A 28 - year - old African American woman presented to the R Adams Cowley Shock Trauma Center in Baltimore , Maryland , for evaluation of right - sided paralysis and left - sided paresthesias after being found down in her home between her nightstand and her bed . The symptoms began after eating at a local restaurant chain , consuming alcohol , and smoking one cigarette dipped in liquid phencyclidine ( PCP ) . She was asymptomatic that night and went to bed without complications . She was found down next to the bed the following morning unable to move . The patient denied any physical trauma other than falling from the bed . She had no history of seizures , sickle cell anemia , fever , urinary or bowel incontinence , or pain . The patient had a history of viral meningitis without residual deficits eight years prior , gastric bypass surgery , and cholecystectomy , and she was treated for a presumed urinary tract infection five days prior to admission at an outside institution . Her triage vital signs were as follows : temperature 36.8 ° C , blood pressure 155/100 mm Hg , heart rate 90 beats per minute , respiratory rate 16 breaths per minute , and an oxygen saturation of 95 % on room air . On physical exam , the patient was alert and oriented to person , place , and time . Her cranial nerves II – XII were grossly intact . She had 2/5 strength in her right lower extremity , 3/5 in her left lower extremity , and 4/5 bilaterally in her upper extremities . Her sensation was intact . She opened her eyes spontaneously , her verbal response was oriented and appropriate , and she obeyed commands , resulting in a Glasgow Coma Scale score of 15 . Pupils were equal , round , and reactive to light . She had tenderness to palpation over the cervical , thoracic , and lumbar spine without obvious external signs of trauma . Her anal sphincter tone was intact . Multiple laboratory abnormalities were noted on admission , including hyperlactemia , elevated liver function tests , and electrolyte derangements . Her complete blood count and remainder of her complete metabolic panel were within normal limits . These values are summarized along with reference ranges in Table 1 . Her toxicology screen was positive for PCP . Blood cultures drawn on admission grew the aerobic bacteria Salmonella enterica serotype 4,12 : i , and then repeat cultures drawn two days later grew group B streptococcus ( aerobic and non - aerobic ) . Subsequent blood cultures were negative , as were stool cultures . Metronidazole and vancomycin therapy was initiated empirically , with transition to ceftriaxone upon learning of the culture results . In addition , she had a detailed autoimmunity workup including anti - nuclear antibody ( ANA ) and HLA - B27 , all of which returned within normal limits . Admission computerized tomography scan of her cervical , thoracic , and lumbar spine revealed no spinal fractures . Magnetic resonance imaging ( MRI ) revealed no acute intracranial abnormalities , but enhancement within the central aspect of C3 – T2 suggested spinal cord edema , disc protrusions at C3–4 , C4–5 , and C5–6 , and bilateral posterior neck muscle edema . A lumbar puncture was not initially performed upon patient admission due to concerns about raised intracranial pressure from suspected trauma . Four hours after presentation , her strength had progressively deteriorated to 1/5 in all extremities . She lost proprioception in her toes bilaterally and developed absent anal sphincter tone . The patient was admitted to the neurotrauma intensive care unit with further deterioration ultimately requiring endotracheal intubation due to respiratory failure . Upon her positive blood culture results , lumbar puncture was performed , which demonstrated a pattern consistent with ATM , the results of which are summarized in Table 2 . An extensive workup for infectious etiologies was also completed , with the results summarized in Table 3 . Plasma exchange therapy was initiated with a suspected diagnosis of ATM . Despite maximal medical therapy , the patient remains quadriplegic , is experiencing neurologic pain , received a tracheostomy due to prolonged respiratory failure , and required a feeding jejunostomy tube placement at the time of this report .


A 60 - year - old male patient had previously undergone a radical resection of a T2bN0M0G3 Stage III retroperitoneal sarcoma , including right nephrectomy and cholecystectomy , in 2002 . Pathologic evaluation demonstrated a 25 cm high - grade liposarcoma with different histologic components , including myxoid , round cell , well - differentiated , sclerosing and pleomorphic patterns . Surgical margins were negative as the tumor did not invade the kidney , and since the renal vein and ureter were free of tumor as well . The patient did not undergo any additional therapy . Surveillance magnetic resonance imaging in 2007 revealed an isolated tumor recurrence in the retroperitoneum . An exploratory celiotomy revealed tumor involvement of the duodenum , head of the pancreas , superior mesenteric vein , vena cava and left renal vein with severe adhesive changes , leading to the intraoperative assessment of unresectable disease . Subsequently , the patient underwent stereotactic body radiation to the retroperitoneal tumor in five fractions for a total dose of 30 Gy , without complication . Postradiation imaging revealed near complete resolution of the retroperitoneal mass . One year later , the patient presented with weight loss , postprandial abdominal pain , nausea and vomiting . Cross - sectional imaging revealed a 7 cm mass with involvement of the third portion of the duodenum ( Figure 1 ) . However , there was no obvious involvement of the pancreatic head or the other structures that were noted to be involved during the previous laparotomy . We elected to perform another resection attempt , including a possible pancreatoduodenectomy or retroperitoneal vascular resection if necessary . At exploration , the residual tumor was an entirely intraduodenal , pedunculated mass at the posterior duodenal wall distal to the ampulla that filled the entire duodenal lumen . The patient underwent a duodenotomy and stalk transection of the polypoid mass , followed by partial duodenal resection with hand - sewn duodenojejunal anastomosis ( Figure 2 ) . Surgical pathology examination revealed a 9.5 cm recurrent high - grade liposarcoma with polypoid intraluminal growth containing myxoid , round cell , well - differentiated , sclerosing and focally pleomorphic areas . Surgical margins were negative as the tumor came within 0.1 cm of the stalk margin and there was no evidence of additional neoplastic components within the remaining resected duodenum . The postoperative course was uncomplicated , and the patient has demonstrated no recurrence up to 30 months from this resection .


A 20 - year - old previously healthy Bengali man presented to our hospital with anuria and features of uremic encephalopathy . Ten days prior to this presentation , he had experienced severe upper abdominal pain and vomiting , and he had been treated in a primary care facility for having a case of acute pancreatitis . His initial symptoms improved ; however , he gradually became anuric and disoriented . Then he was transferred to our hospital for further management . At presentation to our hospital , he was severely agitated , restless , and disoriented . He was tachypneic with acidotic breath . Mild pedal edema was present ; however , his jugular venous pressure was not raised . His pulse was 112 beats / minute , his blood pressure was 140/90 mmHg , and his body temperature was 98 ° F . Signs of meningeal irritation were absent , and his plantar response was bilaterally extensor . His fundus could not be evaluated , and examination of his other systems was unremarkable . His laboratory parameters showed features of renal dysfunction ( serum creatinine 13 mg / dl , serum urea 293 mg / dl ) , raised pancreatic enzymes ( serum amylase 249 U / L [ reference up to 100 U / L ] , serum lipase 227 U / L [ reference 13–60 U / L ) , normal liver function tests ( serum bilirubin 0.9 mg / dl , alanine aminotransferase 38 U / L , aspartate aminotransferase 35 U / L , alkaline phosphatase 122 U / L , serum albumin 37 g / L ) , normal potassium ( 5.1 mmol / L ) , normal bicarbonate ( 19 mmol / L ) , and normal triglycerides ( 173 mg / dl ) . His serological markers , including antinuclear antibodies , cytoplasmic antineutrophil cytoplasmic antibodies , perinuclear antineutrophil cytoplasmic antibodies , C3 , and C4 , were within normal limits . An ultrasonogram of his whole abdomen was unremarkable , but non - contrast - enhanced computed tomography ( CT ) findings were suggestive of acute pancreatitis ( Fig.1 ) . His kidneys were unremarkable , however . The patient was managed as having a case of AKI and acute pancreatitis . Urgent hemodialysis was initiated . After he had received two sessions of hemodialysis , his level of consciousness improved , but he complained of profound visual loss . An assessment revealed only perception of light . A funduscopic examination showed retinal whitening and extensive cotton wool exudates as well as Purtscher ’s flecken ( Fig.2 ) compatible with Purtscher ’s retinopathy . High - dose parenteral methylprednisolone ( 1 g intravenously once daily for 3 days ) was administered . For evaluation of renal dysfunction , a renal biopsy was done ; the histopathological findings were compatible with renal cortical necrosis ( Fig.3 ) . The patient denied substance abuse or alcohol ingestion . He did not consume any nephrotoxic drugs or herbal products in the recent past . No history suggesting connective tissue diseases was available , nor did he have a history of hypotension throughout the course of his current illness . The patient was finally diagnosed with acute pancreatitis complicated with renal cortical necrosis leading to AKI and Purtscher ’s retinopathy leading to complete bilateral blindness . He died 16 months after his initial presentation as a result of a recurrent attack of acute pancreatitis . Before that , he had been undergoing maintenance hemodialysis ; however , his renal function did not recover , though his vision was improved to finger - counting at 2 feet .


We report the case of a previously healthy 28 - year - old woman , Afro - Brazilian , in her first pregnancy . Our patient had no medical records until the 36th pregnancy week and reported allergy to diclofenac . She presented to our emergency department with an acute onset of abdominal pain , jaundice , nausea and vomiting , with no signs of encephalopathy . Her arterial blood pressure was 110/60mmHg , heart rate was 98bpm , axillary temperature was 35 ° C , she was severely dehydrated and with decreased peripheral perfusion . The laboratory examinations revealed hemoglobin 12.3g / dL , leukocytes 13 × 109 / mL , platelets 97 × 103 / mm3 , international nationalized ratio ( INR ) 6.9 , fibrinogen 98mg / dL , total bilirubin 14.2mg / dL , serum creatinine 3.4mg / dL , serum aspartate aminotransferase ( AST ) 306U / L , serum alanine aminotransferase ( ALP ) 302U / L , arterial bicarbonate 11mEq / L , arterial pH 7.21 , blood glucose 65mg / dL and ionic calcium 1.02mmol / L. An abdominal ultrasound depicted fatty infiltration of the liver and confirmed fetal viability . Our patient received an initial fluid load with crystalloids . The calcium , glucose and hypothermia were reversed . The diagnosis of AFLP was confirmed following the Swansea ’s criteria [ 9 ] . Therefore , a cesarean section was indicated . A thromboelastometry ( ROTEM ® , Pentapharm Co. , Munich , Germany ) was performed at the beginning of the surgery . The thromboelastometry analysis showed an intense kinetic and structural hypocoagulable state ( Fig.1 and Table 1 ) . The FIBTEM revealed an impairment in fibrinogen function quality while the EXTEM depicted a coagulation factor deficiency ( Fig.1a - c and Table 1 ) . Based on , respectively , FIBTEM maximum clot firmness ( MCF ) ( 0 mm ; Table 1 ) and EXTEM clotting time ( CT ) ( 228s ; Table 1 ) , 4.0 g of fibrinogen concentrate ( Haemocomplettan ® P , CSL Behring , Marburg , Germany ) and 1000UI of prothrombin complex concentrate ( Beriplex ® P / N 500UI , CSL Behring , Marburg , Germany ) were administered at the beginning of the cesarean section . The fluid input ( crystalloids ) and output during the caesarian section were , respectively , 2000mL and 200mL. The cesarean section succeeded with no major bleeding after the hemostatic therapy . Additional hemocomponent transfusion , such as fresh frozen plasma ( FFP ) , cryoprecipitate , platelets or blood concentrates , was not necessary . A second thromboelastometry analysis was performed at the end of surgery ( Fig.1d - e and Table 1 ) , showing a mild hypocoagulation state . The patient was admitted to the intensive care unit ( ICU ) and remained stable , with no bleeding during the recovery phase . She was discharged from the ICU 3 days after admission and then 3 days later she was discharged from the hospital .


