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Tissue changes around loose prostheses. A canine model to investigate the effects of an antiinflammatory agent. The aseptically loosened prosthesis provided a means for investigating the in vivo and in vitro activity of the cells associated with the loosening process in seven dogs. The cells were isolated and maintained in culture for sufficient periods of time so that their biologic activity could be studied as well as the effect of different agents added to the cells in vivo or in vitro. The biologic response as determined by interleukin-1 and prostaglandin E2 activity paralleled the roentgenographic appearance of loosening and the technetium images and observations made at the time of revision surgery. The correlation between clinical, roentgenographic, histologic, and biochemical loosening indicates that the canine model is suitable for investigating the mechanisms of prosthetic failure. A canine model permits the study of possible nonsurgical therapeutic interventions with the ultimate hope of stopping or slowing the loosening process.

Neuropeptide Y and neuron-specific enolase levels in benign and malignant pheochromocytomas. Neuron-specific enolase (NSE) is the isoform of enolase, a glycolytic enzyme found in the neuroendocrine system. Neuropeptide Y (NPY) is a peptide recently discovered in the peripheral and central nervous systems. Serum NSE and plasma NPY levels have been reported to be increased in some patients with pheochromocytoma. The authors evaluated whether the measurement of these molecules could help to discriminate between benign and malignant forms of pheochromocytoma. The NSE levels were normal in all patients with benign pheochromocytoma (n = 13) and elevated in one half of those with malignant pheochromocytoma (n = 13). Plasma NPY levels were on the average significantly higher in the malignant (177.1 +/- 38.9 pmol/l, n = 16) than in the benign forms of the disease (15.7 +/- 389 pmol/l, n = 24). However, there was no difference in the percentage of patients with elevated NPY levels. These results show that determination of serum NSE may be useful for distinguishing between malignant and benign pheochromocytoma; the measurement of plasma NPY is not useful for differentiating the two kinds of tumors.

Sexually transmitted diseases of the colon, rectum, and anus. The challenge of the nineties. During the past two decades, an explosive growth in both the prevalence and types of sexually transmitted diseases has occurred. Up to 55 percent of homosexual men with anorectal complaints have gonorrhea; 80 percent of the patients with syphilis are homosexuals. Chlamydia is found in 15 percent of asymptomatic homosexual men, and up to one third of homosexuals have active anorectal herpes simplex virus. In addition, a host of parasites, bacterial, viral, and protozoan are all rampant in the homosexual population. Furthermore, the global epidemic of AIDS has produced a plethora of colorectal manifestations. Acute cytomegalovirus ileocolitis is the most common indication for emergency abdominal surgery in the homosexual AIDS population. Along with cryptosporidia and isospora, the patient may present to the colorectal surgeon with bloody diarrhea and weight loss before the diagnosis of human immunodeficiency virus (HIV) disease. Other patients may present with colorectal Kaposi's sarcoma or anorectal lymphoma, and consequently will be found to have seropositivity for HIV. However, in addition to these protean manifestations, one third of patients with AIDS consult the colorectal surgeon with either condylomata acuminata, anorectal sepsis, or proctitis before the diagnosis of HIV disease. Although aggressive anorectal surgery is associated with reasonable surgical results in some asymptomatic HIV positive patients, the same procedures in AIDS (symptomatic HIV positive) patients will often be met with disastrous results. It is incumbent upon the surgeon, therefore, to recognize the manifestations of HIV disease and diagnose these conditions accordingly.

Lipolytic factors associated with murine and human cancer cachexia. We have identified a lipolytic factor in extracts of a cachexia-inducing murine carcinoma (MAC16) that shows characteristics of an acidic peptide and appears to be composed of three fractions of apparent molecular weights corresponding to 3 kd, 1.5 kd, and 0.7 kd, as determined by exclusion chromatography. Material with identical chromatographic and molecular weight characteristics was also present in the serum of patients with clinical cancer cachexia but absent from normal serum, even under conditions of starvation. The MAC16 lipid factor, when injected into animals bearing the non-cachexia-inducing tumor MAC13, was capable of inducing weight loss without a significant reduction in food intake. Similar lipolytic material, although in lower concentration, was also found in the MAC13 tumor extracts. These findings suggest that cachexia may arise from the enhanced expression of a lipolytic factor associated with tumor cells.

Does carotid restenosis predict an increased risk of late symptoms, stroke, or death? The identification of carotid restenosis as an unexpected late complication of carotid endarterectomy has prompted concerns regarding its importance as a source of new cerebral symptoms, stroke, and death. To investigate these concerns, we analyzed a consecutive series of 507 patients undergoing 566 carotid endarterectomies, each documented as technically satisfactory. Post-operative duplex Doppler examination data at 3 days, 1, 3, 6, 12 months, and annually thereafter in 484 arteries (85.5%) permitted classification of these arteries according to the most severe degree of postoperative stenosis: normal (n = 306); 1% to 19% (n = 89); 20% to 50% (n = 40); more than 50% (n = 49, including 8 occluded). The incidence of more than 50% restenosis was 14.5% in female and 7.7% in male patients (p = 0.003). Life table analyses to 10 years revealed a significantly greater life expectancy among those with restenosis (p = 0.05). Stroke was also less likely in patients with restenosis, although this difference did not reach statistical significance. When survival and stroke were both endpoints, the likelihood of patients with more than 50% restenosis remaining alive and stroke free was also greater than the less than 20% stenotic group (p = 0.03). Thus patients with carotid restenosis were less likely than patients with normal postoperative scans to have late symptoms, stroke, or early death.

The shoulder in multiple epiphyseal dysplasia. One hundred shoulders were assessed in 50 patients with multiple epiphyseal dysplasia, of which one-third were found to have bilateral symptoms. Two distinct clinical and radiological groups emerged. Those with minor epiphyseal abnormalities developed painful osteoarthritis in middle age, but retained shoulder movement until the secondary changes were advanced. Those with 'hatchet head' shoulders likewise became painful in the fifth and sixth decades but had minimal glenohumeral movement from an early stage.

