever 6 h later. Next morning, he was transferred to an emergency hospital because of loss of consciousness and purpuric legs. Within a couple of hours, shock vital signs emerged and ecchymoses extended over the lower extremities. The patient was admitted to a pediatric intensive care unit on cardiopulmonary assist and catecholamine support. He had atopic dermatitis and one history of pneumonia in infancy. The growth and development were normal. There was neither consanguinity nor informative family history.
On admission, the comatose patient showed 180/min of tachycardia and unmeasurable blood pressure on the assist ventilation. Light reflex was prompt. The body temperature was 40.1 °C. Capillary refilling time was prolonged over 2 s, while cardiopulmonary sounds were normal. There was no hepatosplenomegaly or lymphadenopathy. Purpura and ecchymoses expanded to the both legs with necrotic toes (Fig. ). Petechiae spread over the face, body and upper extremities. Complete blood counts showed a leukocyte count of 0.329 × 109/L with 80% neutrophils, 17% lymphocytes, 3% monocytes, a hemoglobin concentration of 11.0 g/dL, and a platelet count of 3.8 × 109/L. Schizocytosis and hemoglobinuria indicated hemolysis. Serum biochemistries revealed increased levels of blood urea nitrogen (24 mg/dL, reference range [rr]: 8–20), creatinine (0.5 mg/dL, rr: 0.2–0.45), total bilirubin (1.8 mg/dL, rr: 0.3–0.9), aspartate aminotransferase (381 U/L, rr: 24–43), alanine aminotransferase (99 U/L, rr: 9–30), lactate dehydrogenase (1203 U/L, rr: 190–365), creatine kinase (731 U/L, rr: 43–270), and C-reactive protein (12.83 mg/dL, rr: < 0.04). Coagulation studies showed hypofibrinogenemia (139 mg/dL, rr: 200–400), prolonged prothrombin time (PT 21.2 s, control 10–13.5 s) and activated partial thromboplastin time (APTT 53.1 s, control 26–41 s), and increased levels of fibrinogen degradation products (FDP 445.0 μg/mL, rr: 1–10) and D-dimer (142.1 μg/mL, rr: 0.15–1). Thrombin-antithrombin complex (TAT) was > 120.0 ng/mL (rr: < 4), plasmin α2–antiplasmin complex (PIC) was 25.2 μg/mL (rr: < 0.8) and thrombomodulin 37.7 FU/mL (rr: < 4.5). Plasma activities of protein C (13%, rr: 64–131) and protein S (52%, rr: 62–121) were respectively low, and those of antithrombin were normal (85%, rr: 68–130). The rapid antigen tests were positive for S. pyogenes and negative for Pneumococcus. S. pyogenes, determined later as the serotype M12, was isolated from throat and peripheral blood. Sterile cerebrospinal fluid showed no pleocytosis. These determined the diagnosis of acute infectious PF with S. pyogenes-septic shock. Antibiotic and anticoagulant therapy was started on the intensive care. Next day, leg purpura and acral gangrene extended. Anticoagulation with plasma-derived activated protein C (Anact C®), gabexate mesilate, fresh frozen plasma replacement, and platelet infusion, was intensified by the addition of a direct thrombin inhibitor (argatroban), antithrombin, recombinant thrombomodulin, and low-molecular heparin. The relaxing incisions were then conducted for the compartment syndrome of both legs on the 3rd day of illness. S. pyogenes was also isolated from the debridement tissues, and the histopathology indicated absent necrosis of the fascia and necrotic alteration of the muscle without specific inflammation. The continuous infusion of penicillin G led to the negative blood culture 24 h after the start of antibiotic therapy. Under controlled sepsis and coagulopathy, continuous irrigation and debridement were repeated. On the 13th day, debridement tissues showed necrotic muscle and fascia with chronic inflammatory changes. The histological changes indicated that the necrotic muscle tissues originated from ischemia rather than spreading infection of the fascia. The distal portions of the left metatarsal bone were finally amputated. Repeated skin-autografts resulted in the cosmetic and functional improvement of the extremities. There was no evidence of primary immunodeficiency diseases including asplenia, phagocyte disorders, hypogammaglobulinemia, immunoglobulin G subclass deficiency, complement deficiency or IRAK4/MyD88 deficiency, and absent mutation of STAT3, PI3KD, and PIK3RI genes. The genetic study of protein C or Factor V Leiden excluded heritable thrombophilia. He lives an active life for walking rehabilitation with no delayed development at 6 years of age. The isolated strain possessed T12 and M12 antigens, and emm12.0 gene, but no mutation of csrS/R or rgg.
Twelve reported patients with acute infectious PF with S. pyogenes infection including the present case are shown in Table . Nine patients developed at age ≤ 10 years, and the remaining three were ≥ 50 years of age. All but one cases presented with septic shock and DIC, but not necrotizing fasciitis. Four immunocompromised patients were over 6 years. Five children age < 8 years had no underlying disease, and survived. The youngest one and two immunocompromised patients died. |
pmc-6036688-1 | A 52-year-old Chinese woman presented to our hospital in September 2012 with a complaint of recurrent renal stones for 6 years. The renal stones were first discovered at a local hospital 6 years ago and then she underwent bilateral ureteroscopic lithotomy and ultrasonic lithotripsy several times, but the calculus still relapsed and her serum creatinine gradually increased to 240 to 300 umol/L. She was a teacher living with her husband and two children, all healthy, in a small city. She did not smoke tobacco or consume alcohol. She had no significant past medical history, she had not suffered from any infectious disease, and she did not suffer from any chronic illness. There was no family history of similar disease or any other chronic illness. There was no history of allergy to any food or drugs. On physical examination: temperature (T) 37.5 °C, pulse (P) 82/minute, respiratory rate (R) 20/minute, blood pressure (BP) 130/80 mmHg, weight (W) 51 kg, and height (H) 154 cm. Respiratory movement and cardiac examination were normal. On abdominal examination no masses or tenderness were noted on both light and deep palpation. Her liver and spleen were not palpable. A sensory and motor system examination did not reveal any abnormality. Her neurological reflexes were normal. A routine urine examination showed: white blood cell 87/hpf (WBC2+), red blood cell 34/hpf (RBC2+), and pH 7. 0. A routine blood test showed: hemoglobin (Hb) 95 g/L. Her serum potassium was 3.2 mmol/L, calcium 2.92 mmol/L, phosphate 1.3 mmol/L, carbon dioxide combining power (CO2CP) 17.8 mmol/L, creatinine 249 μmol/L, serum parathyroid hormone (PTH) 1147 pg/ml (Table ), 25-hydroxyvitamin D3 level 42.98 nmol/L with reference range (RR) of 47.7–144 nmol/L, 24-hour urinary calcium 8.98 mmol/day (RR, 2.5–7.5 mmol/day), and 24-hour urine potassium 26.8 mmol/day. Liver and thyroid functions were all normal. Urine culture showed the presence of Escherichia coli. Renal ultrasound showed multiple stones, cysts, and calcified lesions. Computed tomography (CT) of her kidney showed MSK and polycystic kidney disease (Fig. ). Ultrasonography of her thyroid and parathyroid showed two hypoechoic nodules in the right lobe of her thyroid with the larger one of 26 × 18 mm. She was diagnosed as having MSK complicated with tertiary hyperparathyroidism and underwent right parathyroidectomy on September 18, 2012. Histopathologic examination revealed adenomatous hyperplasia of parathyroid glands (Fig. ). After surgery, her serum potassium was 3.3 mmol/L, calcium 2.81 mmol/L, phosphate 1.01 mmol/L, CO2CP 16.1 mmol/L, PTH 742.5 pg/ml, and serum creatinine 231 μmol/L (Table ). The oral form of iron and calcitriol was administered.