We present the case of a 66 - year - old female patient with no significant past medical history presenting with chest pain that was treated with antacids by her primary care physician . The next day she presented to the emergency room , where an electrocardiogram revealed posterolateral ST elevation myocardial infarction . The patient was immediately taken to the catheterization lab . A successful percutaneous intervention was performed on an acutely occluded large ramus intermedius vessel that was supplying a large portion of the lateral wall ( Figure 1 ) . The patient did well initially , but several hours later she suddenly went into cardiogenic shock . An intra - aortic balloon pump was placed , and an echocardiogram demonstrated that she had severe mitral regurgitation from a ruptured papillary muscle ( Figure 2 ) . The patient , on maximal support , deteriorated rapidly with progressive acidosis , oliguria , and florid pulmonary edema . In spite of the very high risk , we decided to intervene surgically . Once in the operating room , transesophageal echocardiography confirmed severe mitral regurgitation with a ruptured anterolateral papillary muscle and a small left atrium . Shortly after the patient was put on cardiopulmonary bypass , the acidosis resolved and she began to make urine . Excellent visualization of the mitral valve was obtained using a vertical transseptal approach through the right atrium . The valve was carefully inspected and the necrotic ruptured anterolateral papillary muscle was seen ( Figure 3 ) . There was a large chunk of muscle attached to the A1 chordal apparatus . The anterior leaflet of the mitral valve was excised while keeping the posterior leaflet intact . A 27 - mm St Jude Epic bioprosthesis was inserted ( Figure 4 ) . She was placed on extracorporeal membrane oxygenation that was successfully weaned 3 days later . Following this , she made a remarkable early recovery . One month later , just prior to discharge , she expired after developing pneumonia that progressed to sepsis with multiorgan failure .


The patient is an 18 - year - old Han female admitted to Peking Union Medical College Hospital due to adrenal crisis triggered by pneumonia . She has developed recurrent respiratory infections since age 5 , and failed to respond to multiple hepatitis B virus ( HBV ) vaccinations . Reduced serum cortisol and ACTH levels were discovered at 16 when glucocorticoid replacement was initiated . In addition , hair loss started from age 4 , and absence of pubic and axillary hair was noticed after development of regular menstruation . Her history includes nephrotic syndrome , which was confirmed to be minimal change nephropathy by renal biopsy . Physical examination at admission revealed alopecia totalis , oral candidiasis , hypohidrosis , and trachyonychia . Facial or dental abnormalities was not noted . She is the only child in her family . Symptom and signs of the above disorders were not identified among her nonconsanguineous parents . Initial immunologic tests revealed remarkable panhypogammaglobulinemia and reduced cell counts of B cells , T cells , and natural killer ( NK ) cells ( Table (Table1).1 ) . CD4 / CD8 ratio , as well as expression levels of various T - cell activation markers were in normal range , except increased proportion of CD8+/HLA - DR+ subset . Antinuclear antibodies ( ANA ) and antineutrophil cytoplasmic antibodies ( ANCA ) were negative . When regular hydrocortisone replacement was suspended , her 8:00 am serum cortisol was measured at 0.93 μg / dL , with ACTH < 5.00 pg / mL. Serum levels of other anterior pituitary hormones , as well as serum and urine osmolality were within reference range . Both antiperoxidase antibody and antithyroglobulin antibody were negative . She had positive antiprotein tyrosine phosphatase antibody , with fasting blood glucose at 5.9 mmol / L. Magnetic resonance imaging with contrast suggested a normal pituitary . She was diagnosed with CVID , isolated ACTH deficiency , and ectodermal dysplasia . Symptoms of fever , cough , and vomiting cleared with antibiotics and stress - dose hydrocortisone treatment . Intravenous immunoglobulin ( IVIG ) therapy was suggested but denied by the parents . At follow - up 1 year after her discharge , she reported no infection events during the previous year as she stayed at home and avoided outdoor activities . Her glucocorticoid replacement was withdrawn 4 months after discharge , and she has only received traditional Chinese medicine ever since . Lymphocyte subsets test done at the follow - up documented increased levels of B cells and T cells , while NK cell count remained below normal limit . To assess her NK - cell activity , a flow cytometric procedure was done following previously described method.[10,11 ] Effector to target cell ratio was set at 10:1 . Less apoptosis of target cell line ( 12.43 % , reference range 15.11–26.91 % ) was observed when cocultured with patient 's peripheral blood mononuclear cells ( PBMC ) , indicating a deficient NK - cell cytotoxicity .


This young boy is the first child of healthy non - consanguineous , white British parents . He was born at term following an uneventful pregnancy weighing 3132 g ( 9th–25th centile ) . Early development was thought to be normal . At approximately 6 months of age he developed tonic upward eye deviation associated with flexion of his arms and neck consistent with infantile spasms . An electroencephalograph ( EEG ) at this time was grossly abnormal ( hypsarrhythmia ) and strongly supported a diagnosis of West Syndrome . Cranial MRI was reported as normal . Prednisolone was prescribed and treated the seizures effectively . Steroids were weaned over 6 weeks and he remained seizure free for a further 6 months . By the age of 1 year , it was apparent that his early developmental progress was not being maintained and that he was functioning at the 6–8 month developmental stage . Seizures returned shortly after his first birthday and were prolonged , frequent and on occasion focal , involving his right arm , leg and right side of face . Clonazepam briefly improved seizure frequency , but subsequently his epilepsy has proved refractory to various combinations of anticonvulsant therapy . Prolonged seizures of more than 60 min have been associated with a stepwise regression in his neurodevelopment . Seizure semiology is now predominantly one of epilepsia partialis continua involving the right side of his face , right arm and right leg . The development of focal seizures and the progressive nature of the condition prompted a second cranial MRI at the age of 2 years 6 months . By contrast with the previous scan , this MRI revealed symmetrical subcortical white matter lesions ( Fig.1A ) with thinning of the anterior and genu of the corpus callosum ( Fig.1B ) . On examination , the patient had small , round , anteriorly rotated ears and a broad nasal root . He demonstrated no visual awareness but startled to loud noise . Tone was increased in all 4 limbs with internal rotation of both legs at the hips . Reflexes were pathologically brisk . Brief myoclonic jerks were evident throughout the examination .


A 44 - year - old man had been diagnosed with LCNEC of the thymus with bone metastasis at a different hospital 3 years previously . The serum levels of ACTH and cortisol were elevated , but brain magnetic resonance imaging revealed that his pituitary gland was normal . However , immunohistochemical staining of the thymic tumor tissue revealed partial anti - ACTH antibody positivity . The tumor was therefore determined to be ectopically producing ACTH . The patient underwent chemotherapy with cisplatin and irinotecan , but the continuation of chemotherapy was problematic due to a lack of tolerance . Treatment for hypertension and diabetes caused by the ectopic ACTH syndrome was initiated , as was treatment with zoledronic acid for bone metastasis . The patient was referred to the Endocrine Center at our hospital after 3 months due to a sensation of weakness and the worsening of his hyperglycemia . A physical examination revealed hypertension , a subcutaneous mass that was palpable in the anterior chest , and redness and swelling of the left lower leg without fever , respiratory symptoms , hypoxemia , or abnormal chest sounds . The oxygen saturation by pulse oximeter in room air and was 97 % and his respiratory rate was 13 breaths per minute . Among the laboratory findings , the white blood cell and neutrophil counts , and blood sugar , triglyceride , and low - density lipoprotein cholesterol levels were found to be elevated , and the patient 's potassium level was decreased to 2.8 mEq / L. The patient 's lactate dehydrogenase ( 468 IU / L ) , beta - D glucan ( 370.8 pg / mL ) , ACTH ( 354.1 pg / mL ) , and cortisol ( 49.1 μg / dL ) levels were also elevated . The patient 's serum was negative for aspergillus , candida , and cryptococcus antigens ; a cytomegalovirus pp65 antigenemia test also yielded a negative result . These data suggested that the increased production of ACTH by the tumor , along with the progression of the disease , had promoted the elevation of the patient 's serum levels of ACTH and cortisol . Computed tomography ( CT ) revealed the progression of the disease in the both thymic and metastatic bone lesions , along with the multiple ground - glass opacities in both lungs ( Fig.1 , 2 ) . The patient underwent bronchoalveolar lavage ( BAL ) in the right B5 segment with 150 mL saline , and 62.7 % of the BAL fluid was recovered . The cell count in the BAL fluid was 0.61×105 per mL , and the cell differentiation in the BAL fluid was 17.0 % macrophages , 81.0 % lymphocytes , and 2.0 % neutrophils , with a CD 4/8 ratio of 0.57 . Gram , Ziehl - Neelsen , and Grocott staining were all negative . A polymerase chain reaction revealed that the patient 's BAL fluid was positive for Pneumocystis jirovecii . PCP was diagnosed based on the above results . The patient was treated with sulfamethoxazole / trimethoprim ( 12 g / day ) for 3 weeks . Anti - cortisol therapy with metyrapone was also initiated at the same time with a small dose to slowly correct the hypercortisolemia . Intensive insulin therapy , potassium supplementation were initiated , while cefazolin was administered to treat cellulitis of the left lower leg . The patient 's PCP fully improved without the exacerbation of his respiratory status . Thereafter , we continued to administer sulfamethoxazole / trimethoprim at a prophylactic dose . The metyrapone dosage was gradually increased from 250 mg / day , and the blood cortisol value was observed to decline slowly ( Fig.3 ) . In order to prevent hypocortisolemia , the patient was temporarily treated with hydrocortisone . After an improvement of his left lower leg cellulitis , PCP , and hyperglycemia , the patient underwent laminectomy and radiation therapy for spinal cord compression , which had been caused by a metastatic spinal tumor . Octreotide therapy was initiated , and he was discharged on the 68th day of hospitalization .


A 21 - year - old man presented to the emergency department with a rash on his arms and neck ( figure 1 ) . It had developed over the course of 36 h from discrete ‘ pin prick ’ areas of erythema into a maculopapular rash with vesicles ( figure 2 ) . It covered his forearms from the dorsal aspect of the hands to the flexor surface of the elbows . It was also beginning to develop around his neck ( figure 3 ) . He described the rash as itchy and painful , and was feeling ‘ under the weather ’ . The patient had no previous dermatological or other medical history of note . He took no medication . He worked in a supermarket handling food items but there was no history of occupational chemical exposure . He had not recently had any change in washing powder or personal hygiene products . Physiological observations were entirely normal and systemic examination unremarkable . Closer examination of the rash revealed that it was present only on skin exposed to the sun when wearing a short - sleeved shirt ; the most striking feature was the ‘ V ’ shape on the patient 's chest in keeping with the neck line of a polo shirt . On further questioning the patient described a period of time 48 h prior to presentation ( and 12 h prior to the development of the rash ) spent strimming the grass under the hedges in his garden . Blood tests including full blood count , urea and electrolytes , liver function test and C - reactive protein were all within normal reference ranges . The list of potential differential diagnoses for a maculopapular rash is long , but given the distribution of the rash and the history of plant material exposure a diagnosis of phytophotodermatitis was made . The patient was discharged with chlorphenamine , simple analgesia and the advice to wear a long - sleeved t - shirt and sunscreen when gardening in the future . The rash resolved over a period of 3 weeks . It remained pruritic throughout healing but the associated pain settled over a few days . Residual scarring is beginning to fade 2 months after presentation .