The management of postoperative chylous ascites. A case report and literature review. Chylous ascites remains rare as a complication of surgical intervention. Although therapeutic manoeuvres to control chylous ascites have been developed over the past 20 years, its pathophysiology is poorly understood. Conservative approaches involving dietary restriction of long-chain triglycerides and salt, together with multiple paracenteses, are still the therapies of choice. Persistent chylous ascites may be treated surgically by ligation of leaking lymphatics or implantation of a peritoneovenous shunt once the lymphatic anatomy has been defined by preoperative investigations. We report a case of chylous ascites developing after laparotomy and duodenotomy for obscure gastrointestinal bleeding, which did not respond adequately to medical therapy and was successfully managed by the insertion of a peritoneovenous shunt.

Pharmacomechanical thrombolysis and angioplasty in the management of clotted hemodialysis grafts: early and late clinical results. The results of pharmacomechanical thrombolysis and angioplasty of 121 thrombosed hemodialysis grafts were reviewed. The initial pharmacomechanical method (used in 65 cases) employed clot maceration with hook-shaped catheters and clot lacing with highly concentrated urokinase. The current technique (used in 56 cases) consisted of pulsed-spray injection of urokinase into the clot. All fully treated grafts (117 cases) underwent complete or near-complete thrombolysis, and 93% remained patent after 1 day. Mean time for pulsed-spray lysis was 46 minutes +/- 21. One patient (less than 1%) had gastrointestinal bleeding and received a transfusion; minor complications occurred in 3% of patients. Primary and secondary graft patency rates for both methods at 1 year were 26% and 51%, respectively. While graft age and results of angioplasty did not influence future graft patency, shorter intervals between graft thromboses was predictive of earlier subsequent graft failure. Results suggest that pharmacomechanical thrombolysis and angioplasty provide rapid, consistent, and safe recanalization of clotted hemodialysis grafts and represent a promising additional therapeutic approach to long-term graft management.

Color Doppler diagnosis of mechanical prosthetic mitral regurgitation: usefulness of the flow convergence region proximal to the regurgitant orifice. In prosthetic or paravalvular prosthetic mitral regurgitation, transthoracic color Doppler flow mapping can sometimes fail to detect the regurgitant jet within the left atrium because of the shadowing by the prosthetic valve. To overcome this limitation, we assessed the utility of color Doppler visualization of the flow convergence region (FCR) proximal to the regurgitant orifice in 20 consecutive patients with mechanical prosthetic mitral regurgitation documented by surgery and cardiac catheterization (13 of 20 patients). In addition, we studied 33 patients with normally functioning mitral prostheses. Doppler studies were performed in the apical, subcostal, and parasternal long-axis views. An FCR was detected in 95% (19 of 20) of patients with prosthetic mitral regurgitation. A jet area in the left atrium was detected in 60% (12 of 20) of patients. In 18 of 19 patients with Doppler-detected FCR, the site of the leak was correctly identified by observing the location of the FCR. A trivial jet area was detected in eight patients with a normally functioning mitral prosthesis; in none was an FCR identified. Thus color Doppler visualization of the FCR proximal to the regurgitant orifice is superior to the jet area in the diagnosis of mechanical prosthetic mitral regurgitation. Moreover, FCR permits localization of the site of the leak with good accuracy.

Noninvasive diagnosis of right-sided extracardiac conduit obstruction by combined magnetic resonance imaging and continuous-wave Doppler echocardiography. Right-sided extracardiac conduits are frequently complicated by obstruction over time. We compared the utility of two-dimensional and Doppler echocardiography and magnetic resonance imaging in the diagnosis of postoperative right-sided obstruction with cardiac catheterization and angiography in 10 patients with xenograft or homograft conduits. Correlation (r = 0.95) between continuous-wave Doppler estimates and catheter pullback pressure gradients across the conduits was excellent. Echocardiography could only visualize five of 10 conduits in their entirety. Magnetic resonance imaging visualized all conduits and showed statistically significant (kappa = 0.58) agreement with angiography in the localization and estimation of severity of a variety of right-sided obstructions in these patients. However, flow voids created by the metallic ring around xenograft valves led to a false negative diagnosis of valvular stenosis in four patients when magnetic resonance imaging was used alone. Doppler studies correctly indicated obstruction in these patients. The combination of magnetic resonance imaging studies and continuous-wave Doppler echocardiography can be useful to noninvasively evaluate right-sided obstruction in postoperative patients with right-sided extracardiac conduits.

Sodium sensitive and sodium retaining hypertension. The differences between sodium sensitive and sodium retaining hypertension were theoretically considered using a water tank model of body fluid volume-blood pressure regulation. If an outlet valve is attached to a tank with a base area corresponding to the reciprocal of total peripheral resistance (TPR) and water is poured into this tank at a rate corresponding to the amount of Na+ intake, then equilibrium should be achieved at a certain water level, volume and output from the outlet, which represent mean arterial pressure (MAP), cardiac output (CO) and urinary Na+ excretion. The height of the outlet from the tank bottom and the size cross-sectional area, of the outlet correspond to the x-intercept and slope of the renal function (pressure-natriuresis) curve, respectively. In both nonsodium sensitive hypertension, due to the shift of the curve toward a higher blood pressure level (elevated height of the outlet) without change in the slope (size of the outlet), and sodium sensitive hypertension, due to the depressed slope of the curve (reduced outlet size), not only MAP (water level) but also CO (water volume) are increased, resulting in sodium retaining hypertension, if TPR (reciprocal of base area) remained unchanged, while CO is relatively unchanged, resulting in nonsodium retaining hypertension, if TPR is elevated. Thus, the MAP and its sensitivity to sodium intake is determined by the renal function curve. Since body fluid volume is determined by both the renal function curve and TPR, however, changes in TPR during the development of hypertension is a major factor in determining whether or not the body fluid volume has to change only a small amount or a large amount. Therefore, the sodium sensitivity of blood pressure and sodium retention must be considered separately.