In March 2013, 6 months after resection of her right parathyroid glands she was re-hospitalized because of generalized bone pain of 1 month’s duration. A routine urine examination showed: white blood cell 45/hpf (WBC1+), red blood cell 27/hpf (RBC1+), and pH 7. 5. A routine blood test showed: Hb 88 g/L. Her serum potassium was 3.1 mmol/L, calcium 2.83 mmol/L, phosphorus 1.21 mmol/L, CO2CP 19.7 mmol/L, creatinine 265 μmol/L, and PTH 1378 pg/ml (Table ). Her 24-hour urinary calcium was 8.5 mmol/L and 24-hour urinary potassium 29.6 mmol/L. A bone scintigraphy for whole body with 99mTc revealed increased activity in skull, rib, and sternum (Fig. ). Parathyroid and thyroid ultrasound showed multiple hypoechoic masses in left lobe, with largest ones being 25 × 23 mm and 22 × 20 mm. She was diagnosed as having MSK and tertiary hyperparathyroidism and underwent left lobe parathyroidectomy on March 27, 2013. A pathological examination of the resected parathyroid tissue showed parathyroid nodular hyperplasia (Fig. ). After surgery, her serum calcium was 2.25 mmol/L, phosphorus 1.07 mmol/L, potassium 3.7 mmol/L, CO2CP 16.8 mmol/L, PTH 47.81 pg/ml, and creatinine 257 μmol/L (Table ). Subsequently she was prescribed with oral form of iron and calcitriol at the time of discharge; later, she did not return for the scheduled follow-up visits.
On July 7, 2016, 4 years after parathyroidectomy she was readmitted to our hospital with the complaint of dizziness. A physical examination showed pale appearance, visible thyroid surgery scar, and mild swelling of both lower extremities. A routine urine analysis showed: white blood cell 41/hpf (WBC +) and pH 7.0. A routine blood test showed: Hb 76 g/L, potassium 4.0 mmol/L, calcium 2.09 mmol/L, phosphorus 2.37 mmol/L, CO2CP 15.6 mmol/L, serum creatinine 766 μmol/L, and PTH 766.4 pg/ml (Table ). Renal ultrasound showed MSK, multiple stones in both kidneys, and polycystic kidney disease. She was diagnosed as having MSK and tertiary hyperparathyroidism and underwent renal replacement therapy (hemodialysis). She was started on replacement therapy. At 1-year follow-up, she was undergoing hemodialysis twice a week. A routine blood test showed: Hb 80 g/L. A routine urine analysis showed: PH 7.5 and protein positive (Pro2+). Her serum potassium level was 4.2 mmol/L, calcium 2.22 mmol/L, phosphorus 1.53 mmol/L, CO2CP 26.1 mmol/L, creatinine 619 μmol/L, and PTH 546 pg/ml.
Given the key role of the GDNF–RET interaction in kidney and urinary tract development, anomalies in these molecules are reasonable candidates for explaining a disorder such as MSK. However, mutations in RET proto-oncogene are also present in 97% of individuals with multiple endocrine neoplasia (MEN) 2A (MEN2A). In order to explore the possible genetic mechanisms, we analyzed the GDNF and RET genes in this patient. After obtaining informed consent from our patient, genomic DNA was extracted from peripheral blood leukocytes by standard phenol–chloroform procedures. All 20 exons and flanking splice sites of the GDNF and RET genes were amplified by polymerase chain reaction (PCR) with the primers listed in Table . Mutations were identified by direct sequencing of PCR products on an ABI 3730xl automated sequencer (Applied Biosystems, USA). Two RET polymorphisms were found in this patient, one was nonsynonymous c.2071G>A (G691S; rs1799939) located in exon 11 (Fig. ); the other was synonymous c.2712C>G (p.S904S; rs1800863) located in exon 15 (Fig. ). No mutation was found in the GDNF gene. Both parents of our patient are deceased. Further genetic testing of her husband, daughter, and sister were all negative for this polymorphism. |
pmc-6036694-1 | A 52-year-old man was first diagnosed with HIV in 1991. He was maintained on antiretroviral therapy with emtricitabine-tenofovir and raltegravir. The HIV viral load was undetectable (less than 20 copies/ml) and the CD4 count of 850 cells/uL at the time of presentation. In December 2016, he presented to the emergency department with chief complaint of diplopia. A magnetic resonance imaging (MRI) of the orbits revealed a mass in the left orbit with involvement of the optic nerve. He was referred to ophthalmology and underwent a lateral orbitotomy and removal of the orbital mass. Pathology showed metastatic small cell carcinoma. A Computed Tomography (CT) scan of the chest, abdomen and pelvis and a Positron Emission Tomography (PET) scans were negative for any intrathoracic mass; however, there were multiple liver lesions and a large pancreatic tail mass. Given these findings his final diagnosis was extrapulmonary high-grade small cell carcinoma of the pancreas. Next Generation Sequencing of his tumor showed an intermediate tumor mutation burden with 9 mutations/megabases and deleterious alterations in TP53, MLL3, MEN1, FAT1, CDKN2A, BCORL1, BCOR, ATRX and TSC2 genes. There is currently no approved targeted therapy for any of these mutations. He was started on chemotherapy with carboplatin and etoposide. He had a partial response (PR) after 2 cycles of chemotherapy. He had disease progression after 6 cycles of chemotherapy with carboplatin and etoposide. He was then started on chemotherapy with FOLFIRINOX (5-Fluorouracil, irinotecan, leucovorin and oxaliplatin) as second line therapy. He received four cycles but continued to have disease progression on imaging. He was then treated with carboplatin and paclitaxel but his disease continued to progress with clinical deterioration and significant abdominal pain. At that point, treatment with dual CPI therapy (nivolumab and ipilimumab) was pursued given the available data in refractory SCLC. Before the start of this therapy, his CD 4 count was 294 cells/uL with an undetectable HIV viral load (less than 20 copies/ml). He received Nivolumab 1 mg/Kg along with Ipilimumab 3 mg/kg every 3 weeks. After 2 doses of the combination, he developed acute kidney injury with creatinine of 4.2 mg/dl from a baseline of 1.0. The therapy was suspended and he was admitted to the hospital and a renal biopsy was performed which showed severe drug-induced acute interstitial nephritis (AIN). He was treated with high dose steroids; 500 mg of IV methylprednisolone for 3 days followed by a steroid taper. His renal function improved after 4 weeks with return of creatinine to baseline. He was then re-started on single agent Nivolumab at 1 mg/Kg and later ipilimumab at 1 mg/kg was added. The patient had significant clinical improvement soon after starting the dual CPI therapy with resolution of abdominal pain which previously required high-dose opioids. Repeat scans at 12 weeks (including MRI of the head and PET/CT scan) showed complete response (CR) as per PERCIST criteria with disappearance of all metabolically active lesions (Fig. ). Patient was continued on antiretroviral therapy. The HIV viral load was undetectable before starting the CPIs (< 20 copies/ml) and increased to a high of 175 copies/ml. At the same time his absolute CD4 count increased from 294 cells/uL before treatment to a high of 593 cells/uL. His CD8 count also followed the similar pattern. It was 111 cell/uL before starting treatment and reached a high of 247 cells/uL (Fig. ). The patient’s complete radiological response is still ongoing at the time of this report (24 weeks after the start of the dual CPI therapy). |