A 64 year old female known for HHT is referred to our clinic for recurrent epistaxis for nearly 50 years . She has had recurrent symptoms since the age of 16 when her condition was diagnosed . All three of her siblings also were diagnosed with HHT and her mother passed away from an intracranial hemorrhage . Eight years prior to presentation she had undergone a left - sided septodermoplasty via a lateral rhinotomy approach . This operation had significantly reduced the frequency of her symptoms and for several years her epistaxis was under control with the use of low dose thalidomide . However , she was referred to our clinic due to increased epistaxis severity and frequency over the prior 12 months necessitating more frequent transfusions . At the time of consultation the patient was concerned about daily severe left sided epistaxis despite several months use of topical bevacizumab and oral tranexamic acid . She required intravenous iron and blood transfusions every two months . Her baseline hemoglobin at the time of our consultation was 75 g / L ( normal = 120 – 160 g / L ) . Her HHT epistaxis severity score [ 17 ] was severe ( normalized score 9.49 ) . On examination , she had multiple telangiectasia on her fingers , face , lips and palate . Her endoscopic examination revealed bilateral telangiectasia along the nasal septum . There was extensive crusting along the entire left nasal cavity with which any manipulation resulted in immediate profuse epistaxis . Given the severity of the patient ’s epistaxis despite medical therapy , she was offered endoscopic left - sided septodermoplasty . The surgical goals were to improve her quality of life by reducing the number and severity of epistaxis episodes while diminishing the need for blood transfusions . The patient was content with the treatment plan and agreed to undergo surgical intervention . The endoscopic procedure was performed under general anesthesia with endotracheal intubation . The nasal cavity was prepared by inserting pledgets soaked in topical adrenaline ( 1:1000 ) placed in both nostrils for decongestion . Using a zero degree endoscope the residual STSG and mucosa of the left septum was dissected in a supraperichondrial plane that resulted in the expected significant diffuse hemorrhage . Immediate hemostasis was attained using a topical gelatin - thrombin matrix , Floseal ( FloSeal Hemostatic Matrix ; Baxter Healthcare Corporation , Deerfield , IL , USA ) ( Fig.1 ) . The mucosal defect ( Fig.2 ) measured approximately 3 cm in anterior - posterior dimension . A 4 x 2 cm split thickness skin graft was harvested from the right thigh , pie - crusted with a 15 blade and then placed endoscopically along the length of the septal defect . As seen in Fig.3 , the graft was placed with an overlap of the mucosa of the nasal floor and the residual superior septal mucosa . Finally , 2 mL of fibrin sealant ( TISSEEL fibrin sealant , Baxter Healthcare Corporation , Deerfield , IL , USA ) was then applied first to the edges then central portion of the STSG ( Fig.4 ) . Packing was not used post - operatively and the patient was discharged home on the same day of surgery . Clinical follow - up two weeks after surgery ( Fig.5 ) showed that the entire graft had taken and the left - sided epistaxis had dramatically diminished . The patient was very content with the results of the procedure . At 6 months follow - up , her baseline hemoglobin had improved to 102 g / L and she was requiring transfusions every 4 months with her hematologist ’s intent to stop the transfusions if her hemoglobin remained greater than 100 g / L. Her epistaxis severity score at 6 month follow up was mild ( normalized score 3.05 ) .


A 30 - year - old woman with a history of HIV and HCV presented to the emergency department with a 4 - month history of gradual but progressive swelling of her lower limbs and abdomen . She denied any chest pain , dyspnoea , paroxysmal nocturnal dyspnoea or orthopnoea . Vitals were stable . Cardiovascular examination was remarkable for an elevated jugular venous pressure ( JVP ) of about 12 cm with a pronounced ‘ y ’ descent . There was a prominent pericardial knock sound heard over the praecordium . There was no pericardial rub or murmur . The lungs were clear to auscultation . The patient was markedly oedematous from the abdomen to the lower limbs . She was admitted for further diagnostic work - up and management . She was diagnosed with HIV around May 2010 and had been on antiretroviral therapy . She had not used illicit drugs for approximately 5–6 years . Renal and hepatic diseases were ruled out through blood tests and imaging . Peritoneal fluid was negative for malignancy . Echocardiogram showed a thickened bright pericardium adjacent to the right heart border ( figure 1 ) with parallel separation between epicardial and pericardial echoes ( railroad track sign ) , septal bounce and lack of pericardial slide . Annulus paradoxus was demonstrated on tissue Doppler ( figure 2 ) . Left ventricular ejection fraction was 64 % . The right ventricle was normal in size and function . There was moderate tricuspid regurgitation . There was no pericardial effusion , but the pericardial space was remarkable for debris . Constrictive physiology was demonstrated by Doppler study of tricuspid and mitral inflows ( figure 3 ) during inspiration and expiration ; diastolic flow reversal was also demonstrated in the hepatic veins during expiration . There was marked dilatation of the inferior vena cava with no change during inspiration or expiration . CT of the chest revealed thickening of the pericardium with no evidence of calcification . There were no lung nodules . Bilateral pleural effusions and interstitial thickening were present in the lung bases . Cardiac MRI confirmed the presence of a diffusely thickened pericardium at 5 mm . The inferior vena cava and hepatic veins appeared plethoric . There was a rapid early diastolic filling and associated diastolic septal bounce secondary to hindered late diastolic filling ( figure 4 ) . The patient showed a good response to diuresis . She underwent right and left heart catheterisation to confirm constrictive physiology .


This is the case of a 58 - year - old white Hispanic woman with a history of uveal melanoma in her right eye ( Fig.1 ) . She was admitted to the hospital with jaundice and abdominal pain for 10 days . On admission , laboratory tests were obtained ( a complete blood count was within normal limits , amylase : 136 U / L , total bilirubin : 6.37 mg / dL with a direct fraction of 5.30 mg / dL ) . Cross - sectional , abdominal computed tomography ( CT ) with contrast , showed a low - attenuating lesion localized in the pancreatic head ( measuring 4 × 3 cm ) and a thinner section of the distal bile duct suspicious for compression ) . After a multidisciplinary meeting , our patient was scheduled for EUS - FNA . EUS showed a solid , heteroechoic with predominantly hypoechoic areas , well - defined lesion with regular contours ( measuring 3.1 × 2.6 cm ) , localized between the head and neck of the pancreas ( Fig.2 ) . There was no vascular or lymph node invasion identified . EUS - FNA was performed with a 22 G needle using the fanning technique . In the cytology specimens ( Fig.3 ) , the cells presented have a discohesive dispersed pattern , with marked variation in size and shape including epithelioid and spindle - shaped cells with plasmacytoid and round nuclei . The nuclear chromatin is clumping and irregular with excessive parachromatin clearing , there are single or multiple macronucleoli , and abundant cytoplasm with deep brownish - black granules obscuring the cell details . The background shows necrosis and hemorrhage . Our patient underwent right orbital exenteration and followed by duodenopancreatectomy ( the Whipple procedure ) , without any complications ( Fig.4 ) . The surgical specimen showed undifferentiated cells , with extended disposition of brown pigment , infiltrating the pancreas and the serosa of the duodenal wall . Immunohistochemistry was positive for Melan - A , HMB45 , vimentin , S-100 protein and negative for cytokeratin , all consistent with metastatic malignant melanoma . At the moment , our patient is receiving adjuvant chemotherapy at an outside oncology clinic .


A 24 - year - old Malay male patient was referred to the respiratory clinic because of an abnormal pre - employment chest radiograph . He had been smoking about 10 cigarettes a day since he was 21 years of age . He stopped smoking 10 months ago after he noticed he had being having reduced effort tolerance for the past three years . He was an office worker and did not have a history of exposure to organic or inorganic dusts . His two siblings were asymptomatic . On examination , the patient was not tachypnoeic . There were no signs of finger clubbing or pulmonary hypertension . His oxygen saturation on room air at rest was 94 % and dropped to 92 % after climbing up four flights of stairs . Spirometry testing revealed a restrictive pattern of lung disease with a forced expiratory volume in 1 second ( FEV1 ) and a forced vital capacity ( FVC ) of 2.7 L ( 69 % of predicted ) and 3.2 L ( 68 % of predicted ) , respectively . The FEV1 / FVC ratio was 85 % . His haemoglobin ( 168 g / L ) , serum parathyroid hormone ( 2.9 pmol / L [ normal , 1.1 - 7.3 ] ) and calcium ( 2.34 mmol / L ) levels were normal . 24 - hour urine calcium was also normal 6.9 mmol with a 24 - hour urine volume of 2.8 L. His chest radiograph ( Fig.1A ) revealed dense micronodular opacities distributed symmetrically and predominantly in the middle to lower zones of both lungs giving the classical " sandstorm " appearance . The cardiac borders were obscured by the sand - like opacities . A high - resolution computed tomography ( HRCT ) scan of the lungs ( Fig.1B ) showed widespread tiny microcalcifications throughout the lungs with a preponderance of microliths in the lower lobes . There were associated areas of interlobular septal thickening and ground - glass changes . Subpleural cystic changes were also seen in both lower lobes giving rise to the ' black pleura sign ' ( Fig.1C ) ( 2 ) . No pneumothorax or pleural effusion was present . Both the bronchial system ( including the small bronchioles ) and the size of the pulmonary vessels were normal . As this was diffuse parenchymal lung disease , videoassisted thoracic surgical ( VATS ) lung biopsy was planned but the procedure was converted into a mini - thoracotomy because there was difficulty in manoeuvering the endostapler . There was a moderate pneumothorax postmini - thoracotomy from which the patient fully recovered after 5 days in the ward . The lung biopsy specimen revealed features consistent with PAM , with numerous calcospherites within the alveolar spaces ( Fig.1D ) . The intervening alveolar septae were congested and showed mild fibrosis with infiltrates of mainly lymphoplasmacytic cells .