Imaging bone tumors in the 1990s. Progress in bone tumor management has occurred as a result of cooperation among surgeons, oncologists, pathologists, and radiologists. During the 1990s radiologists will contribute to care of patients with bone tumors in major ways. Tumor detection and preliminary diagnosis will be accomplished by radiography. Tumor local extent will be assessed by magnetic resonance imaging (MRI) and to a lesser degree by computed tomography (CT). Distant spread of malignancy will be documented by radionuclide scintigraphy (skeleton) and by CT (lungs). The combined estimate of local extent and distant spread will assure adequate staging before definitive management decisions. Preoperative closed percutaneous biopsy for histologic diagnosis will be accomplished on an outpatient basis under fluoroscopic or CT guidance. Arteriography will be employed for delivery of local chemotherapy. Some combination of arteriography, MRI, and MR spectroscopy will be used to evaluate tumor response. After limb-salvage surgery, MRI will sequentially assess the tumor bed; bone scintigraphy and CT will detect skeletal and pulmonary metastases. The radiologist's role will undergo continuous redefinition.

Closure of a bronchopleural fistula with bronchoscopic instillation of tetracycline. Persistent bronchopleural fistulas (BPF) due to infection, trauma, or thoracic surgical procedures are often difficult to manage. We report a patient with fulminant Staphylococcus aureus pneumonia complicated by chronic BPF formation which prevented weaning from mechanical ventilation due to severe air leak. Fistula closure was obtained by instillation of tetracycline into the fistula via a fiberoptic bronchoscope using a balloon catheter and blood clot occlusion technique. This closed the BPF and allowed successful weaning from mechanical ventilation.

Utility of frozen-section evaluation of lymph nodes in the staging of bronchogenic carcinoma at mediastinoscopy and thoracotomy. We conducted a retrospective analysis of the utility of frozen-section diagnoses in determining lymph node status at mediastinoscopy in 122 consecutive patients with bronchogenic carcinoma. Thirty-five of 122 patients had one or more lymph nodes with frozen-section evaluation positive for metastatic carcinoma. Subsequent nodal sections not in the original frozen-section study revealed metastatic carcinoma in two additional patients. The false-negative rate was 1.6%. Sensitivity was 94.6%. Predictive value of negative frozen-section evaluation results was 97.7%. Because there were no false-positive frozen-section results, specificity and predictive value for positive results of frozen-section evaluation were 100%. The statuses of individual lymph nodes from these 122 patients were also evaluated. Six hundred twenty lymph nodes were sampled from the mediastinum at mediastinoscopy. Frozen-sections in 47 lymph nodes were positive. Subsequent nodal sections not in the original frozen-sections examination revealed metastatic carcinoma in four additional lymph nodes. The false-negative rate was 0.6%. Sensitivity was 92.2%. Predictive value of negative results from frozen-section evaluation was 99.3%. Because there were no false-positive frozen-section results, specificity and predictive value for positive results of frozen-section examination were 100%. We conclude that frozen-section evaluation of lymph nodes at mediastinoscopy reliably indicates lymph node status, thus enabling the physician to decide whether to proceed to thoracotomy. Thus staging of the carcinoma and definitive surgery can be accomplished during a single anesthetic procedure. Combining mediastinoscopy and thoracotomy with frozen-section diagnostic control also reduces both the length and cost of hospitalization.

Antihypertensive drugs and glucose metabolism: comparison between a diuretic, a beta-blocker and felodipine, a new calcium antagonist in subjects with arterial hypertension and diabetes. Felodipine, a new antihypertensive calcium antagonist, was compared with metoprolol, a beta-blocker, and hydrochlorothiazide, a diuretic, with respect to glucose tolerance in a randomized double-blind cross-over study consisting of 11 patients, of age range 50-70 years, who had developed diabetes during antihypertensive treatment. Each treatment period lasted for 10 weeks. The blood pressure was similar irrespective of treatment. Serum glucose levels during the oral glucose tolerance test were significantly lower when the patients were treated with felodipine than when they were taking hydrochlorothiazide. Serum insulin levels appeared to decrease at an earlier stage of the test when the patients were treated with felodipine, the calcium antagonist, than with the other two antihypertensive substances, which suggests that glucose tolerance is impaired to a lesser extent during treatment with the calcium antagonist. Glucose tolerance was never completely normalized with any of the drugs tested.

Gallbladder perforation: correlation of cholescintigraphic and sonographic findings with the Niemeier classification. We retrospectively analyzed the cholescintigrams and sonograms of 36 consecutive patients with gallbladder perforation to (a) determine the sensitivity of each for the preoperative detection of gallbladder perforation and (b) correlate the findings with the modified Niemeier classification. Cholescintigraphic criteria of perforation (free spill, pericholecystic hepatic activity, and scintigraphic gallstone ileus sign) were detected in 14 of 28 (50%) cases, while sonographic criteria of perforation (pericholecystic fluid or pneumobilia with gallstones) were present in 18% (4 of 22) of patients (p less than 0.05). Cholescintigraphic patterns of perforation associated with the Niemeier classification were: Type I (acute free perforation), 3 of 7 scans demonstrated free spill; Type II (subacute pericholecystic abscess), 9 of 19 scans showed pericholecystic activity; and Type III (chronic cholecystoenteric fistula), 1 of 3 scans showed a scintigraphic gallstone ileus. Thus, although cholescintigraphy appears superior to sonography, both modalities are relatively insensitive for the detection of gallbladder perforation.

Left ventricular fibroma: echocardiographic diagnosis and successful surgical excision in three cases. The management of three patients with left ventricular fibromas is outlined. All were asymptomatic children. Routine chest radiography suggested cardiac masses. M-mode and two-dimensional echocardiography were valuable adjuncts to conventional angiography in assessing these children. Electrocardiographic changes, present in all cases, were shown to regress postoperatively. We stress the importance of these noninvasive aids in the initial investigation and outline our operative methods of reconstruction.

Tongue ischemia from a soft-drink can: report of case. A young girl was brought to the emergency department because her tongue became entrapped while she was drinking from an aluminum soft-drink can. A dental drill was used to remove the can. The tongue was observed for 48 hours, during which an ischemic area recovered spontaneously.