pmc-6036831-1 | A 21-year-old male with a history of Tetralogy of Fallot (TOF) repair (with Dacron Patch over a large ventricular septal defect (VSD)) was admitted with complaints of fever and weight loss for 2 months and left sided abdominal pain since 1 week. He was recently admitted with similar complaints to another hospital where he was found to have a right sided pneumonia and was being treated with intravenous ceftriaxone. Later, suspecting infective endocarditis, gentamicin was added but as the patient was still having persistent fevers of 40°C, he was referred to our hospital. On examination, the patient was of lean built with grade IV clubbing without cyanosis, and there were no peripheral stigmata of infective endocarditis. He had a loud pansystolic murmur on the left sternal edge and had tenderness on palpation of the left upper abdomen. His initial investigations showed a high white blood cell count and C-reactive protein (CRP) (). A chest X-ray showed left mid and right lower lung zone infiltrates, and an ultrasound of the upper abdomen showed an ill-defined splenic lesion without internal vascularity suggesting either an abscess or infarct. Three sets of blood cultures were subsequently negative. Echocardiogram showed vegetation on the VSD patch along with dehiscence, a large VSD, and moderate right ventricular outflow obstruction. A CT abdomen with contrast was done which showed multiple liver, splenic, and lung abscesses with infarcted left kidney and thrombus at the bifurcation of the aorta secondary to the septic embolic phenomenon. Cardiothoracic surgery consultation was sought, and the patient underwent a redo-sternotomy and removal of vegetations from right ventricular outflow tract site, removal of Dacron Patch, and complete repair of TOF. Postoperative echocardiogram did not show any residual VSD or vegetation, only mild left ventricular dysfunction and moderately reduced right ventricle function was seen. The vegetation removed from right outflow tract and Dacron Patch was sent for bacterial, mycobacterial, and mycology cultures. Chocolate, Sheep Blood (SBA), and MacConkey Agar plates were incubated at 37°C for aerobic culture and a SBA plate incubated anaerobically. For fungal culture, tissue was stabbed onto the surface of Sabouraud's dextrose (SDA), potato dextrose (PDA), and Mycosel agar plates incubated at 25°C and an additional SDA and SBA incubated at 37°C. The initial Gram stain and 10% potassium hydroxide (KOH) preparation revealed clusters of oval conidia and hyphae mistaken for yeast cells and pseudohyphae as well as septate hyphae. Patient was empirically started on intravenous amphotericin deoxycholate at 1 mg/kg (40 mg/dose), his fever gradually subsided, and the patient was discharged on amphotericin and oral voriconazole. Bacterial cultures remained negative, and a filamentous mould grew from the vegetation at 48 h that was finally identified phenotypically as Acremonium species (). It grew on all plates except MacConkey agar. Mycobacterial cultures remained negative. Susceptibilities were performed by E-test® (bioMerieux, France) on RPMI agar, and the isolate showed an MIC of >32 mcg/ml against amphotericin and 2 mcg/ml against voriconazole. After final identification amphotericin, deoxycholate was discontinued and oral voriconazole 200 mg twice daily was continued. However, treatment was discontinued at 3 months based on clinical improvement and due to inability of the patient to bear the cost of the treatment. After 2 months of stopping therapy, patient had recurrence of symptoms along with new vegetation on the pulmonary valve. Blood cultures were submitted in BD BACTEC™ aerobic and anaerobic bottles and incubated in the BD BACTEC 9240 system, as BD BACTEC MYCO/F Lytic system was not available. The bottles flagged positive after 3 days of incubation and two sets of blood cultures grew Acremonium species with similar antifungal susceptibilities (). The mould was identified on the colony and microscopic morphology. It was a hyaline mould appearing in 3 days on solid media, with cream coloured wrinkled surface and an off-white to tan reverse. By day 6, the colonies became slightly floccose. On microscopic examination, the hyphae were thin, hyaline, and septate, with short nonseptate conidiophores giving rise to elliptical conidia clinging to the philaides in wet masses of 10–20. Molecular confirmation could not be made; however, the isolate was phenotypically differentiated from Fusarium and Phialemonium species due to colony appearance, absence of macroconidia, and septations at the base of conidiophores.
Voriconazole was restarted and surgical excision of the pulmonary valve was performed two months later with growth of the similar fungus. The patient was continued on oral voriconazole for a period of one year with clinical improvement, improvement in inflammatory biomarkers, no residual vegetation on repeat echocardiogram, and serially negative blood cultures. |
pmc-6036836-1 | The proband is a 2-year and 7-month-old Mexican mestizo male (, individual III.2). He was referred at 5 months of age due to cholestatic syndrome characterized by jaundice and pale stools, which began as of the third week of life. He is the only child of a young, apparently healthy, and unrelated couple.
The pregnancy lasted 39 weeks and was complicated by maternal cholelithiasis at the fourth month. Antibiotics were indicated for repetitive urinary infections as of the fifth month. Delivery was carried out by cesarean section due to fetal distress. The newborn's weight was 2200 g (P<5), height was 48 cm (P<5), and Apgar score was 8/9.