A 73 - year - old Hispanic male with a history of stage IV chronic kidney disease and three - vessel coronary artery disease was admitted for coronary artery bypass graft surgery and mitral valve repair . On post - operative day two , the patient developed cardiac tamponade and subsequently progressed to a cardiac arrest while temporary pacing wires were being removed . Advanced cardiovascular life support and emergent exploratory sternotomy were performed , and the patient was transferred to the intensive care unit ( ICU ) for further care . During the patient ’s ICU admission , his renal failure progressed , requiring hemodialysis . A nasogastric tube was placed on post - operative day two for establishing early enteral nutrition . PEG tube insertion was suggested given his cognitive impairment and poor swallowing function on the Modified Barium Swallow test . His coagulation profile prior to the procedure revealed an international normalized ratio of 1.04 and partial thromboplastin time of 36 seconds . A complete blood count showed a hemoglobin of 11.6 grams per deciliter , a hematocrit of 34.9 % , and 281×103 platelets per microliter . His blood urea nitrogen was 55 milligrams / deciliter , and his serum creatinine was 5.22 milligrams / deciliter . The patient was taking aspirin 81 mg per day due to recent cardiac surgery , but was not on any anticoagulant agents . He also did not have a history of coagulopathy . PEG tube insertion was performed on post - operative day eight under intravenous anesthesia . The esophagogastroduodenoscopy ( EGD ) was accomplished without difficulty , revealing diffuse moderately erythematous mucosa without bleeding in the gastric antrum . While attempting PEG tube placement , a rapidly expanding hematoma ( Figure 1 ) developed at the needle insertion site . The procedure was stopped and the patient was sent back to the ICU with a nasogastric tube . A single dose of intravenous desmopressin ( 0.3 microgram / kilogram ) was administered under the suspicion of uremic bleeding . No further gastrointestinal bleeding events were noted . To assess platelet function , a platelet function assay ( PFA ) was ordered after desmopressin injection . PFA collagen / epinephrine closure time was 195 seconds ( normal < 174 seconds ) , and collagen / ADP closure time was 76 seconds ( normal < 120 seconds ) , indicating platelet dysfunction due to either aspirin or uremia . Aspirin was discontinued , and he was monitored with a daily complete blood count and metabolic panel . Three days later the patient underwent a second attempt at PEG tube placement . PFA collagen / epinephrine closure time prior to this procedure had gone down to 141 seconds , possibly due to withholding aspirin and an improvement in renal function . EGD showed an area of flat , bluish gastric submucosal bruising at the site of the previous hematoma ( Figure 2 ) . The PEG tube was placed successfully at an adjacent site . Over the course of the following month , the patient continued to undergo feeding through the PEG tube with no adverse events .


A newborn boy of healthy nonconsanguineous parents was delivered at 37 weeks ’ gestation by cesarean section . His mother ( primipara , 32 years old ) had been admitted to our hospital 2 weeks previously because of reduced fetal intrauterine movements and polyhydramnios . The child ’s birthweight was 2,330 g ( < 10th percentile ) , length 47 cm , and head circumference 33.2 cm ( 25th percentile ) . He had low - set ears and bilateral clubfoot . Apgar scores were 2 , 6 , and 7 at 1 , 5 , and 10 minutes . The child presented with severe hypotonia and generalized muscle weakness , requiring ventilatory assistance and total parenteral nutrition . Weaning failed because of inadequate pulmonary ventilation and respiratory acidosis . Hearing loss was detected by auditory evoked potentials , while cranial MRI showed mildly enlarged ventricles and liquor spaces . Two days after birth , the infant presented with severe abdominal distension with a hypoactive bowel . MRI revealed marked intestinal dilation without mechanical obstruction . Laboratory investigations showed hypoglycemia ( 27 mg / dL ) , hypomagnesemia ( 0.58 mmol / L ) , and hypokalemia ( 2.4 mmol / L ) . Blood lactate was normal ( 1.3 mmol / L , normal range 0.5–2.2 mmol / L ) and liver enzymes were unremarkable . A skeletal muscle biopsy was performed and showed scattered , hypertrophic cytochrome c oxidase ( COX)-deficient and succinate dehydrogenase – positive muscle fibers ( figure ) , suggesting a mitochondrial disorder . Molecular genetic studies revealed marked mtDNA depletion in muscle ( 93 % decrease as compared to age - matched controls ) , while a screen for mtDNA rearrangements within individual COX - positive and COX - deficient fibers4 was negative . We sequenced the entire coding region and intron - exon boundaries of the POLG1 gene , identifying two reported heterozygous missense mutations in compound c.679C > T predicting p. R227W and c.2542G > A predicting p. G848S. Sequencing of parental samples confirmed recessive inheritance . The infant died at 20 days of respiratory failure . At autopsy , the brain did not show remarkable changes on gross examination . Histology was not informative due to poor preservation of tissue ; there was no evidence of neuronal damage in the spinal cord . The liver showed diffuse cholestasis , consistent with total parenteral nutrition ; hepatocyte steatosis , necrosis , or liver fibrosis were not observed . The testicles were undescended , while remaining visceral organs were normal except for a marked dilation and thinning of the bowel wall . Despite normal histology , analysis of stomach , ileum , and colon homogenates revealed severe mtDNA depletion ( up to 94 % decrease ; table e-1 on the Neurology ® Web site at www.neurology.org ) . Laser capture microdissection analysis5 revealed that the mtDNA depletion was confined to the muscularis propria , being most prominent in its external layer ( figure ) . Ganglion cells from the myenteric plexus showed milder mtDNA depletion , restricted to the small intestine ( figure ) . There was no mtDNA depletion in liver ( not shown ) .


A 52 - year - old man with a 30 - year history of smoking presented with an abnormal nodule measuring 0.8 × 1.5 cm in the left upper lung lobe imaged through chest computed tomography ( CT ) scanning in March 2012 in a community hospital . He had previously been well without any additional relevant or abnormal symptoms . Physical examination suggested no significant abnormalities . Laboratory findings were within normal range , except for a carcinoembryonic antigen ( CEA ) level of 30.25 ng / mL ( normal range , 0–5 ng / mL ) found in the serum . Subsequently , a positron emission tomography ( PET)/CT scan showed a lung lesion with a standardized uptake value of 7.94 , which was highly suspected to be a malignant tumor ( Fig.1 ) . Subsequently , he underwent left upper lung lobectomy and lymph node dissection . Postoperative pathological examination revealed an adenocarcinoma ( ADC ) ( Fig.2A ) . Immunohistochemistry ( IHC ) staining results were positive for CK7 , TTF-1 , p63 , and NapsinA and negative for CK 5/6 , Syn , cgA , and Ki-67 ( 20%–30 % ) . The clinical stage was classified as IA2 ( pT1bN0M0 ) . Because the tumor was still in an early stage , the patient did not receive adjuvant treatment but continued to be monitored through regular hospital visits every 3 months . The patient 's condition had been stable until the onset of left chest pain in April 2015 . The patient was transferred to our hospital ( a tertiary care hospital ) for further treatment . Physical examination suggested a significant tender point in the left chest wall . Laboratory findings showed only a single parameter outside the normal range : the cytokeratin19 fragment antigen 21–1 ( CYFRA21–1 ) level was 17.45 ng / mL in the serum ( normal range = 0–3.3 ng / mL ) . CT and PET / CT scans highlighted the serious involvement of left pleura , showing bilateral lung lesions ( Fig.3A and E ) . At that point , the clinical stage was upgraded to IVB . After the CT - guided left pleura puncture , pathological IHC analysis showed positive staining for p63 , p40 , CK 7 , and CK 5/6 , while TTF-1 and NapsinA staining were absent , supporting a diagnosis of squamous cell carcinoma ( SCC ) ( Fig.2B ) . Meanwhile , molecular evaluation confirmed an L858R mutation in exon 21 and a T790 M mutation in exon 20 ( Fig.4B and C ) . Considering the pathology of the SCC was quite distinct compared with that of the originally resected NSCLC specimen , we retrospectively reassessed the surgery specimen to verify our primary diagnosis . The morphologic and IHC results were in complete agreement with the initial diagnoses , but an L858R mutation was identified using the newly added molecular evaluation ( Fig.4A ) . To relieve the acute pain in the left pleura as quickly as possible , palliative three - dimensional conformal radiotherapy ( 3D - CRT ) for the chest lesion was delivered at a total dose of 42 Gy/14 fractions ( f ) , once daily and 5f / week ( Fig.5 ) . The volume over radiation dose 5 Gy ( V5 ) values of the left , right , and total lungs were 20 % , 0 % , and 7.8 % , respectively ; the volume over radiation dose 20 Gy ( V20 ) values were 10 % , 0 % , and 5 % , respectively ; the mean lung dose ( MLD ) was 5 Gy . Meanwhile , considering the MST pathology was that of SCC , the patient received GP chemotherapy ( cisplatin 75 mg / m2 d1 , gemcitabine 1250 mg / m2 d1 , d8 , for 21 days as a cycle ) for 4 cycles , and the patient achieved partial remission ( PR ) after 2 cycles , followed by stable disease ( SD ) after 4 cycles ( Fig.3[B3[B and F ] and [ C and G ] ) according to response evaluation criteria in solid tumors ( RECIST ) 1.1 . Then , gefitinib ( 150 mg qd ) was used as maintenance treatment from November 2015 onward . During the treatment , Ibandronate sodium was given monthly for skeletal - related events . The patient is currently under surveillance with SD ( Fig.3D and H ) and with PS scores of 0 .


A 33 - year - old Moroccan man with a 5 - month history of recurring sinusitis presented with temperature , myalgia , cough , and hemoptysis . Thoracic physical examination revealed reduced vesicular murmur , and chest X - ray showed bilateral diffuse alveolar infiltrates . Laboratory analyses showed elevation of acute - phase proteins ( CRP 26 mg / dL ) and creatinine ( 1.2 mg / dL ) with nonnephrotic proteinuria and microscopic glomerular hematuria . An antibiotic therapy for community acquired and atypical pneumoniae with levofloxacin 500 mg daily iv was established , but on the 3rd day from admission the patient developed respiratory failure unresponsive to noninvasive ventilation and had to be intubated . White lung with consolidations and ground glass areas at computed tomography scan ( Fig.1 ) , diffuse airways bleeding at fiberoptic bronchoscopy , mild normocytic anemia ( 9 g / dL ) , and ANCA - PR3 positivity ( 18.9 U / mL ) were consistent with the diagnosis of GPA . Despite maximal ventilatory support , gas exchange did not improve ( pH 7.33 , PaO2 71 mm Hg , PaCO2 51 mm Hg , HCO3 – 25 mmol / L , SaO2 94 % ) requiring extracorporeal membrane oxygenation ( ECMO ) . Treatment with high - dose methylprednisolone ( 1 g daily for 3 days and 1 mg / kg daily thereafter ) , cyclophosphamide ( 1.2 g / pulse every 2 weeks for the 1st 3 pulses , followed by infusions every 3 weeks for the next 2 pulses ) , and plasma exchange ( PE ) , according to European vasculitis study group recommendations,[4 ] was immediately started with respiratory improvement that allowed ECMO and orotracheal tube withdrawal and subsequent discharge from intensive care unit . After 9 days from the beginning of treatment ANCA - PR3 levels normalized ( 3 U / mL ) . Two months later , after the 5th bolus of cyclophosphamide and with prednisone 0.3 mg / kg / day , the patient complained arthromyalgia and testicular pain with edema , cough , and shortness of breath : laboratory data showed worsening of renal function , mild anemia , and hypoxemia ( PaO2 61 mm Hg ) . Epididymitis was confirmed by ultrasound . Because of rapidly progressive renal failure ( estimated glomerular filtration rate 35 mL / min ) renal biopsy was performed and showed prominent intracapillary and extracapillary proliferation , with the formation of some fibrocellular crescents and focal capillary necrosis ( Fig.2 ) . Lung computed tomography - scan and fiberoptic bronchoscopy also confirmed alveolar hemorrhage relapse . Treatment with high - dose methylprednisolone ( 1 g daily for 3 consecutive days ) and rituximab ( 375 mg / m2 weekly for 4 weeks ) was started with rapid resolution of urologic and pulmonary involvement but delayed and partial improvement of renal function . At a 1 - year follow - up , the patient is alive and under treatment with azathioprine ( 150 mg daily ) and prednisone ( 15 mg daily ) . Renal function has markedly improved ( estimated glomerular filtration rate 94 mL / min ) same as gas - exchange ( pH 7.42 , PaO2 86 mm Hg , PaCO2 36 mm Hg , HCO3 – 23 mmol / L , SaO2 95 % ) . The patient signed institutional informed consent form at the time of admission to hospitalization . An approval by ethics committee was not necessary because of the routine health care .