In vitro and in vivo effects within the coronary sinus of nonarcing and arcing shocks using a new system of low-energy DC ablation. DC shocks within the coronary sinus have been abandoned because of the risk of cardiac rupture and tamponade. Catheter ablation using DC energy to electrodes straddling the ostium of the coronary sinus, when used clinically, has been reported to result in cardiac tamponade in as many as 16% of patients. A new system of energy delivery maximizes voltage while decreasing the undesirable effects caused by barotrauma. This system includes 1) a low-energy ablation power supply with a brief time-constant capacitive discharge that delivers up to 40 J and 3,000 V and 2) a low-energy ablation catheter with a contoured distal electrode. We performed in vitro and in vivo studies of this new system and compared arcing shocks with nonarcing shocks. Ablations were performed using unipolar distal shocks (D) and unipolar shocks to both electrodes made electrically common (P-D). In vitro studies were done in a large tank filled with physiological saline while recording voltage, current, and pressure. High-speed cinematography (32,000 frames/sec) of shocks of 10-40 J permitted detailed analysis of the vapor globe. Anodal shocks of less than 20 J showed no arcing or only minimal vapor globe formation. For D and P-D anodal shocks of 40 J, the diameters of the vapor globe were 31 and 22 mm, respectively, corresponding to pressure recordings of 11 and 4.9 atm. The pressure rise lasted less than 50 mu sec. In vivo studies involved 18 dogs that received nonarcing shocks (one to six shocks of 15 J) and 18 dogs that received arcing shocks (one to three shocks of 40 J). Each group was divided between D and P-D shocks; catheter ablation was performed at a mean +/- SEM distance of 2.94 +/- 0.92 cm within the coronary sinus. All dogs tolerated the procedure without cardiac rupture or tamponade. When killed 2-4 days later, the dogs had edema and hyperemia or hemorrhage in the area of the coronary sinus. We compared the effects of multiple (three to six) nonarcing shocks with the effects of one to three arcing shocks. Disruption or rupture of the coronary sinus within the epicardial fat space occurred in two of 12 dogs (17%) with multiple nonarcing shocks but in 13 of 18 dogs (72%) with arcing shocks (p less than 0.003). Occlusion of the coronary sinus occurred in two of 12 dogs (17%) with multiple nonarcing shocks and in nine of 18 dogs (50%) with arcing shocks (p less than 0.06).(ABSTRACT TRUNCATED AT 400 WORDS).

Chondrosarcoma of the jaws: clinical findings, histopathology, and treatment. Three cases of chondrosarcoma involving the jaws are presented, one in the maxilla and two in the mandible. The salient points of clinical presentation elucidated by this series of cases are that a widened periodontal ligament space is present in chondrosarcomas as well as in osteosarcomas, and that a slowly increasing diastema may be the earliest clinical sign. The most important lesson to be learned from the histopathology is that one should not accept a diagnosis of a benign cartilaginous tumor of the jaws. Treatment of these lesions should consist of wide surgical excision and consideration of adjunctive or palliative radiotherapy, especially in the maxilla. It should also be noted that recurrences may develop 10 to 20 years later, and follow-up should be lifelong.

Ear involvement in the yellow nail syndrome. Recognized features of the yellow nail syndrome include yellow nails, lymphedema, and pleural effusions. We report a patient with the additional feature of keratosis obturans, which may be a manifestation of this syndrome in the external ear.

A modified rhombic transposition flap and its application in dermatology. A modified rhombic transposition flap based around a circular primary defect is described. We have found it to be a versatile design that produces good cosmetic results with a low incidence of postoperative complications. It has become the most frequently used transposition flap in our dermatologic practice.

Hepatic injury associated with small bowel bacterial overgrowth in rats is prevented by metronidazole and tetracycline. Susceptible rat strains develop hepatobiliary injury following the surgical creation of self-filling blind loops that cause small bowel bacterial overgrowth. Luminal bacteria or their cell wall polymers were implicated in the pathogenesis of the lesions because sham-operated rats and rats with self-emptying blind loops, having only slightly increased bacterial counts, did not develop hepatic injury. In this study, antibiotics with different spectra of activities were continuously administered starting 1 day or 22 days after surgery to determine which intestinal flora may be responsible for the development of hepatic injury in rats with small bowel bacterial overgrowth. Four weeks following surgery, Lewis rats with self-filling blind loops receiving no antibiotics had elevated liver histology scores (8.2 +/- 1.3 vs. 0.7 +/- 0.4) and plasma aspartate aminotransferase levels (269 +/- 171 vs. 84 +/- 24) compared with sham-operated rats, P less than 0.001. Oral gentamicin as well as oral and intraperitoneal polymyxin B, which binds endotoxin, did not prevent hepatic injury in rats with self-filling blind loops. However, oral metronidazole and tetracycline therapy continuously administered beginning 1 day after surgery diminished hepatic injury (histology score 3.0 +/- 1.8, 2.9 +/- 1.1; aspartate aminotransferase 87 +/- 25, 98 +/- 34; respectively P less than 0.001 compared with self-filling blind loops receiving no antibiotics). Metronidazole also protected Wistar rats that require 12 weeks to develop hepatic injury following experimentally induced small bowel bacterial overgrowth compared with rats with self-filling blind loops that received no antibiotic treatment (histology score 10.4 +/- 1.3 vs. 0.7 +/- 1.1, and aspartate aminotransferase 273 +/- 239 vs. 76 +/- 20, P less than 0.001). When rats started metronidazole therapy 22 days after self-filling blind loop surgery, elevated aspartate aminotransferase values decreased to normal during the next 77 days and final histology scores were normal. All rats with self-filling blind loops had negative peritoneal, liver, spleen, and blood cultures but approximately 75% of mesenteric lymph node cultures were positive irrespective of antibiotic treatment. Because Bacteroides species have been implicated in causing vitamin B12 and disaccharidase deficiencies in rats with self-filling blind loops, we documented the presence or absence of these organisms from blind loops using selective culture techniques. Metronidazole and tetracycline eliminated Bacteroides sp. from blind loops, but polymyxin B and gentamicin did not.(ABSTRACT TRUNCATED AT 400 WORDS).