The cognitive development of the patient is normal. At nine months of age, his weight was 6 kg (P<5), height was 64 cm (P<5), and head circumference was 44.5 cm (P25-50). He manifested generalized jaundice, dry skin, and an anterior fontanelle that had not yet closed. He had sparse eyebrows, a broad forehead, deep-set eyes, a triangular face, prominent ears, a heart murmur, hepatomegaly, and hypotrophic limbs (Figures and ). His liver function tests were abnormal () and an abdominal ultrasound analysis demonstrated generalized thickening of the biliary tract. The X-ray analysis showed a butterfly-like image in several dorsal vertebrae (). Right and left pulmonary hypoplasia were diagnosed by echocardiogram analysis. A magnetic resonance image analysis at the age of 1 year and 2 months displayed widening of the subarachnoid space and bilateral subarachnoid cysts in the temporal fossa. The optic nerve in both eyes was normal. At 2 years and 2 months of age, he developed xanthomata in both elbows and in his knuckles, and at 2 years and 4 months of age, posterior embryotoxon was diagnosed. A hepatic biopsy detected intracytoplasmic cholestasis and the absence of interlobular conducts.
JAG1 gene molecular analysis was conducted with institutional approval and after obtaining informed consent by second-generation sequencing in a private laboratory (Nanolab, Mexico City, Mexico) from blood samples, revealing the c.91dupG variant in a heterozygous state. This causes a frameshift mutation and a premature stop codon at amino acid number 72 of the JAGGED1 protein (). Capillary sequencing was carried out in our laboratory and confirmed the presence of the mutation, which was not found in the parents (). |
pmc-6036849-1 | We report a 27-year-old man known case of schizophrenia on clozapine who developed a painful erythema with tense skin blisters coalescing to form bullae filled with serous fluid on bilateral upper limbs few hours after excessive sunlight exposure (). The patient reported being exposed directly to the sun without sun protection when he was on vacation. He also developed miliaria rubra over bilateral axillae and back which is attributed to heat exposure (). The patient works in the military and denies previous episodes of sunburns before starting clozapine. He was managed with pain killers and supportive treatment and totally recovered 2 weeks after the episode. |
pmc-6036857-1 | A 47-year-old patient with an enormous uterine leiomyoma reaching beyond the navel and up to the costal arch was admitted. During the 14 years since its detection, because of the patient's extreme fear of an abdominal incision, the myoma was merely monitored and all suggested laparotomies thus far had been refused. At the moment of admission, the patient only agreed to a minimally invasive surgery. She was informed in detail about all risks, side effects, and alternatives as well as the potential risk for an emergency open abdominal surgery. Before surgery, we performed imaging diagnostics by means of computed tomography (CT) of the abdomen ().
When performing a hysterectomy of a very large uterus (>2500 g), the anatomical changes in the abdomen caused by the size of the uterus need to be taken into account. The large uterus divides the abdominal area, and only 3 narrow spaces are left to manipulate surgical instruments: between the left uterine wall and left abdominal wall, between the right uterine wall and right abdominal wall, and between the fundus uteri and liver and diaphragm. Successful surgery is only feasible when both instruments (forceps and coagulator or scissors) are in the same space simultaneously. Hence, we performed LASH with the “changeover technique” as described previously []. As such, we inserted 6 trocars including 3 on the left: one trocar in the lower, one in the middle, and one in the upper abdomen, allowing access to the left narrow space ().
The 3 trocars on the right side were placed in a mirror-like fashion. Surgery was initiated at the patient's left side, using the upper left trocar to introduce the camera () and the other two left-located trocars to introduce forceps and coagulator or scissors.
The patient was slightly tilted to the opposite side to facilitate visualization and preparation of structures. For uterus manipulation and movement, we used blunt forceps and palpation probes. After parameterization and resection of the uterine adnexa on the patient's left side, the plica vesicouterina was exposed and the bladder was pushed caudally.
Next, the surgeon and his team switched sides positioning to the right side of the patient, thus accessing the narrow space on the right side in between the right uterine wall and right abdominal wall (). Again, the patient was slightly tilted to the opposite side, enabling better exposure and preparation of organ structures.
The procedure continued analogically as on the left side. As was seen on the CT scan, multinodal, intraligamentary, and parametric myomas were found on the right side. Consequently, further exposure and protection of the ureter was done by visualizing followed by preparing or abscising the right adnexa, parametria, and blood vessels ().
The corpus uteri were removed from the abdominal cavity by power morcellation. The entire procedure lasted 4 hours and 53 minutes, of which surgery on the uterus took 2 hours and morcellation 2 hours and 53 minutes. No postoperative complications occurred, and the patient was taken to outpatient care 2 days after surgery. Before discharge, a vaginal and renal ultrasound did not reveal any conspicuous intra-abdominal findings. Because of the young age of our patient, a very slow growth of the leiomyoma (over 14 years), and negative Doppler test results before surgery, we assumed no presence of malignancy. Histological analysis of the removed specimen confirmed this assumption. Furthermore, to prevent morcellation-induced spreading of occult malignancy, the patient was alternately tilted from a head-down to head-up position, after which we extensively rinsed the abdominal cavity with Ringer's lactate solution, a routine postsurgical measure at our facility. The total weight of the uterus was 4065 g (Institute for Pathology PPO Berlin; Mona Tawfik, M.D.; 06.05.2014). |
pmc-6037330-1 | A 72-year-old Caucasian female with a history of stage II lymphocyte-rich classical Hodgkin lymphoma was treated with combination chemotherapy, including doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) with resultant remission. After seven years in remission, she developed persistent leukocytosis and anemia. Positron emission tomography-computed tomography (PET-CT) done for the evaluation of recurrence showed a new, enlarged left axilla, right paraesophageal, right precarinal, aortopulmonary, portohepatic, peripancreatic, and pericaval lymph nodes. The report of the biopsy of the enlarged right paratracheal lymph nodes via mediastinoscopy was consistent with HL. A diagnosis of recurrent stage III Hodgkin lymphoma was made. She was treated with gemcitabine, vinorelbine, and doxorubicin. Although imaging showed a response to therapy, chemotherapy was discontinued after four months of treatment due to significant functional decline and poor performance status. One year after the discontinuation of chemotherapy, she presented with a productive cough and persistent choking, preventing oral intake and marked weight loss. A physical examination revealed pallor, signs of dehydration, and bibasilar crackles. Due to a high suspicion of aspiration pneumonia, she was admitted and started on intravenous antibiotics. Computed tomography (CT) of the chest (Figure ) and CT soft tissue of the neck (Figure ) with intravenous (IV) contrast showed a 7.4 x 5.6 cm cavitary lesion involving the right upper lobe with irregular wall thickening and a fistulous communication with the esophagus. Bronchoscopy with an evaluation of the bilateral bronchial tree and all subsegments showed no evidence of a fistula (Figure ). Bronchoalveolar lavage and cytology were negative. Upper gastrointestinal endoscopy revealed a 4-cm long stricture in the proximal esophagus and features suggestive of a fistulous tract (Figure ). The histopathology of multiple biopsies obtained from the fistulous tract showed highly atypical cells with immunostaining positive for CD30 and PAX5 (Figure ), consistent with the involvement of esophageal tissue with Hodgkin lymphoma. Esophageal stent placement was planned but could not be performed due to severe thrombocytopenia. Percutaneous endoscopic gastrostomy was considered but the patient declined and eventually opted for hospice care. She died a month after the initial presentation. |
pmc-6037331-1 | A 77-year-old Caucasian female was admitted to the hospital for an evaluation of congestive heart failure. She gave a history of progressive peripheral edema over eight months, extending up to the knees bilaterally. She felt weak, exhausted, and had lost her appetite. She denied any orthopnoea or paroxysmal nocturnal dyspnea. She conceded to a long-standing history of hypertension, chronic atrial fibrillation, and hypothyroidism but denied any history of diabetes. Initial investigations, which included a urine analysis, revealed the presence of protein and no evidence of blood. Her admitting creatinine was 148 (micromol/l); serum albumin 15 g/L, and ACR 1025 mg/mmoL (normal <2.8 mg/mmoL). She had a normal white cell count and platelets and her hemoglobin was 115 g/L.