A 19 - year - old woman presented with a lump in her right breast . She had macrocephaly and milimetric papules on the tongue giving a cobble stone appearance . The breast ultrasonography ( US ) showed more than twenty circumscribed , oval masses in each breast . An US - guided core biopsy was performed for the palpable masses which were confirmed as juvenile fibroadenomas . After the biopsy , a breast US was done as follow - up every 6 or 12 months for 3 years . During the surveillance period , core biopsies were performed six times , followed by US - guided vacuum - assisted core needle excisions or surgical excisions of lesions with increased sizes . Pathologic results were fibroadenoma , tubular adenoma or atypical ductal hyperplasia ( ADH ) involving fibroadenoma . At the age of 19 years , the patient underwent a neck US for a palpable mass in the left neck . Several indeterminate nodules were revealed in both thyroid glands . A follow - up US revealed that some of those nodules increased in size . The patient underwent a total thyroidectomy and the pathologic result revealed an invasive follicular carcinoma in the left thyroid and follicular adenoma in the right thyroid ( Fig.1A ) . At the age of 21 years , she visited the hospital due to a swelling of her left cheek . Head and neck CT scan revealed a vascular mass in the left parotid gland extended into the left forehead . An external carotid angiography confirmed the diagnosis of an AVM with feeder vessel arising from the left internal maxillary artery ( Fig.1B ) . A partial embolization decreased the blood flow through the AVM , alleviating the patient 's symptom . She was referred to genetic counseling under the suggestion of a Cowden syndrome and the PTEN DNA sequencing test of her blood sample revealed a frameshift mutation , c.301dupA ( p. I101NfsX6 ) . At the age of 22 years , the patient presented with a mass with increased size and increased vascularity in the left breast . An US - guided vacuum - assisted core needle excision revealed a ductal carcinoma in situ ( DCIS ) of non - comedo type and a low grade involving fibroadenoma ( Fig.1C ) . The contrast - enhanced breast MRI for the preoperative evaluation demonstrated multiple , well - circumscribed , enhancing masses in both breasts ( Fig.1D ) . All masses showed high or intermediate signal intensity on T2 - weighted images ( Fig.1E ) . Several masses showed an early rapid enhancement with washout kinetic pattern on time - signal intensity curve evaluation , which tends to be associated with malignancy ( Fig.1F ) . A bilateral prophylactic mastectomy with immediate breast reconstruction was performed in view of multiple bilateral breast masses with suspicious kinetic features on breast MRI and a high risk for breast cancer of Cowden syndrome . The surgical histopathology revealed ADH involving tubular adenoma without residual carcinoma in the left breast and multiple tubular adenomas , fibroadenomas and intraductal papillomas in both breasts . The patient had no family history of breast cancer or Cowden syndrome .


A 23 - year - old man with a history of severe aplastic anemia ( SAA ) underwent bone marrow transplantation from his HLA - haploidentical mother in January 2014 . The conditioning regimens consisted of busulphan cyclophosphamide and antithymocyte globulin ( BUCY+ATG ) ( 10 ) . Cyclosporine A ( CsA ) and short - term methotrexate ( MTX ) plus mycophenolate mofetil ( MMF ) were used as prophylaxis against graft - versus - host disease ( GVHD ) ( 11 ) . Standard measures were adopted for the prevention of infectious complications , which included fluconazole for antifungal prophylaxis and acyclovir to prevent herpes - related infections . A hemogram revealed the reconstruction of granulocytes ( ANC>0.5×109 / L ) on day +12 post - transplantation . The patient developed grade II acute GVHD of the skin on day +42 post - transplantation . This was treated by treatment with a standard - dose of methyl - prednisolone , which achieved a complete response ( CR ) . The patient 's chronic GVHD ( cGVHD ) of the skin gradually progressed from day +100 post - transplantation and he was treated with prednisolone and CsA. On day 120 post - transplantation , he complained of a cough and antibiotics were administered . A blood analysis revealed the following : WBC , 2.34×109 / L ; ANC , 1.72×109 / L ; hemoglobin , 85 g / L ; and platelets , 72×109 / L. Although both a chest computed tomography ( CT ) scan and tests for pathogens via routine culturing , including blood tests for Beta - D glucan ( G - test ) and Galactomannan ( GM - test ) were all negative , the patient 's cough did not respond to antibiotics and we empirically initiated treatment with voriconazole ( 6 mg / kg/12h for the first day , followed by 4 mg / kg/12h ) . Liver toxicity occurred during voriconazole treatment , thus the anti - fungal regimen was changed to micafungin ( 100 mg / d ) . However , the persistent cough did not improve and hoarseness developed after two weeks of treatment - ulcers were then observed in the throat by laryngoscopy ( Fig.1a ) . The patient developed severe dyspnea in the following week when anti - infection and topical treatments were applied . Fiberoptic bronchoscopy revealed an irregular , nodular material with white moss , which nearly obstructed the bronchus ; however , chest CT imaging was negative ( Fig.1b and c ) . The histopathological examination of biopsy specimens revealed an Aspergillus species ( Fig.1d ) . The patient was diagnosed with pseudomembranous Aspergillus tracheobronchitis type ITBA based on the results of bronchoscopy and a pathological examination ( 12 ) . The antibiotic and micafungin treatments were ceased and liposomal amphotericin B ( liposomal AmB ) was administered daily at a target dose of 3 mg / kg . The patient 's serum creatinine level rose from 60.4 μmol / L to 168 μmol / L during the first 7 days of liposomal AmB treatment . Due to progressive renal dysfunction , the anti - fungal regimen was switched to a combination of posaconazole ( 400 mg/12 h ) and caspofungin [ 50 mg , daily ( 70 mg for the first dose ) ] . The combination therapy continued for 2 weeks , until the previous nodules in the throat completely disappeared under bronchoscopy ( Fig.2a ) ; however , a repeat chest CT scan showed progression ( Fig.2b ) . The symptom of dyspnea gradually progressed , thus fiberoptic bronchoscopy was performed to remove the obstructive material from the patient 's airways once a week for two weeks . All of the symptoms were relieved and the final chest CT scan showed negative results before the discontinuation of anti - fungal therapy , and all of the tests were negative for Aspergillus . Posaconazole was administered as a secondary prophylactic treatment and the patient was discharged from hospital . The patient is still being followed and remains free of any recurrence of invasive fungal infection .


A 40 - year - old woman visited the Department of Obstetrics and Gynecology in our hospital because of a complaint of sudden dyspnea on effort . She was previously diagnosed with endometriosis and prescribed a combination oral contraceptive pill ( drospirenone / ethinylestradiol betadex , trade name ; Yaz ™ , Bayer , Leverkusen , Germany ) . Following the Kumamoto Earthquakes and subsequent aftershocks in April 2016 , she spent 7 nights in a vehicle . She noticed sudden dyspnea on effort when she walked outside the car on the 8th morning after the first earthquake . Although she walked around for personal reasons during the daytime , she spent nights in her small car with her legs in a hanging position . She noticed swelling and pain in her left lower extremity , and her serum D - dimer levels were 13.2 μg / mL. Deep vein thrombosis ( DVT ) was suspected by her home doctor . A contrast - enhanced computed tomography ( CT)-scan revealed a contrast deficit in the bilateral pulmonary artery ( Fig.2A ) and in the left lower extremity ( posterior tibial , soleus and gastrocnemius vein ) ( Fig.2B ) . Her right ventricular function was intact ( estimated pulmonary artery pressure=29/8 mmHg in cardiac ultrasound ) . Her plasma brain natriuretic peptide and high - sense troponin T concentration levels were 97.2 pg / mL and 0.0061 ng / mL , respectively . She was diagnosed with PTE and admitted to our department . On admission , her blood pressure was 129/80 mmHg and her heart rate was 72 beats per minute . Her degree of oxygen saturation ( SaO2 ) in arterial blood gas was 97.7 % . Her body mass index was 24.4 kg / m2 . Electrocardiography and cardiac ultrasound did not indicate heart failure . An investigation of most factors related to thrombus formation ( including protein C , protein S , antithrombin , and antiphospholipid antibodies ) revealed that all such factors were normal . The discontinuation of the oral contraceptive and the administration of rivaroxaban ( 30 mg , daily ) , a direct oral anticoagulant ( trade name : Xarelto ™ , Bayer ) was initiated according to the results of the EINSTEIN - PE trial ( 3 ) . After 7 days , a follow - up contrast - enhanced CT scan revealed a reduction in the thrombosis ( Fig.3 ) . The patient was followed up with rivaroxaban . The patient gave her consent for the publication of this study .