Clear cell adenocarcinoma of the vagina and cervix. A report of the Central Netherlands Registry with emphasis on early detection and prognosis. The Central Netherlands Registry (CNR) of women with vaginal or cervical clear cell adenocarcinoma (CCAC) was established in 1985. An overview is presented of clinical and pathologic data of 55 patients who were registered at the CNR until July 1, 1988. All Netherlands Departments of Pathology (NDP) maintain a patient registry and 95% of the Institutes are connected with a Central Archive via a computer network. The histologic slides and clinical status were reviewed at the CNR. Twenty-five tumors were classified as vaginal carcinoma and 30 as cervical carcinoma. The mean age of the patients was 22 years. Fifty-five percent of patients (63% of patients with known maternal history) were exposed to diethylstilbestrol (DES) in utero. The majority of cases was initially diagnosed after 1980. Cytologic examination before the initial histologic diagnosis indicated that cervical tumors were detected in 80% of cases, but vaginal tumors were detected only in 33% of cases. It was concluded that an examination of DES-exposed women should consist of colposcopic inspection of the cervix and vagina, cytologic examination of the cervix and four quadrants of the vagina, and careful palpation of the cervix and the entire vaginal wall. The most important prognostic parameter for patients with CCAC was stage and grade of nuclear atypia. The results of a statistical analysis showed that these features were the most effective to distinguish between nonsurvivors and patients surviving more than 5 years.

Polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudo-obstruction: POLIP syndrome. We describe 5 individuals (from three separate families) with a progressive neurological disorder characterized by sensorimotor peripheral polyneuropathy, cranial neuropathies (external ophthalmoplegia, deafness), and the syndrome of chronic intestinal pseudo-obstruction. Magnetic resonance imaging showed widespread abnormality of the cerebral and cerebellar white matter in the 2 patients studied. Autopsy examination in 3 revealed widespread endoneurial fibrosis and demyelination in the peripheral nervous system, possibly secondary to axonal atrophy, and poorly defined changes in cerebral white matter (leukoencephalopathy). The cranial nerves and spinal roots were less severely involved and the neurons in the brainstem and spinal cord were intact. The fatal gastrointestinal dysmotility was due to a severe visceral neuropathy. We suggest that these patients manifested a hereditary disorder with distinctive clinical, radiological, and neuropathological features, and propose the acronym POLIP to emphasize the distinctive tetrad of polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudo-obstruction.

Insulin resistance in noncirrhotic idiopathic portal hypertension. To explore further the pathogenesis of glucose intolerance and insulin resistance observed in patients with cirrhosis and portal hypertension, we studied a 35-year-old woman with presinusoidal portal hypertension without cirrhosis due to nodular regenerative hyperplasia of the liver. After oral glucose ingestion, glucose tolerance remained normal; however, this occurred at the expense of a markedly hyperinsulinemic plasma response, suggesting the presence of insulin resistance. To examine this question more directly, we performed a stepwise euglycemic insulin clamp study in combination with an infusion of [6-3H]glucose and [1-14C]palmitate and indirect calorimetry. The ability of insulin to promote total body (primarily muscle) glucose uptake was markedly impaired, whereas its effect to suppress hepatic glucose production was normal compared with results obtained in nine healthy subjects. Moreover, insulin failed to normally suppress plasma free fatty acid turnover and oxidation in this patient. This informative case demonstrates that portal hypertension alone, without hepatic dysfunction from cirrhosis, is associated with impaired insulin-mediated glucose and plasma free fatty acid metabolism and may also play a predominant role in the development of insulin resistance in many cirrhotic patients.

Effects of lactulose and lactitol on protein digestion and metabolism in conventional and germ free animal models: relevance of the results to their use in the treatment of portosystemic encephalopathy. Protein digestion and metabolism have been studied in laboratory rats and miniature pigs to investigate the mechanisms of action of lactulose and lactitol when used in the treatment of patients with portosystemic encephalopathy. Lactulose (beta-D-galactopyranosyl-(1----4)-beta-D-fructofuranose) and lactitol (beta-D-galactopyranosyl-(1----4)-D-glucitol) increased the excretion of nitrogenous material in the faeces and decreased nitrogen excretion in the urine in a similar degree to that reported for human patients. In studies with germ free rats given lactulose no such effect was observed, suggesting that, for lactulose at least, these effects are mediated by the gut flora. Measurement of the alpha-, epsilon-diaminopimelic acid content of the faeces confirmed that the enhancement of faecal nitrogen was due to an increased contribution from bacteria. The similarity in the results for lactulose and lactitol suggests that, from the perspective of protein metabolism, lactitol acts in a similar way to lactulose in the treatment of portosystemic encephalopathy.

Splenic artery aneurysm rupture [published erratum appears in Am J Emerg Med 1991 Mar;9(2):205] Splenic artery aneurysms are rare and usually asymptomatic. However, rupture can produce severe abdominal pain and prove lethal. Two cases of ruptured splenic artery aneurysm are presented. In one, cocaine injection occurred immediately prior to the onset of symptoms and may have precipitated the event.

Acute gastroenteritis in infants under 6 months old. Sixty two babies under the age of 6 months who were admitted with gastroenteritis completed a study of gradual refeeding compared with abrupt refeeding after a period of rehydration. There was no difference in the incidence of recurrence of diarrhoea due to lactose intolerance, effect on weight, or duration of hospital stay. Twenty six babies (42%) had recurrence of diarrhoea after refeeding, all of whom settled with the introduction of a lactose free soya based formula. Well nourished babies under 6 months of age with mild to moderately severe gastroenteritis can be fed immediately with full strength milk feeds after rehydration. The introduction of a lactose free soya based preparation may provide an alternative to repeated attempts at regrading with cows' milk feeds in those patients with lactose intolerance.