The cause of proteinuria was investigated further and the findings were - serum IgG: 18.4 g/L (5.5-17.24), IgA: 0.51 g/L (0.7-3.94), IgM: 0.53 g/L (0.44-2.47). Serum-free kappa light chains were elevated: 92.3 mg/L (3.3-19.4), and free lambda was 11.7 mg/L (5.7-26.3), kappa/lambda ratio 7.89 (0.26-1.65). b2-microglobulin was 6.8 mg/L (0.0-3.4) and on serum protein electrophoresis (SPEP), there was a presence of an M-spike of 17.2 g/L. Serum immunofixation revealed IgG kappa, and 24-hour urine protein was 9.09 g/day (normal <150 mg/d) with 1.97 g/d of monoclonal IgG kappa. She subsequently underwent a bone marrow biopsy, which revealed 5%-10% small clonal plasma cell population and bone marrow for flow cytometry showed the presence of clonal cell population. The presence of a monoclonal protein and renal impairment raised the possibility of AL amyloidosis, multiple myeloma, immunotactoid glomerulopathy, monoclonal immune deposition disease (light chain disease and heavy chain disease), proliferative glomerulonephritis (GN) with monoclonal deposits, and paraprotein-associated C3 GN.
She subsequently underwent a confirmatory kidney biopsy. On light microscopy, there were 21 glomeruli, four of which were sclerosed. Congo red positive staining was identified. Electron microscopy revealed haphazardly arranged thin fibrils in the mesangium and glomerular basement membrane (Figure ). Upon further interrogation of the biopsy with liquid chromatography tandem-mass spectrometry (LC MS/MS), a peptide profile consistent with AL (kappa) type amyloid deposit was identified. These changes were histologically consistent with a diagnosis of AL amyloidosis. Clinically, she was diagnosed with AL amyloidosis with nephrotic syndrome.
During the biopsy, it was noted that there was a solid mass in the left kidney. We investigated it further with computed tomography (CT) of the abdomen, which identified a 2.1 x 1.9 cm cystic lesion (23 Hounsfield units), but in the absence of contrast, the radiologist couldn’t make a further determination. Subsequent magnetic resonance imaging (MRI) revealed a 2.3 X 2.2 cm lesion in the upper pole of the left kidney suspicious of a tumor (Figure ) and the presence of a single retroperitoneal lymph node, which was biopsied and identified to be benign with no evidence of amyloid. The biopsy of the renal mass was consistent with grade 1 renal cell carcinoma. Her inpatient stay was further complicated by progressive ascites, which required repeated paracentesis. The presence of two concurrent, yet unrelated, diseases involving the kidney led to a therapeutic dilemma. After a discussion with the oncologist, it was decided that RCC was a slow-growing carcinoma and that surgical treatment could be offered upon stabilization of her underlying amyloidosis. She was initiated on 28-day cycles of 1.2 mg/m2 of bortezomib, 500 mg of cyclophosphamide, and 20 mg of dexamethasone. Unfortunately, despite the initiation of therapy, her proteinuria worsened, leading to repetitive drainage of her ascites and, later, she became neutropenic and septic. The family later withdrew active care, and she was transitioned to comfort measures and passed away peacefully three days later. |
pmc-6037334-1 | A 43-year-old Caucasian male with a medical history significant for intravenous (IV) drug use presented to the emergency department with restlessness, agitation, and profuse sweating. He reported no pain in the chest, neck, back, or abdomen. He denied a history of diabetes mellitus, hypertension, coronary artery disease, or connective tissue disease and was not taking any medication. He reported smoking one pack per day for the last 20 years. On presentation, he had a blood pressure of 120/32 mmHg; pulse of 90 beats per min; temperature of 100.1 F, and respiratory rate of 24 breaths/min. His oxygen saturation was 95% on two-liter of oxygen. He was fully oriented but found to be agitated and restless. A cardiovascular examination revealed grade 3/6 decrescendo diastolic murmur heard best on the left parasternal border on expiration. There were clear breath sounds bilaterally with no audible wheezes or crackles. The abdominal and neurological exams were benign.
His electrocardiogram showed nonspecific T wave changes in V1-V2 with sinus rhythm. His chest X-ray was unremarkable as well. The laboratory reports were normal except for creatinine of 2.5 mg/dl. Based on the history of IV drug abuse and auscultation consistent with a murmur and low-grade fever, the presumptive diagnosis of infective endocarditis was made. Blood cultures were drawn and empiric broad-spectrum antibiotics were started. A transthoracic echocardiogram (TTE) was ordered to look for possible valvular pathology/vegetation. TTE showed aortic root dilatation and aortic insufficiency along with the possibility of dissection in the ascending aorta. A computed tomography (CT) angiogram was ordered emergently that revealed aortic dissection involving the ascending aorta, arch (Figures -) with an aneurysm measuring up to 5.5 cm, extending into the descending aorta (Figures -). Emergent aortic root replacement along with ascending aorta and hemi-arch replacement were performed. The postoperative course was uneventful and he was discharged in a stable condition. |
pmc-6037337-1 | A 10-year-old male patient presented with a 13-mm well-demarcated, dome-shaped, dark red nodule on the left ala (Figure ). It had been present for eight months. During that time, it had increased in size and bled. The lesion received no prior treatment. The remainder of the physical exam was unremarkable.
A shave biopsy was performed, and histopathology revealed a diffuse infiltrate of spindle-shaped histiocytes in a storiform pattern (Figure ), few multinucleated giant cells, scattered lymphocytes, and eosinophils (Figure ). Immunohistochemical studies showed tumor cells positive for cluster of differentiation 68 (CD68) and the proliferation marker Ki-67 (Figure ). The lesion was negative for S-100 protein, anti-melanoma antibody (HMB45), protein Melan-A, and smooth muscle actin (SMA). These histologic features supported the diagnosis of SCXG. The nodule resolved spontaneously several months later. |
pmc-6037340-1 | A 68-year-old female presented with right upper quadrant abdominal pain and swelling. The pain was described as sharp, intermittent pain with 10/10 severity that started the day prior to admission. It was not associated with food, and she denied any fevers or chills. Her last bowel movement was the day prior to admission and was of normal caliber. She denied diarrhea, melena, hematochezia, and unintentional weight loss.