A 60 - year - old man presented with a retroperitoneal metastatic lymph node in magnetic resonance ( MR ) imaging with a history of right liver resection in 2010 , pulmonary wedge resection in 2012 , and transarterial chemoembolization twice in 2014 , in which the postoperative pathology suggested the mixed liver cancer , and poorly differentiated lung cancer from liver metastasis . At examination , the tumor was very close to the duodenum , pancreas , stomach , and hepatic portal vein . The patient with unresectable masses had hypertension over 10 years and chronic HBV - related cirrhosis for 30 years when he was treated with lamivudine 100 mg qd , and adefovir dipivoxil 10 mg qd for 6 years . He kept a constant body temperature of 37 ° C , the blood pressure of 148/96 mm Hg , and the pulse of 77 beats every minute . The patient had no obvious sense of abdominal pain , abdominal distention , nausea , and vomiting . On a test of tumor markers levels , the results were normal , which showed carcinoembryonic antigen 3.7 ng / mL , alpha fetoprotein 17.3 ng / mL , and carbohydrate antigen 19–9 levels 6.1 U / mL. In this study , the procedure was approved by the ethics committee of the First Affiliated Hospital of Zhejiang University . The efficacy of local ablation was estimated with Choi criteria , which appraised the variations both in tumor size and lesion density on computed tomography ( CT ) imaging : complete response , disappearance of all lesions and no new lesions ; partial response ( PR ) , a decrease in size of 10 % or a decrease in tumor density over 15 % on CT and no new lesions ; stable disease , not fit for complete response , PR , or progressive disease ; and progressive disease , an increase in tumor size over 10 % and not fit for PR by tumor density on CT or new lesions.[19 ] Preoperative MR imaging scan showed a mass of 2.8 × 3.5 cm in size close to the duodenum , pancreas , and blood vessels ( Fig.1A ) . During substance phase , MR images indicated mildly high signal intensity around the tumor and intermediate low signal intensity in the solid component and in the walls ( Fig.1B ) . Before the initial ablation , there was a retroperitoneal mild hyperechoic area on axial US image ( Fig.1C ) . In this study , the inpatient underwent percutaneous transhepatic biopsies and ablations to avoid the nearby blood vessels , the procedure of which was performed about 40 minutes under local anesthesia and mild sedation . We used ultrasonography to real - timely guide the inserting of 2 Nd : YAG laser fibers ( Echolaser X4 , ESAOTE , Italy ) in 0.3 mm diameter through a 21 - gauge Chiba needle into the lesions ( Fig.1D ) . Although part of the heat ran off by blood flow,[20 ] ablation could keep valid area from 12 to 15 mm with the power of 5 W in 5 to 6 minutes . Subsequently , immediate ultrasonography showed that the whole lesion was covered with hyperechoic zone ( Fig.(Fig.1E and F ) . However , the next day contrast - enhanced ultrasound ( CEUS ) revealed an abnormal residual of proximal part of the pancreatic head ( Fig.2A ) . At 5 - day follow - up contrast - enhanced CT images , the lower mass had low signal intensity and upper tumor revealed intermediate high signal intensity of residual in substance phase ( Fig.2B and C ) . Surprizingly , 1 - week follow - up CEUS image showed an enlarged retroperitoneal lymph node located near the pancreatic head , which could be fusion of these residuary small nodules ( Fig.2D ) . Then the patient undergoing the 2nd ablation with 2 laser fibers showed the lesions well - defined hyperechoic zone ( Fig.2E and F ) . On the 2nd day , postoperative CEUS images showed they were still remanent ( Fig.2 G and I ) . At the corresponding MR imaging , it also showed this in left of tumor ( Fig.2H ) . Thus , the 3rd laser ablation was performed ( Fig.2J)2J ) and 3 days later CEUS found that there was still a minor lesion located in the separation gap of last 2 needles ( Fig.3A ) . Subsequently the patient had the 4th ablation along this separation gap until hyperechoic overlay ( Fig.3B – D ) , then 3 days later , based on these 4 treatments , enhanced CT image of the retroperitoneal mass suggested complete necrosis ( Fig.3E – G).3E – G ) . After the following 1 month , substance phase MR revealed low - intensity signal of tumor necrosis with resolution of his problems ( Fig.3H ) . Until 5 months after 4 ablations , CT showed the tumor PR with little enhanced recurrence ( yellow arrows ) , which located in the left lower edge of original lesions ( Fig.4A and B ) . On laboratory test of tumor markers , the levels of carcinoembryonic antigen , alpha fetoprotein , and carbohydrate antigen 19–9 indicated 3.6 ng / mL , 26.7 ng / mL , and 8.1 U / mL , respectively . No obvious side effects were discovered in this case during these procedures .


We report the case of a 68 - year - old female with no past medical history , presented with an upper gastrointestinal bleeding and pain in left upper abdomen . Physical examination found a splenomegaly and signs of portosystemic collateral formation including abdominal wall dilated veins and rectal haemorrhoids . No biological abnormalities were noted besides those due to hypersplenism ( anaemia and thrombocytopenia ) . The patient underwent upper gastrointestinal endoscopy that showed stage III oesophageal varices with portal hypertensive gastropathy and red signs . Ultrasonography revealed a fusiform dilatation of the portal bifurcation measuring 40 mm in diameter with dilated branches ; no signs of portal thrombosis were detected ( Figure 1 ) . The portal Doppler study showed a normal hepatopetal flow . Other signs of portal hypertension were found , including splenomegaly , repermeation of the umbilical vein and a splenorenal collateral circulation . A complementary angio - CT allowed a better assessment of the portal system that contained some thin calcifications in the aneurysmal wall and the main portal trunk ( Figure 2 , Figure 3 , Figure 4 ) . Other signs of portal hypertension were noted such as oesophageal and gastric varices , parietal collateral circulation associated to ultrasonography findings . Liver cirrhosis was confirmed by transcient elastometry and biopsy .


A 34 - year - old woman , 1 week postpartum , presented to our dermatology clinic with an intensely pruritic generalized rash . Two days after delivery of her child , the patient developed an itchy rash on the abdomen . On discharge , she was instructed to follow up with the dermatology department if the rash did not resolve . After leaving the hospital , she reported that the eruption had progressively spread to the buttocks and legs and the itching seemed to be worse . The patient 's prenatal course was uneventful . She gained 13 kg during pregnancy , with a prepregnancy weight of 72 kg . A healthy male neonate was delivered by caesarean section at 38 weeks ' gestation without complication . The patient 's medical history was unremarkable . She was currently not taking any medications , and she reported no known drug allergies . On physical examination , the patient was afebrile and her blood pressure was normal . Examination of the skin revealed erythematous papules and urticarial plaques involving the abdominal striae with periumbilical sparing . Similar lesions were noted on the legs and buttocks ( Fig.1a ) . The face , palms , and soles were uninvolved . No vesicles or pustules were noted . Based on the characteristic clinical presentation and disease course , she was diagnosed with PUPPP . She was informed of the safety profile and potential benefits of medications but remained reluctant to use medications during lactation , despite her severe symptoms . AWB injection was then considered for her treatment . Venous blood of 10 mL was drawn from the patient , followed by intramuscular injection of 5 mL of the blood on each side of her buttock . Seven days later , both subjective and objective improvements of symptoms were noticed and she received 1 more session of AWB injection ( Fig.1b ) . On follow - up after 12 days , all subjective symptoms had improved , leaving only postinflammatory hyperpigmentation ( Fig.1c ) . No complications such as infection , abscess formation , or hematoma were observed at the injection site .


A 48 year - old female presented with vaginal bleeding and abnormal Pap smears . Upon diagnosis of invasive non - keratinizing SCC of the cervix , she underwent a radical hysterectomy with salpingo - oophorectomy which demonstrated positive spread to the pelvic lymph nodes and the parametrium . Pathological examination revealed that the tumour also extensively involved the lower uterine segment . 5 months after this surgery , the woman underwent external beam radiotherapy and intracavitary brachytherapy . Two years later , the patient presented with a three - month history of a productive cough , shortness of breath , and a 2 - 3 week history of progressive exertional dyspnea . X - rays of the chest demonstrated a reticular nodular pattern , and CT scans revealed multiple bilateral patchy areas of ground glass opacity scarring with focal areas of subsegmental atelectasis within both lungs . A differential diagnosis included interstitial pneumonia versus non - cardiogenic edema . The woman underwent a bronchoscopy , left thoracoscopy , and an open wedge left lung biopsy . Pathological examination of the left lung biopsy confirmed the presence of neoplastic sheets of cells classically distributed along the septal vessels , perivascular , peribronchial , and subpleural lymphatics . Subpleural nodules were also identified with the presence of neoplastic cells distending the subpleural lymphatics confirming LC ( Figures 1A , 1B , 1C ) . On immunohistochemical analysis , the lesional cells were strongly positive to p16 ( Figure 1D ) , high and low molecular weight keratins ( Figure 1E ) , cytokeratin-7 ( CK7 ) ( Figure 1F ) , CK19 , and pan keratin , and negative to CK20 , p63 , and EGFR . Based on these findings , she was diagnosed to have lymphangitic carcinomatosis in the lung metastatic from SCC of the cervix . She was started on chemotherapy ( Carbo / Taxol ) with corticosteroids while in the hospital , and was discharged ten days later . Post - treatment improvement of clinical symptoms was paralleled by radiographic imaging that showed marked interval improvement of the nodular opacifications and the interstitial thickening that had previously been noted . Despite this improvement , she subsequently died 13 months after the initial diagnosis .


A 47 - year - old female patient presented progressively worsening pain in the chest wall , back , and bilateral lower extremities as well as muscle weakness . She did not have a family history of bone disease or fractures . Physical examination was unremarkable excluding bone and muscular abnormalities . Bone scintigraphy using technetium-99 m methylenediphosphate showed increased uptake in the shoulder , multiple ribs , thoracic and lumbar spines , bilateral sacroiliac joints , left ilium , and left foot ( Fig.1 ) . Plain radiographs revealed osteoporosis change of thoracic and lumbar spine as well as compression change of several spines , consistent with findings of magnetic resonance imaging and computed tomography ( CT ) ( Fig.1 ) . Decreased bone mass was presented in the lumbar spine ( T - score : −3.4 ) , femoral neck ( T - score : −3.1 ) , and hip ( T - score : −3.5 ) . Main laboratory data are shown in Table 1 . She demonstrated hypophosphatemia , hypokalemia , hypouricemia , elevated level of ALP , C - telopeptides and chloride , and decreased carbon dioxide combining power . The urinalysis revealed a high pH value , increased level of potassium , and decreased level of specific gravity , chlorine , and phosphate . Persistent glycosuria and proteinuria were repeatedly found , despite normal HbA1c and plasma glucose level . Other laboratory test results including thyroid function test , serum parathyroid hormone , 25 - hydroxyvitamin D , and protein electrophoresis were within normal range . In addition , all of serum tumor markers were negative . Due to limited technique , we can not determine the level of serum fibroblast growth factor 23 . With the clinical diagnosis of HO , fluorodeoxyglucose positron emission tomography / computed tomography ( 18F - FDG PET / CT ) and technetium-99 m octreotide ( 99mTc - OCT ) scintigraphy were performed to confirm whether the occult causative tumor exist . However , the results of these 2 tests were negative except that mild uptake in the seventh rib was found on PET / CT , which identified no evidence of a neoplastic lesion potentially responsible for HO ( Fig.2 ) . The immunological examination showed elevated level of serum IgG , IgM , and IgA , as well as positive antinuclear antibody , anti - SSA antibody , and rheumatic factor . Subsequently , Schirmer test was abnormal and lip biopsy supported the diagnosis of SS ( Fig.3 ) . Eventually , this patient was diagnosed with HO secondary to SS , and she was then treated with alkalinization ( citrate 4 g / day and potassium citrate 3 g / day for 2 weeks ) , steroids ( prednisone 20 mg / day for 1 month , 10 mg / day for 4 months ) , neutral phosphate ( 1.0 g / day for 5 months ) , calcium supplements ( 600 mg / day for 5 months ) , and together with activated vitamin D ( 0.5 g / day for 5 months ) . So far , she recovered uneventfully with relieved pain and increased serum phosphorus level . This case report was approved by the ethics committee of West China Hospital of Sichuan University , Chengdu , China , and the written informed consent was obtained .


A 66 year old patient with a history of UC treated by mesalazine since 5 years consulted in July 2012 for a painful erythematous swelling of the back of the right hand . The bacteriological samples were negative , the lesion was considered as an abscesses and the patient was treated with antibiotics and local antiseptics without improvement . The evolution was characterized by the appearance of an erythematous vesiculobullous centrifugal expansion taking the whole back of the right hand treated several times by various antibiotics unsuccessfully . Biopsy of this lesion was initially not specific . In October 2012 , the patient was hospitalized for fever and polyarthralgia in the context of impaired general condition . He had a quiescent UC disease . The skin examination found an erythematous plaque of 10 cm of diameter , with a raised border and vesicules , taking the back of the right hand and wrist ( Figure 1 ) . There was also an infiltrated erythematous plaque on the right leg ( Figure 2 ) and another topped with a large pustule at the left ankle ( Figure 3 ) . In biology , there was a biological inflammatory syndrome and high leukocytosis with neutrophils . Hepatic and renal functions were normal . Skin biopsy showed at the edge of the back of the closet right hand ulcerated epidermis and the dermis infiltrate rich in neutrophils with leukocytoclastic vasculitis finding a PG ( Figure 4 ) . Other biopsies taken at infiltrated erythematous plaques of the ankle showed a normal appearance of skin , edema of the superficial dermis based on an infiltrate rich in neutrophils without vasculitis confirming the diagnosis of SS ( Figure 5 ) . Corticosteroid treatment was then started with prednisolone at a dose of 1mg / Kg / j. The evolution was marked since day 7 of treatment by the desinfiltration of the plaques ( Figure 6 ) , the recovery of the general condition and disappearance of biological inflammatory syndrome .