Dilatation in the duplex kidney: 3 unusual cases. Dilatation in the upper limb of a duplex ureter is usually secondary to obstruction and may be associated with a ureterocele. By contrast, vesicoureteric reflux is the usual cause of a dilated lower moiety ureter. We report 3 cases in which unusual or complex causes of dilatation of 1 limb of a duplex ureter were demonstrated.

Pregnancy in a patient with treated Wilson's disease: a case report. Pregnancy should have a successful outcome in a patient with treated Wilson's disease if complications are excluded before conception. Chelating treatment must be maintained, although there is some concern about its teratogenicity. We describe the course of pregnancy in a patient followed up in our department.

Dialysis-induced alterations in left ventricular filling: mechanisms and clinical significance. Quantitative two-dimensional (2-D) and Doppler echocardiography were used to determine whether hemodialysis results in alterations in left ventricular (LV) diastolic filling that might contribute to dialysis-induced hypotension, as well as to assess whether any hemodynamic variables or indices of diastolic filling might be used to identify which patients were at the greatest risk of becoming hemodynamically unstable during dialysis. Sixteen male patients undergoing routine maintenance hemodialysis for end-stage renal disease were prospectively studied before and after hemodialysis. Following hemodialysis there was a significant prolongation (P less than 0.05) in LV isovolumetric relaxation time (IVRT), as well as a significant reduction in the rate and extent of early rapid ventricular filling (P less than 0.005); in contrast, late atrial-assisted filling did not change significantly. A multiple stepwise linear regression analysis of predialysis hemodynamic parameters and noninvasive indices of LV filling showed that there was a significant independent inverse relationship between the frequency of dialysis-related hypotensive episodes and the duration of early LV filling (r = -0.81; P less than 0.001). These results suggest that hemodialysis results in discrete alterations in early LV filling, with no significant compensatory increase in late atrial-assisted ventricular filling. Further, patients with the shortest early LV filling times appeared to have the greatest predilection for becoming hemodynamically unstable during dialysis.

Ptychotropism as a cutaneous feature of the CHILD syndrome. The hallmark of the CHILD syndrome is a unilateral ichthyosiform nevus that displays a pronounced affinity for the body folds. For this phenomenon the term ptychotropism is proposed. The expression is composed from the Greek words ptyche (fold) and trope (a turning). It is concluded that the cutaneous lesions of the CHILD nevus are determined by two factors, lyonization and ptychotropism. The phenomenon of ptychotropism constitutes an additional argument to distinguish the CHILD nevus from the inflammatory linear verrucous epidermal nevus because the latter is not ptychotropic.

Microcystic adnexal carcinoma following radiotherapy in childhood. A 36-year-old man was treated by radiotherapy for tinea capitis many years before discovery of microcystic adnexal carcinoma (MAC). Because of patient's refusal of any surgical intervention, we were able to follow the natural course of this tumor for 13 years. This case emphasizes the typical slow development of (MAC). The implication of the association of MAC and radiotherapy are discussed.

Feasibility of digital teleradiology for imaging evaluation of patients with acute right upper quadrant abdominal pain. To assess the utility of a commercially available digital teleradiology system in evaluating patients with acute pain in the right upper quadrant, hard-copy images from 100 examinations (50 hepatobiliary scintigrams and 50 sonograms of the right upper quadrant) were digitized, transmitted via standard telephone lines, and viewed remotely on a video monitor. Video and hard-copy interpretations were then compared for degree of concordance. For the scintigraphic studies, hard-copy and video images were equal in demonstrating gallbladder and bile duct activity. Video images failed to depict the presence of bowel activity in one case. Gallstones were depicted equally well on hard-copy and video sonographic images. The video interpreters overestimated the presence of abnormal hepatic parenchyma and overlooked one case of right hydronephrosis. The video interpretations of the scintigrams and sonograms showed an overall error rate of 4%, comparable to the rate obtained when radiographs are interpreted remotely with digital teleradiology systems.

Long-term follow-up comparing subclavian flap angioplasty to resection with modified oblique end-to-end anastomosis The definitive surgical procedure for correction of aortic coarctation remains controversial. Therefore, we retrospectively reviewed a total of 56 children under 4 years of age with coarctation repair between 1977 and 1986. Thirty-four had the subclavian flap angioplasty technique and 22 had resection with oblique end-to-end anastomosis. The group was further subdivided to include only the 23 infants less than 3 months of age--eight infants with resection with oblique end-to-end anastomosis (less than or equal to 3ETE) and 15 infants with subclavian flap angioplasty (greater than or equal to 3SFA). The remaining 33 patients older than 3 months of age were divided into 14 patients with resection and oblique end-to-end anastomosis (greater than 3ETE) and 19 patients with the subclavian flap angioplasty technique (greater than 3SFA). The overall mortality was not significantly different between techniques. Postoperative hypertension was significantly more prevalent with end-to-end anastomosis than with the subclavian flap angioplasty technique (p less than 0.01). Seven patients had recurrent coarctation. The 6-year actuarial freedom from recoarctation was 93% +/- 6% in the less than or equal to 3SFA group compared with 53% +/- 20% in the less than or equal to 3ETE group (p less than 0.02), but there was no significant difference in those children operated on at a later age regarding the type of coarctation repair. Therefore, we recommend subclavian flap angioplasty in patients less than 3 months of age. In those older than 3 months either procedure is safe and the risk of recoarctation is similar.

Catheterization of coronary artery bypass graft from the descending aorta. The increasing frequency of reoperation for coronary artery disease has led to the use of a variety of grafts. This report describes the catheter technique for selective opacification of a saphenous vein graft from the descending thoracic aorta to the posterior coronary circulation.