She had a past medical history of estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor 2 (HER2)-positive invasive ductal carcinoma of the left breast diagnosed in 1996. She received a modified left radical mastectomy and right simple mastectomy, adjuvant systemic chemotherapy with doxorubicin and cyclophosphamide, radiation to the left chest wall, and was started on Tamoxifen. Five years later, she developed metastatic disease to the lumbar spine (ER+/PR+) and received palliative radiation. She was started on zoledronic acid for bone metastasis and her endocrine therapy was switched to letrozole. At the time of admission, she was currently on letrozole and methadone for her back pain. She denied any tobacco use, alcohol, or recreational drug use. She has no family history of cancer.
Physical exam revealed stable vital signs and tenderness in the right upper quadrant with a large mass palpated. Laboratory data were unremarkable. Computed tomography of the abdomen and pelvis showed a colonic mass measuring 3.5 centimeters in the hepatic flexure, causing obstruction of ascending colon (Figure ).
A colonoscopy was performed and showed mild diverticulosis in the colon along with petechiae in the proximal to mid-transverse colon likely representing brief ischemia from possible volvulus, but no obvious mass was appreciated. Surgery was consulted due to worsening pain and abdominal swelling and a right hemicolectomy with ileocolic anastomosis was performed.
Pathology revealed metastatic carcinoma measuring 6.5 centimeters in greatest dimension at the junction of the cecum and ascending colon and involved the appendix (Figure ).
The tumor was transmural with involvement of serosal fat through the lamina propria and percolated between the colonic crypts with six out of 11 lymph nodes positive. Immunohistochemical staining was positive for cytokeratin 7, estrogen receptor (Figure ), and GATA-3 (Figure ), and negative for cytokeratin 20 (Figure ), caudal-related homeobox transcription factor - 2 (CDX-2) (Figure ), MELAN-A, CD 56, and synaptophysin. This was consistent with metastatic breast cancer. Further stains were positive for E-cadherin (Figure ), consistent with her history of invasive ductal carcinoma.
Given progression of metastatic invasive ductal carcinoma while on letrozole, she was transitioned to exemestane. |
pmc-6037625-1 | A 9-year-old male patient with recurrent cough was referred for an evaluation of left-lung pneumonia. The patient’s medical history revealed that he had been treated for this complaint several times. The chest X-ray showed an increased opacity in the left lower lobe. He was given amoxicillin/clavulanic acid, inhaled prednisolone, and salbutamol, which conferred only a partial reduction in symptoms. The chest computed tomography illustrated a mass of parenchymal origin in the lower lobe of the lung invading the left atrium (). Transthoracic needle biopsy was performed, and the histopathological examination demonstrated mixed inflammatory cells. An IMT was, therefore, considered.
The operative approach was considered for the patient to manage the invasive tumor. A left thoracotomy incision revealed the tumor invading the left atrium. Subsequently, left lower lobectomy was performed. Next, a portion of the tumor invading the left atrium was resected together with the intact atrial wall. Finally, the left atrium was repaired with pledgeted sutures. The procedure did not require cardiopulmonary bypass. After the operation was terminated, the histopathological examination of the mass confirmed the diagnosis of an IMT. The postoperative period was uneventful, and the patient was discharged on the sixth postoperative day. |
pmc-6037631-1 | A 53-year-old man was admitted to our cardiology department with exertional angina. He had hypertension and hyperlipidemia as cardiac risk factors. On physical examination, the patient’s vital signs were unremarkable and he had a New York Heart Association functional capacity of II. The respiratory and cardiovascular examinations were normal. The electrocardiogram showed nonspecific ST–T wave changes in leads V1–V6, and the laboratory work was within the normal range except for a low-density lipoprotein level of 170 mg/dL. The exercise stress test was nondiagnostic, and the myocardial perfusion scan revealed inducible ischemia in the anterior septum, mid, and basal portions of the anterior wall. Cardiac catheterization was performed and showed a giant left main coronary artery aneurysm ( and ). All of the left coronary system arose from the aneurysm. The patient had no history of Kawasaki disease in childhood, and nor was the type of the lesion compatible with Kawasaki disease. Multidetector computed tomography coronary angiography confirmed a huge left main coronary artery, measuring 33 × 28 mm in size ( and ). Because of the nonobstructive coronary artery disease in the other parts of the coronary system, the patient was managed with medical treatment. After 3 months of treatment, he referred to us again with exertional angina, which continued for more than 10 minutes. Coronary angiography illustrated no changes in his coronary system. After consultation with the cardiovascular surgery department, the patient was followed up with medical therapy. In the first hospitalization, medical therapy included acetylsalicylic acid (100 mg once a day), metoprolol (50 mg once a day), ramipril (5 mg once a day), isosorbide mononitrate (50 mg once a day), and trimetazidine HCL (35 mg twice a day). After the second hospitalization, warfarin treatment was added because of the giant aneurysm and slow flow. The patient’s first visit was in March 2013, and he was subsequently followed up until September 2016. |
pmc-6037633-1 | A 55-year-old man with a history of chest discomfort was referred to our clinic. The patient reported that he had angina of 6 months’ duration, but his angina had changed from the Canadian Cardiovascular Society (CCS) I-II to CCS III over the preceding 2 days. His physical examination, echocardiogram, and electrocardiogram reports were all normal. Following physical examination and initial tests, a diagnostic coronary arteriography was performed. Conventional angiography revealed that the left anterior descending coronary artery (LAD) had a critical proximal lesion and the left Cx (LCx) was normal and originated from the left main coronary artery (). Additionally, there was another nondominant Cx (RCx) arising from the proximal part of the right coronary artery with a significant diffuse stenosis (). There was also 35% stenosis in the distal left main coronary artery. An EBU guiding catheter was used to cannulate the left main ostium and the target lesion was passed using a 0.014″ guide wire. Thereafter, stenting was successfully performed with a 2.25 × 16 mm drug-eluting stent for the LAD lesion. The patient’s symptoms were relieved after the successful intervention on the LAD. He was discharged on the postoperative day in good condition. He came to our clinic for control after 2 weeks and reported that he had not experienced any angina since his discharge. |