A 65 - year - old man was referred to our hospital for evaluation of stuttering chest pain for 10 days , and he was a common worker . There was no positive finding from the relevant physical examination . He has no medical , family , and psychosocial history including co - morbidities , and relevant genetic information . His electrocardiogram showed T - wave inversion over leads V1 to V4 ( Figure 1 ) . Coronary angiography showed 90 % stenosis in the mid - left anterior descending coronary artery ( LAD ) , which was stented ( Figure 2 ) . T - wave still inversion over leads V1 to V4 after the percutaneous coronary intervention ( PCI ) ( Figure 3 ) . The patient discharged after the PCI in 5 days and recharged in the hospital because of a palpation . His electrocardiogram demonstrated ventricular tachycardia ( Figure 4 ) , and severe hypertension , remarkable blood pressure fluctuation between 224/76 and 70/50 mm Hg . Although several antihypertensive drugs were used , ventricular tachycardia still occurred on him for 2 times , each was preceded by a period of blood pressure fluctuation and burst out concomitantly at the peak of a hypertension crisis . The patient felt abdominal pain and his abdominal ultrasound showed suspicious right adrenal gland tumor . Enhanced computed tomography of adrenal gland conformed that there was a tumor in right adrenal gland accompanied by an upset level of aldosterone ( Figure 5 ) . The tumor was removed by laparoscope , and pathological examination showed pheochromocytoma ( Figure 6 ) . After the surgery , the blood pressure turned normal gradually . There was no T - wave inversion in lead V1 - V4 ( Figure 7 ) .


A 41 - year - old man with a history of alcoholism for 10 years and sober for the last 3 years , recurrent alcoholic pancreatitis , and no known liver disease , presented with several episodes of hematemesis and abdominal pain for 2 days . Esophagogastroduodenoscopy ( EGD ) , performed 3 years earlier for abdominal pain , had revealed no esophageal varices , gastric varices , portal hypertensive gastropathy , or other GI lesions . Physical examination revealed a blood pressure = 100/60 mm Hg , pulse = 60 beats / min , no jaundice , no stigmata of chronic liver disease , a soft abdomen with mild epigastric tenderness but no rebound tenderness , no abdominal bruit , and no pulsatile abdominal mass . Rectal examination revealed gross melena . Laboratory tests revealed hemoglobin = 12.5 g / dL , platelets = 301,000 / mL , INR ( international normalized ratio ) = 1.0 , blood urea nitrogen = 20 mg / dL , and creatinine = 1.1 mg / dL. Serum aspartate aminotransferase = 21 IU / L , alanine aminotransferase = 16 IU / L , total bilirubin = 0.6 mg / dL , alkaline phosphatase = 64 IU / L , albumin = 4.4 gm / dL , and lipase = 32 U / dL. The hemoglobin declined acutely to 9.3 g / dL. Abdomino - pelvic computerized tomography ( CT ) , with intravenous contrast , revealed a 5 - cm wide , irregular , pancreatic / peripancreatic mass , compressing both the lesser curvature of the stomach and the SV ( Fig.1A , B ) , a normal portal vein , and normal liver parenchyma . The SV compression was pathophysiologically significant as indicated by proximal SV dilatation . EGD revealed in the proximal stomach a fine , reticular , pale - white , polygonal , mucosal , network in a snakeskin pattern , and characteristic of portal hypertensive gastropathy that was actively oozing ; extensive coffee - ground , blood clots in the stomach ; small gastric cardial and fundal varices without stigmata of recent hemorrhage ( SRH ) ; and no esophageal varices ( Fig.2 ) . The extrinsic mass produced a large , round bulge extending into the lumen of the proximal gastric body along the lesser curvature ( Fig.2 ) . Magnetic resonance cholangio - pancreatography ( MRCP ) revealed a 5 - cm wide , enhancing , vascular mass likely arising from the LGA and located between the gastric lesser curvature and distal pancreatic body ; compressing the stomach ; compressing the middle SV ; and resulting in large collateral veins draining the SV into the superior mesenteric vein ( Fig.3A , B ) . Abdominal ultrasound with Doppler studies demonstrated large , turbulent arterial flow into this vascular mass , suggesting a large PA ( Fig.4 ) . Visceral arteriogram showed a 5.3 × 2.2 - cm - wide PA supplied by an LGA branch ( Fig.5A ) , which was embolized and occluded with microcoils ( Fig.5B ) . Eight weeks later , the patient had a stable hemoglobin level with no further GI bleeding . Abdomino - pelvic CT angiography demonstrated the PA had markedly decreased in diameter , contained numerous microcoils , and had no blood flow (Fig.1C ) .


A 70 - year - old male was admitted to our hospital for perioperative cardiac evaluation of abdominal aortic aneurysm . The patient had undergone coronary artery bypass grafting ( CABG ) for severe stenosis of the left main coronary artery ( LMCA ) 4 years before , which consisted of the right internal thoracic artery ( RITA ) to the left anterior descending artery ( LAD ) and saphenous vein graft ( SVG ) to the left circumflex artery ( LCX ) . Due to continual growth of the abdominal aortic aneurysm over the years , surgery was indicated . Left coronary angiogram showed 90 % stenosis of the LMCA and total occlusion of both grafts . PCI was performed for LMCA stenosis ( Fig.1 ) . A 7 - Fr sheath was inserted into the right femoral artery , and a CLS4 guiding catheter ( Boston Scientific , Natick , MA , USA ) was engaged into the left coronary artery ( LCA ) ostium . A guidewire ( SION Blue , Asahi Intecc , Aichi , Japan ) was inserted into the distal LAD , and another guidewire ( Hi - Torque Pilot 50 , Abbott Vascular , Santa Clara , California , USA ) was inserted into the distal LCX . Intravascular ultrasound ( IVUS ) ( Intra - focus WR , Terumo Corp , Tokyo , Japan ) demonstrated heavy circumferential calcification in the LMCA lesion . After a 4.0 × 15 - mm Quantum Maverick balloon catheter ( Boston Scientific ) was inflated in the lesion , LCA angiogram revealed a large dissection ( Fig.2 ) . A 4.0 × 28 - mm Multi - Link Vision stent ( Abbott Vascular ) was immediately deployed . Angiographic image of the residual dissection disappeared and the stent was dilated by a 5.0 × 15 - mm Quantum Maverick balloon catheter . The absence of residual dissection was confirmed by angiography after stent implantation . Next , LMCA - LAD and LMCA - LCX kissing balloon angioplasty was initiated using a 4.0 × 28 - mm stent delivery balloon in the LAD and a 3.0 mm × 15 - mm Ikazuchi balloon ( Kaneka Medics , Tokyo , Japan ) in the LCX . The final angiographic imaging showed optimal results , and IVUS imaging of the lesion showed completely sealing by the stent ( Fig.3 ) . A large hematoma at the femoral puncture site and local bleeding was observed after removal of the femoral sheath and manual compression was applied for haemostasis . The hematoma did not appear to have spread the next day and the patient was discharged from the hospital on the fifth day after PCI . Unexpectedly , the patient was admitted to our emergency room with new - onset chest pain and dyspnoea the next day after the discharge . His vital signs indicated cardiogenic shock . Electrocardiogram showed ST elevation in leads V1 - V3 . Although echocardiography could not detect left ventricular asynergy , a large mass on the outflow tract of right ventricular chamber was observed ( Fig.4 ) . Contrast - enhanced computed tomography showed a 40 - mm hematoma compressing the main pulmonary artery trunk and the right ventricle ( Fig.5 ) . During examination , the patient suffered sudden cardiopulmonary arrest . He received immediate cardiopulmonary resuscitation and returned to spontaneous circulation after the insertion of a percutaneous cardiopulmonary support device . The patient underwent emergent surgery for the removal of the hematoma after informed consent was obtained from the patient 's family . During surgery , large , dark red clots between the pulmonary artery trunk and aorta were observed . The suspected origin of the epicardial hematoma was blood oozing from the LMCA stent site . Despite successful surgical repair , the patient died from aortic rupture induced by external cardiac massage .


A 24 - year - old Azeri male presented with a chief complaint of blurred vision in both eyes . He was diagnosed with achondroplasia in early childhood . He was born at term with normal birth weight . There was no history of hereditary ocular or systemic disease . This patient had characteristic features of achondroplasia ( i.e. , short stature , lumbar lordosis , short proximal long bones , large hands , frontal bossing , and flat nasal bridge ) [ Figure 1 ] . His height was 108 cm . Best corrected visual acuity was 20/70 in the right eye with −0.75 + 1.25 × 60 ° and 20/70 in the left eye with OS : +0.25 + 0.50 × 130 ° . Pupils were equal in size , round and reactive to light with no reverse afferent pupillary defect . Ocular motility was normal in both eyes and slit lamp examination was unremarkable . The intraocular pressure was 12 mm Hg by Goldmann applanation tonometry . Central corneal thickness values of the right and left eyes were , 561 and 557 μ , respectively . Dilated fundus examination revealed a well - demarcated circular macular lesion in both eyes . In the right eye , this lesion was approximately 2.5 disc diameters ( DD ) in width and 2.7 DD in length . In the left eye , this lesion was approximately 2.5 DD in width and 2.8 DD in length . The border of the lesions was pigmented . Underlying choroidal and overlying retinal vasculature appeared normal . In addition , a stalk of tissue emanated from the optic disc and coursed into the vitreous [ Figure 2 ] . Fluorescein angiography of the posterior segment and optical coherence tomography of the optic nerve head ( Stratus ; Carl Zeiss GmbH , Jena , Germany ) were performed [ Figures [Figures3 and 4 ] . Due to the background maculopathy , the patient could not appropriately fixate on the target during image acquisition resulting in slightly decentered fluorescein angiography images [ Figure 3 ] .