Improved survival and reduced myocardial necrosis with cardiopulmonary bypass reperfusion in a canine model of coronary occlusion and cardiac arrest. STUDY QUESTION: Does cardiopulmonary bypass (CPB) improve resuscitation rates and limit infarct size after cardiac arrest and acute myocardial infarction? DESIGN: Controlled randomized trial with all animals undergoing left anterior descending coronary artery occlusion and subsequent ventricular fibrillation and resuscitation. All animals were supported for four hours after resuscitation in an intensive care setting. INTERVENTION: Group 1 (eight) was resuscitated with standard external CPR and advanced life support. Group 2 (eight) was resuscitated with CPB. MEASUREMENTS AND MAIN RESULTS: Group hemodynamic, resuscitation variables, number resuscitated, and number of four-hour survivors were compared. Ischemic and necrotic myocardial weights were determined with histochemical staining techniques in four-hour survivors. Infarct size was measured as the ratio of necrotic weight to ischemic weight. Significantly fewer dogs were resuscitated in group 1 (four of eight) than in group 2 (eight of eight) (P less than .05). Group 2 survivors required significantly less epinephrine and lidocaine than group 1 survivors (P less than .05) and higher aortic diastolic and coronary perfusion pressures after CPB (P less than .001). The ratio of myocardial necrotic weight to ischemic weight at four hours was 0.82 +/- 0.25 in group 1 and 0.22 +/- 0.25 in group 2 (P less than .05). However, collateral blood flow was not measured in this study. CONCLUSION: This pilot study further substantiates the improvement in resuscitation rates obtainable with CPB. CPB may also limit infarct size during the postresuscitation period and requires further study.

Psychosomatic factors in borderline hypertensive subjects and offspring of hypertensive parents. Psychosomatic factors, sympathoneural and sympathoadrenal as well as cardiovascular mechanisms, were studied in 24 patients 18-24 years of age with borderline hypertension, 50 age-matched normotensive offspring of hypertensive parents, and 49 controls with no family history of hypertension. They were compared by projective and questionnaire-based psychological tests and their circulatory and neurohormonal reactivity to mental (Stroop color-word conflict test and arithmetic test) and physical stressors (orthostasis and bicycle ergometry test) were measured. Borderline hypertensive subjects externalized aggression less (p less than 0.05) but internalized it more (p less than 0.05) and were more submissive (p less than 0.05) when compared with controls. Offspring of hypertensive parents showed a similar but weaker pattern. Both risk groups reported more positive interactions with their parents (genetic risk subjects versus controls, p less than 0.05; borderline hypertensive patients versus controls, p = 0.08) and had higher state-anxiety levels (p less than 0.05). There were more subjective symptoms of beta-adrenergic receptor-mediated functions (e.g., tachycardia, tremor) in borderline hypertensive subjects and offspring of hypertensive parents, elevated heart rates (analysis of repeated measures, p less than 0.001), and enhanced plasma norepinephrine concentrations (p less than 0.05) when compared with controls. These findings in subjects at risk for the development of hypertension suggest that psychosomatic factors and sympathetic overactivity are involved in the early phase of hypertension.

Therapy of renal cell carcinoma with interleukin-2 and lymphokine-activated killer cells: phase II experience with a hybrid bolus and continuous infusion interleukin-2 regimen. Forty-seven patients with metastatic or unresectable renal cell carcinoma were treated with interleukin-2 (IL-2) and lymphokine-activated killer (LAK)-cell therapy, using a hybrid IL-2 regimen. IL-2 was administered initially by intravenous bolus (10(5) U/kg [Cetus Corp, Emeryville, CA] every 8 hours for 3 days) during the priming phase, and subsequently by continuous infusion (3 x 10(6) U/m2 for 6 days); during this second treatment period, in vitro-generated LAK cells were administered. Despite selection of patients for good performance status (PS) (29, PS 0; 18, PS 1) prior nephrectomy (43 of the 47 patients), and low tumor burden, the response rate was low (two complete [CRs] and two partial responses [PRs], for an overall objective response rate of 9%). Toxicity was comparable to that experienced with the high-dose bolus regimen. These results suggest that the dose and schedule of IL-2 administration may influence the likelihood of response to IL-2 in renal cell carcinoma.

Clinical, radiographic, and hemodynamic correlations in chronic congestive heart failure: conflicting results may lead to inappropriate care. PURPOSE: Clinical and radiographic examinations are commonly used for estimating severity and titrating therapy of chronic congestive heart failure. The purpose of this study was to establish the relationship between findings on history, physical examination, chest roentgenogram, and pulmonary capillary wedge pressure (PCWP). PATIENTS AND METHODS: Fifty-two consecutive patients with chronic congestive heart failure, referred for evaluation for heart transplantation, were studied; all patients underwent history, physical examination, upright chest roentgenogram, and cardiac catheterization. The mean left ventricular ejection fraction was 0.19 +/- 0.06. Patients were divided into three groups according to their PCWP: Group 1, normal PCWP (less than or equal to 15 mm Hg, n = 19); Group 2, mild to moderately elevated PCWP (16 to 29 mm Hg, n = 15); Group 3, markedly elevated PCWP (greater than or equal to 30 mm Hg, n = 18). RESULTS: Physical and radiographic signs of congestion were more common in the groups with higher PCWP, but they could not be used to reliably separate patients with different filling pressures. Physical findings (orthopnea, edema, rales, third heart sound, elevated jugular venous pressure) or radiographic signs (cardiomegaly, vascular redistribution, and interstitial and alveolar edema) had poor predictive value for identifying patients with PCWP values greater than or equal to 30 mm Hg. These findings had poor negative predictive value to exclude significantly elevated PCWP (greater than 20 mm Hg). Radiographic pulmonary congestion was absent in eight (53%) patients in Group 2 and seven (39%) in Group 3. In patients in Group 2 and 3, those without radiographic congestion were in a better New York Heart Association functional class (3.5 +/- 0.5 versus 2.8 +/- 0.6, p less than 0.01). There was good correlation between right atrial pressure and PCWP (r = 0.64, p less than 0.001). A normal right atrial pressure had no predictive value, but a pressure greater than 10 mm Hg was seen in all but one patient with a PCWP value greater than 20 mm Hg. CONCLUSION: Clinical, radiographic, and hemodynamic evaluations of chronic congestive heart failure yield conflicting results. Absence of radiographic or physical signs of congestion does not ensure normal PCWP values and may lead to inaccurate diagnosis and inadequate therapy. It is not known whether therapy aimed at normalizing PCWP is superior to relieving clinical and radiographic signs of congestion.