pmc-6037656-1 | A 68-year-old woman underwent a left mastectomy and axillary lymph node dissection for left breast cancer (T4bN2M0). A pathological examination revealed an estrogen receptor-positive (ER-positive), progesterone receptor-positive (PgR-positive), and human epidermal growth factor receptor 2-positive (HER2-positive) invasive ductal carcinoma. For postoperative therapy, 6 cycles of 5-fluorouracil+epirubicin+cyclophosphamide and oral tamoxifen were given. A right renal cell carcinoma was incidentally noted on computed tomography imaging performed at follow-up 2 years later, and a right nephrectomy was performed. A further 4 years later, a bone biopsy was performed for a suspected bone metastasis found at the distal end of the left femur. This lesion was diagnosed as a metastasis from the primary breast cancer. Since the bone metastasis was localized within a single site, radiation therapy to this site and high-dose toremifene therapy were administered. Fifteen years after the initial surgery, she developed a left lower abdominal pain, anuria, and body swelling. Computed tomography imaging revealed an irregular thickening of the left bladder wall, left hydronephrosis, and hydroureter (Fig. ). As the ureteral orifice was occluded, an urgent left nephrostomy was immediately performed. A cystoscopy revealed a broad-based tumor extending from the left wall to the triangle of the bladder. The ureteral orifice could not be identified. The tumor was biopsied, and a histopathological examination revealed a proliferation of cells with eosinophilic cytoplasm and a rounded dentate macronucleus in the mucosal lamina propria (Fig. ). The immunostaining results revealed CD7 positivity, CD20 negativity, ER positivity, and HER2 positivity, confirming a diagnosis of bladder metastasis from breast cancer (Fig. ). High-dose toremifene was considered ineffective, and chemotherapy with eribulin mesylate was started. |
pmc-6037657-1 | A 54-year-old female patient presented with an abnormal shadow discovered on a routine chest X-ray. She had a history of smoking 4–5 cigarettes per month for 5 years but quit over 10 years ago. Her past medical history included a colorectal benign polyp resected by endoscopy. She did not have respiratory symptoms and laboratory findings were unremarkable. The serum levels of the tumor markers (carcinoembryonic antigen, squamous cell carcinoma antigen, and cytokeratin 19 fragment) were within normal limits. A chest radiograph showed a nodular shadow at the right middle lung field (Fig. a), and a computed tomography (CT) scan confirmed an 18-mm lobulated nodule at the middle lobe (Fig. b, c). An F18-fluoro-deoxy-glucose positron emission tomography/CT (FDG-PET/CT) scan did not indicate abnormal FDG uptake. Bronchoscopy showed the round, tan, solid endobronchial nodule reducing the lumen of the right subsegmental bronchus (B5a) (Fig. d). A bronchoscopic biopsy was performed, and the patient was diagnosed with an epithelial-myoepithelial carcinoma (EMC). Examination of otolaryngologist and magnetic resonance imaging (MRI) of the head revealed no salivary gland pathologies. A right pulmonary middle lobectomy was performed, along with hilar and mediastinal lymph node dissections.
The tumor was measuring 15 mm in diameter and had a white surface; it was well-circumscribed and was present along the bronchial wall (Fig. a). On histological examinations, the tumor was located in the submucosal layer of the bronchus, oppressing the adjacent bronchioles, and partly necrotic (Fig. b, c). The tumor consisted of two different components: the duct-forming epithelial cells and outer multilayered polygonal cells with clear cytoplasm (Fig. –). Duct-forming epithelial cells were positive for cytokeratin 7, while the outer cells were negative (Fig. a). The outer cells were positive for S100 protein, smooth muscle actin (SMA), p63, and cytokeratin 5/6 (Fig. –), suggesting myoepithelial phenotype. Neither vascular, lymphatic, nor neural invasion was observed, and the mitotic rate is rare. The Ki-67 labeling index was less than 5% (Fig. e). No mutation was found in the KRAS or EGFR gene. We finally diagnosed the patient with P-EMC. The patient is doing well without any sign of recurrence 3 years after surgery. |
pmc-6037711-1 | A 29-year-old male patient was admitted to the second affiliated hospital of Zhejiang University, Hangzhou, China, in August 2014 with complaints of toothache and fever for over 1 week. A diagnosis of acute monocytic leukemia was made and the patient received chemotherapy and antimicrobial treatment with meropenem and vancomycin. Exhibiting signs of a lung infection on September 6, 2014, he was given a combination of meropenem and isepamicin for 5 days before being discharged from the hospital. The patient was readmitted for a second round of chemotherapy on September 29 and developed a fever, but was discharged upon treatment with meropenem, isepamicin, and vancomycin for 2 weeks. Blood and sputum cultures remained negative during this period (Table ).
The patient received the third round of chemotherapy in November 2014. A week later, he developed a high fever (38.6 °C) and diarrhea and was again given a combination of meropenem and isepamicin. One Escherichia coli isolate, EC-1, and a Klebsiella pneumoniae strain, KP-Y1, both susceptible to carbapenems, were isolated from a diarrheal fecal sample (Table ). The treatment regimen was then changed to meropenem, isepamicin, and vancomycin, and maintained for 4 days. Caspofungin was subsequently added and a carbapenem-resistant K. pneumoniae (CRKP) strain, KP2, was isolated for the first time from a fecal sample (Fig. , Table ). The symptoms of fever and diarrhea persisted, and KP2-like strains remained detectable in fecal samples until early December of 2014. The patient was then given the fourth and fifth rounds of chemotherapy in December 2014 and February 2015, respectively. Symptoms were unremarkable except for intermittent fever and knee swelling. Prophylaxis with a combination of meropenem and isepamicin, followed by meropenem and vancomycin, continued for 3 weeks after each chemotherapy round. During this period, all culture findings remained normal.
The patient underwent hematopoietic stem cell transplantation in April 2015 and received prophylactic treatment with piperacillin-sulbactam. With fever and diarrhea developing soon afterwards, the treatment was reinforced by teicoplanin and posaconazole. A CRKP strain, KP3, was recoverable from a fecal sample. Diarrhea symptoms soon worsened, resulting in bloody stool and tenesmus. Meropenem treatment continued for 3 days before switching back to piperacillin-sulbactam and tigecycline. KP3 type strains remained detectable in stool, and a CRKP strain, designated as KP4, was isolated for the first time from blood in May 2015. The patient developed a high fever that reached 40 °C and was given high doses of tigecycline, isepamicin, and fosfomycin. Two more isolates, KP5 and KP6, were isolated from blood samples on the 18th and 24th of May 2015, respectively. A right liver lobe abscess was observed on May 29 when another fecal CRKP strain, designated KP7, was isolated (Fig. , Table ). Antibiotics including meropenem, tigecycline, amikacin, and voriconazole were provided but were ineffective in relieving symptoms. The patient then developed fever, diarrhea and pan-resistant sepsis. The hospital ran out of antibiotic choices and the patient was subsequently treated with tigecycline and caspofungin, which were supplemented with colistin purchased from overseas. Tigecycline was given to the patient with 100 mg delivered IV every 12 h from May 8, 2015, through June 3, 2015.