A 4 and a half years old male child presented to our hospital for abdominal swelling . The child occasionally felt stomach ache that was relieved for no reason . The child showed no obvious tenderness and rebound . He was born as a premature baby in the 32nd week of his mother 's pregnancy . His medical history included inguinal hernia on 2 sides , but he had only received an operation for the left inguinal hernia . No family or genetic history was found . Physical examination of this child showed that the abdomen was obviously bulging . The mass could be touched below umbilicus about 3 fingers . There were no other positive signs . The results of laboratory tests were almost normal , including alpha - fetoprotein ( AFP ) level . The Child – Pugh score was less than 5 . Abdominal ultrasonography showed an uneven lesion in the liver and a diagnosis of HB was considered . Enhancement computed tomography ( CT ) scanning showed a large mass in the left upper abdomen and left lobe of the liver with an irregular edge . The size of the mass was 12.3 × 10.2 × 7.2 cm ( Fig.1 ) . There was an uneven nodular shadow and the edge was obvious when the mass was enhanced . Calcification and an expanded bile duct were not observed . The left branch of the portal was pressed and the distant part was shown to be unclear . Abnormal was not observed in the right branch . According to the medical history , the diagnosis of HB was considered . We did not recommend a biopsy to the patient because it might cause bleeding and tumor diffusion . An operation was necessary . The patient underwent DVSS surgery with sufficient preparation . Exploration showed an extrahepatic 12 × 10 cm mass in the left lobe of the liver ( Fig.2 ) . After the operation , the sample was split . The edge of the tumor was clear but no envelope . Tremelloid mesenchyme was seen on the section ( Fig.2C ) . The pathological examination showed that the sample was grey red and 14 × 13 × 7 cm in size . The color of the section was grey red and grey white . MHL was diagnosed ( Fig.3 ) . Immunohistochemistry results ( Fig.4 ) were the following : mesenchymal CD34 ( + ) , D2–40 ( + ) , S-100 ( - ) , SMA ( + ) , CD7 ( - ) , epithelium CK19 ( + ) , AFP ( - ) , HCG ( - ) . After 9 days of recovery , the patient returned home without complications . A follow - up examination , which included blood routine , liver renal function , coagulation routine , and ultrasound scan at 30 days , did not show any abnormality . No adverse or unanticipated event was presented .


A 49 - year - old woman was admitted to the Department of Radiology of the Second Affiliated Hospital of Zhejiang University in October 2004 with right upper quadrant pain and weight loss . She was a hepatitis B virus carrier . Her α - fetoprotein level was 1185.3 ng / mL. Ultrasonography and computed tomography ( CT ) revealed a 10 - cm mass in the posterior segments of the right liver lobe . A 1.5 - cm mass was also found in the left lateral segment . These clinical signs indicated that the patient had inoperable HCC and Child - Pugh class A cirrhosis . TACE was offered to the patient . Angiogram demonstrated no obvious hepatic arterio - venous shunt , but multiple smaller masses in both lobes of the liver . An emulsion of oxaliplatin , pirarubicin , hydroxycamptothecin and lipiodol were prepared , 35 mL and 3 mL of the mixture were administered intra - arterially to the right and left hepatic artery , respectively . The patient experienced right upper quadrant pain after TACE and had an uneventful recovery . One month later , a second TACE procedure was performed via the right hepatic artery and 40 mL of the mixture was administered . On the next day , she experienced sudden acute dyspnoea and the peripheral oxygen saturation decreased to 90 % . The chest X - ray showed some increased reticular shadows in the left lung , especially in the lower zones , and a chest CT scan revealed multiple iodized oil - like high - density materials in parenchyma of the lung ( Figure 1 ) . After 10 mg dexamethasone i.v . and other supportive therapies were administered , the respiratory symptom was attenuated . Two days later , the patient suffered from a serious headache and transient consciousness loss , accompanying nausea and vomiting followed by confusion , lower extremity weakness . Non - contrast enhanced CT scanning showed multiple disseminated hyper - intense lesions in the brain , consistent with deposition of iodized oil ( Figure 2 ) . One week later , her respiratory and neurologic symptoms disappeared completely , and she was discharged . The patient also consequently completed the other three TACE procedures , during which no similar symptoms occurred .


A 46 - year - old woman presented with severe acute pain in the right iliac fossa and periumbilical region . Ultrasound and a computed tomography scan revealed a mucocele in the vermiform appendix , with a well defined lesion located at the mid zone of the appendix . The patient underwent right hemicolectomy , and her postoperative clinical course was uneventful . Gross examination of the surgical specimen showed an enlarged appendix , which was filled with thick mucinous material . A distinct lesion which involved the appendiceal wall , and measured 1.5 cm maximally , was identified in the mid - portion of the appendix . There was no evidence of perforation , extravasation of mucin into the periappendiceal tissue , or pseudomyxoma peritonei during surgery . Histopathological examination showed combined GCC and MCA of the vermiform appendix ( Figure 1A ) . The appendiceal lumen was dilated and lined by mucin - containing columnar epithelial cells ( Figure 1B ) . There was no significant cytologic atypia , and no mitotic figures were identified . Focal papillary configurations of the lining epithelium , and mild epithelial pseudostratification were present . In addition , the appendiceal wall was infiltrated by glandular structures of various sizes which were arranged in nests and tubules . These glandular structures comprised 2 distinct types of cells : ( 1 ) small to intermediate sized monotonous neuroendocrine cells with a small amount of finely granular eosinophilic cytoplasm , and mild cytonuclear atypia ( Figure (Figure1C);1C ) ; ( 2 ) mucin - filled intermediate sized cells ( goblet cells ) , with peripherally located small , crescent - like hyperchromatic nuclei , and indistinct nucleoli ( Figure (Figure1D).1D ) . Scattered infiltrating single goblet neoplastic cells were focally present . As previously described[1 ] the tumor nests appeared to arise from the basiglandular region of the intestinal crypts in close proximity to the MCA ( Figure (Figure1E).1E ) . There was no lymphovascular invasion , although perineural and intraneural invasion was present . The tumor infiltrated the full thickness of the appendiceal wall and extended to the mesoappendix . Ten lymph nodes were histologically identified , of which all were negative for malignancy . Immunohistochemically ( Table 1 ) , the tumor cells of the GCC were positive for chromogranin , synaptophysin , and serotonin , which are neuroendocrine markers . Diffuse staining for cytokeratin ( CK ) 20 ( Figure 2 ) , CK19 , and CD99 was also present . The Ki67 proliferating index revealed nuclear staining in approximately 15 % of the tumor cells . There was no staining for CK7 .


A 68 - year - old female nonsmoker , nondrinker with a medical history of hypertension presented with new - onset painless jaundice and pruritus , a three - month history of 9.9 kg weight loss and chronic diarrhea with four to five loose bowel movements per day . Medications included vitamin D , amlodipine and eprosartan . Physical examination was normal except for jaundice and muscle wasting . Recent colonoscopy had been normal . Total and direct bilirubin levels were 6.84 mg / dL ( 116.96 μmol / L ) and 9.18 mg / dL ( 156.98 μmol / L ) , respectively . Other results included an international normalized ratio of 1.0 , alanine aminotransferase level 247 U / L ( normal <33 U / L ) , aspartate aminotransferase level 139 U / L ( normal < 32 U / L ) and alkaline phosphatase level 524 U / L ( normal 35 to 104 U / L ) . Viral hepatitis serologies , and antimitochondrial antibody and anti - smooth muscle antibody tests were negative . Her alpha - fetoprotein level was 2.4 ng / mL ( normal < 5 ng / mL ) , total immunoglobulin ( Ig ) G was 1880 mg / dL ( normal < 640 mg / dL ) , carbohydrate antigen 19 - 9 was 856 U / mL ( normal <33 U / mL ) and IgG4 was 890 g / L ( normal <3 g / L ) . Doppler ultrasound , magnetic resonance cholangiopancreatography and magnetic resonance imaging of the liver were suspicious for a subtly enhancing mass ( 2.8 cm to 4.2 cm in diameter ) in the region of the hilum and porta hepatis , obstructing both the right and left hepatic ducts . Endoscopic retrograde cholangiopancreatography identified strictures in the central portions of the right and left hepatic duct , which was concerning for cholangiocarcinoma ( Figure 1 ) . Biliary brushings were negative for malignancy . Esophagogastroduodenoscopy was normal . Biopsies of the ampulla of Vater revealed chronic active duodenitis ( Figures 2 and and3);3 ) ; an ancillary test confirmed the diagnosis ( Figure 4 ) . Treatment with corticosteroids normalized the patient ’s biochemical and radiological abnormalities within three months .


A 14 - month - old boy was referred to our hospital from another hospital because of dilated and hypertrophied left ventricle ( LV ) , neutropenia , and developmental delay on 27 October 2011 . He was born at full term , with a body weight of 3.2 kg . Sixteen days after birth , he was hospitalized owing to persistent irritability . A chest radiograph showed cardiomegaly , and an echocardiogram revealed decreased LV contractility ( ejection fraction : 24 % ) . Under the impression of myocarditis , he had been managed for 1 yr before presentation in our hospital . Moreover , the patient showed feeding difficulty and developmental delay from birth . Before referral , he had been admitted to other hospitals 7 times because of infection episodes . He was taking furosemide , spironolactone , enalapril , and carvedilol before referral . When he was referred to our hospital at 14 months old , his body weight was 6 kg ( less than 3rd percentile ) and his height was 71 cm ( less than 3rd percentile ) . His overall motor development was delayed , and he could not sit alone . He could say " mama " and " papa . " The recorded blood pressure and heart rate were 94/30 mmHg and 132 beats per minute , respectively . On physical examination , no definite heart murmur was audible and the liver was not palpable . He also showed persistent neutropenia , which started during his stay in the previous hospital . His WBC and neutrophil counts were 8,800/µL and only 2 % ( 176/µL ) , respectively . The B - natriuretic peptide level was 1,045 pg / mL. A chest radiograph showed mild cardiomegaly ( cardiothoracic ratio : 62.8 % ; Fig.1A ) , and an electrocardiogram showed a low QRS voltage at the limb leads . An echocardiogram revealed a dilated and hypertrophied globular LV with a hypertrophied papillary muscle and hyper - trabeculation , which did not meet the criteria of LV non - compaction . The other echocardiographic parameters were as follows : LV internal diameter at diastole , 37.7 mm ( Z = 10.2 ) ; ejection fraction , 36.6 % ; and LV mass index , 75.6 g ( Z = 6.3 ; Fig.1B ) . To rule out the systemic cause of the dilated and hypertrophied LV , we performed a thoraco - abdominal computed tomographic ( CT ) angiography with contrast dye . The CT findings showed no abnormality in the kidney and other organs and vessels . However , after undergoing CT angiography , the patient showed abrupt high - grade spiking fever ( Fig.2 ) and developed secretory diarrhea ( 800 cc per day ) associated with metabolic acidosis . At this time , his WBC and neutrophil counts decreased to 3,290/µL and 1 % ( 33/µL ) , respectively . The B - natriuretic peptide level was greater than 4,901 pg / mL. He did not show associated respiratory symptoms . The results of the respiratory and gastrointestinal viral studies were negative , and the blood and stool cultures were negative for pathogens . The C - reactive protein level was 7.08 mg / dL. Despite supportive care including intravenous fluid resuscitation and empirical antibiotics , the patient 's condition worsened , with aggravated metabolic acidosis and respiratory difficulty , requiring transfer to the intensive care unit ( ICU ) . Just before the ICU transfer , the serum pH was 6.881 ; bicarbonate level , 7.8 mmol / L ; and total CO2 was 41.4 mm Hg . Although the patient had been treated with intensive ventilator care , several inotropic agents , and other supportive care measures , he eventually died from the aggravated metabolic acidosis and acutely decompensated heart failure 7 days after the ICU care . During the stay in ICU , we performed genetic analysis for Barth syndrome from the evidence of displayed cardiomyopathy , neutropenia , and developmental delay . The gene sequence analysis revealed that his TAZ gene harbored a novel hemizygous frameshift mutation , c.227delC ( p. Pro76LeufsX7 ) , which he inherited from his mother ( Fig.3 ) .