The combined use of colistin and multiple antibiotics intravenously and orally eventually eased the diarrheal symptom, but could not immediately clear CRKP from the bloodstream. Perianal pain, fever, and signs of liver abscess persisted in the following weeks. Colistin treatment continued until September 1, 2015, and then treatment was switched to polymyxin B. CRKP remained detectable in fecal samples and occasionally in blood until early September, when blood samples became sterile, indicating that colistin was eventually effective in eradicating CRKP strains (Fig. ). On the other hand, mcr-1-positive, colistin-resistant strains of E. coli, Morganella morganii, and Citrobacter freundii were consecutively isolated from fecal samples from July 13, 2015, onwards (strains EC2-7), indicating that colistin selected its own resistance during the treatment process (Fig. , Table ).
Seven months after the transplant, an ulcer was observed at the sacrococcygeal site. Meropenem and linezolid treatment was initiated, followed by colistin, fosfomycin (IV), and then polymyxin B (IV). Colistin treatment was started with polymyxin B with 1 million units IV every 8 h and then changed to colistin with the same dose from June 3, 2015 to November 30, 2015. During treatment, CRKP was detectable in sputum, and both CRKP and mcr-1-positive E. coli were recoverable from fecal samples in retrospective analysis. However, blood samples remained sterile. In early December of 2015, pneumonia and GI tract symptoms appeared, prompting intravenous and oral administration of polymyxin B and E. CRKP and mcr-1-positive E. coli strains continued to be detectable in fecal samples and CRKP strains could be recovered occasionally in sputum but not blood. The symptoms began to resolve a few days later and the patient was discharged on December 15, 2015, with carriage of CRKP and mcr-1-positive, colistin-resistant E. coli in stool. CRKP isolated from sputum and a fecal sample during this period were selected for further study (strains KP9 and KP10, respectively). Two mcr-1 positive E. coli isolates, which were recovered from fecal samples collected in November 2015 and designated EC-Y9 and EC-Y10, respectively, were also subjected to microbiological and genetic characterization (Fig , Table ).
The patient recovered gradually and remained healthy since being discharged from the hospital. To determine if mcr-1-positive Enterobacteriaceae and CRKP persisted in his GI tract, we performed fecal isolation for these organisms in October 2016. CRKP strains, but not mcr-1-positive Enterobacteriaceae, were recoverable from fecal samples. We designated these CRKP strains as KP12–KP17, respectively. Another seven follow-up procedures were performed, and similar results were obtained (Fig. , Table ). |
pmc-6037727-1 | The 61-year-old Hungarian woman was referred to the out-patient clinic of the Department of Dermatology and Allergology (Szeged; Hungary). Generalized erythroderma with mild infiltration and whitish fine scales were observed on her body (Figure A). The first onset of her skin symptoms occurred in childhood and she was under regular dermatological care for 28 years. Previous phototherapy and oral acitretin (25 mg/day) therapy were not effective, while methotrexate therapy was aborted due to serious side effects. Based on clinical and histological findings (Figure B) atypical PRP phenotype was diagnosed. Since the patient developed symptoms in early childhood turning into a chronic course with no sustained clearance, our patient was classified as a PRP type V patient. The patient was not aware of any family members affected by PRP; however, both her daughter and a grandchild had psoriasis and were also under regular dermatological care.
The association between CARD14 gene variants and PRP has recently been reported (, ). Direct sequencing of the CARD14 coding regions of the PRP patient revealed three heterozygous missense variants: c.1641G/C p.Arg547Ser (rs2066964) in exon 14, c.2044C/T and p.Arg682Trp (rs117918077) in exon 17, and c.2458C/T p.Arg820Trp (rs11652075) in exon 20. The patient carried a splice site variant in homozygous form c.676-6G/A (rs28674001), located six nucleotides away from the 5′ end of exon 7. According to the results of analysis with pathogenicity prediction tools, the p.Arg682Trp missense variant is expected to be pathogenic, whereas the other three variants are expected to be benign.
Previous studies suggested that CARD14 variants contribute to the development of PRP by increasing the activity of the NFκB signaling pathway (, ). To investigate whether the detected CARD14 variants in the investigated PRP patient increase NFκB activity, immunofluorescent (IF) staining was performed on paraffin-embedded samples of lesional and non-involved skin from the PRP patient and from healthy individuals (Figure ). IF staining of the NFκB p65 subunit demonstrated that p65-positive nuclei (in 31.68% of epidermal cells) were present in the suprabasal epidermis of the lesional PRP skin, but were not present in the non-involved skin of the PRP patient or in the healthy skin samples. Moreover, analysis of fluorescence intensity revealed a stronger staining in PRP samples compared to healthy samples (Figure S2A in Supplementary Material). As an additional control group, patients with moderate-to-severe psoriasis vulgaris (PASI 22.4 and PASI 4.1) were also enrolled into the study, but p65-positive nuclei were not found either in lesional or non-lesional samples from psoriatic patients (Figures S4 and S5 in Supplementary Material). Among all examined sections, p65 appeared in the nuclei in the epidermis exclusively of the lesional skin from the PRP patient.
To further confirm increased NFκB activity in keratinocytes from the PRP patient, an NFκB-luciferase reporter assay was performed. The NFκB activity was significantly higher in the cultured keratinocytes of the PRP patient compared to healthy keratinocytes (Figure G).
In parallel, IF staining of the NFκB p65 subunit was also performed on peripheral blood mononuclear cells (PBMCs) derived from the PRP patient and healthy individuals (Figure S3 in Supplementary Material). Staining was more intense in the PBMCs of the PRP patient compared to cells of healthy individuals (Figure S2B in Supplementary Material). These results demonstrated increased NFκB activity in various cell types derived from the PRP patient.
To determine whether the increased NFκB activation had any functional consequence in the PRP patient, expression and secretion of NFκB-induced cytokines were determined.
Keratinocytes of the PRP patient showed a tendency for higher basal mRNA expression and significantly higher secretion of interleukin (IL)-1α and IL-1β compared to healthy NHEKs (Figure ). Upon LPS treatment in healthy keratinocytes mRNA expression induction was only detected for IL-1α (Figure A).
We found a significantly higher basal mRNA expression of IL-1α, IL-1β, IL-6, IL-8, and tumor necrosis factor (TNF)-α in PBMCs of the PRP patient compared to healthy control (Figure ). In healthy PBMCs, LPS treatment significantly induced the mRNA expression of IL-1β and IL-8 compared to the untreated control samples, while in the PRP PBMCs significant induction (p < 0.001) was observed upon LPS treatment for all examined cytokines. The induction of IL-1α, IL-6, IL-8, and TNF-α was significantly (p < 0.05) higher in PRP cells compared to healthy control. The unstimulated PRP PBMCs secreted IL-8, while the secretion of the other cytokines was detected only after LPS treatment. IL-1α, IL-1β, and TNF-α were secreted at significantly higher levels in PRP cells compared to the PBMCs of healthy controls (Figure ).
No significant difference was found between the basal CARD14 mRNA expression of healthy and PRP keratinocytes, but in healthy keratinocytes LPS treatment decreased CARD14 expression significantly, while CARD14 levels in PRP keratinocytes were not affected (Figure E). |