Prognostic significance of type IV collagen and laminin immunoreactivity in urothelial carcinomas of the bladder. Invasion of a carcinoma involves the degradation and penetration of the subepithelial basement membrane (BM). This phenomenon might be used for histopathologic evaluation of neoplasms of the bladder. The authors studied the clinicopathologic data and tissue specimens of 125 cases of urothelial carcinomas collected prospectively. Penetration of the BM was evaluated by immunohistochemical staining of the BM components laminin and type IV collagen. The use of this parameter as a prognostic indicator in bladder cancer was assessed. The 5-year survival rate of patients having tumors with an interrupted or absent BM was significantly lower than that of patients having tumors with an intact BM. The rate of progression was greater in tumors with an interrupted or absent BM than in tumors with an intact BM. No association was found between BM status and recurrence. However, a significant correlation between tumor stage and BM staining was found. A correlation was also found between ploidy and BM staining as well as between histologic grade and BM staining pattern. When evaluating histologic grade, stage, ploidy, age, and BM score as prognostic parameters, the stage of bladder carcinomas turned out to be the most important factor in predicting the survival rate and the progression-free survival. However, BM staining was found to be of value for early identification of microinvasion and is helpful for correct staging of urothelial carcinomas.

Protein phosphorylation during ischemia. Many investigations have shown that calcium and adenosine triphosphate are crucial to central nervous system functions. It is probable that alterations of these substances during central nervous system ischemia are involved in the processes that cause irreversible neural damage. Calcium regulates several protein kinases that are responsible for phosphorylation of proteins vital for many central nervous system functions. Using a rabbit spinal cord ischemia model, we found protein kinase C and calcium/calmodulin-dependent kinase were severely affected during the first hour of ischemia. Protein kinase A was not significantly affected. The time course of lost protein kinase C enzyme activity closely corresponded to irreversible loss of neurologic function, and there is evidence that protein kinase C inhibitor activity is generated. Also, drugs that inhibit protein kinase C increased neurologic damage when administered during the early phases of ischemia. These results suggest that protein phosphorylation, particularly by protein kinase C, is critical to maintenance of neurologic function.

Bimanual simultaneous motor performance and impaired ability to shift attention in Parkinson's disease. The ability to share time and to shift attention between bimanual simultaneous motor tasks were studied in 18 patients with Parkinson's disease (PD) and 19 age- and intelligence-matched controls. The task consisted of drawing triangles with the dominant hand and squeezing a rubber bulb with the nondominant hand. Motor performance was measured using the variables: amplitude of squeezing, frequency of squeezing and velocity of drawing triangles. After eliminating variance due to baseline differences in single-handed performance, the bimanual simultaneous performance of PD and controls turned out to be similar to the frequency of squeezing and the velocity of drawing triangles. The amplitude of squeezing, however, differed between the two groups: it was significantly reduced in PD. Arguably the disturbance in the bimanual performance of PD patients was not due to a disorder of time sharing, but to a decreased ability to shift attention from the visually cued task to the non visually cued task. The results agree with current evidence that PD patients are more impaired when they have to rely upon internal control for the regulation of shifting attention than when external cues are available.

Use of neuroleptic-induced extrapyramidal symptoms to predict future vulnerability to side effects. OBJECTIVE: Susceptibility to neuroleptic-induced extrapyramidal syndromes varies widely, even within age and sex subgroups. Individual vulnerability to extrapyramidal syndromes has been assumed to explain this, but the utility of past history for predicting future occurrence of extrapyramidal syndromes has not been studied extensively. This investigation was undertaken to determine whether patients' previous histories of extrapyramidal syndromes predict future episodes of extrapyramidal syndromes and to compare the importance of this predictive factor with patient age, sex, neuroleptic dose, and anticholinergic dose as predictors of extrapyramidal syndromes. METHODS: The charts of 62 schizophrenic patients with multiple neuroleptic treatment episodes were reviewed. Extrapyramidal syndromes, neuroleptic drug doses, and anticholinergic drug doses during the first 21 days of each treatment episode were recorded. RESULTS: Previous extrapyramidal syndromes correctly predicted extrapyramidal syndromes in subsequent treatments for 84% of the patients. Variations in neuroleptic potency, neuroleptic dose, and anticholinergic dose partially explained incorrect predictions. CONCLUSIONS: These results support the hypothesis that patients with a history of extrapyramidal syndromes are at greater risk for future extrapyramidal syndromes. If confirmed, these results strongly support individual susceptibility as a major predictor of extrapyramidal syndromes and indicate that prophylaxis of extrapyramidal syndromes should be considered for patients who have previously suffered extrapyramidal syndromes from similarly prescribed neuroleptic therapy.

All-trans retinoic acid in acute promyelocytic leukemias. II. In vitro studies: structure-function relationship. All-trans retinoic acid induces leukemic cells from patients with acute promyelocytic leukemia (M3) to differentiate in vitro to mature granulocytes which express the CD15 antigen and are capable of respiratory burst function. Of 35 M3 samples, only one failed to respond. In eight cases, we compared the efficacy of two naturally occurring isomers of retinoic acid, all-trans RA and 13-cis RA. Both isomers induce maximal differentiation at 10(-6) mol/L. The maximal response was maintained at 10(-7) mol/L for the all-trans but not for the 13-cis RA. We also observed that the metabolites 4-oxo-all-trans and 4-oxo-13-cis were effective at 10(-6) mol/L. This 1 order of magnitude difference in the in vitro differentiating potencies of all-trans RA and 13-cis RA in the blasts of promyelocytic leukemias predicts a difference in the clinical efficacy of the two drugs.

The metabolic consequences of a "washed" cardiopulmonary bypass pump-priming fluid in children undergoing cardiac operations. The substrate load of the pump-priming fluid, especially glucose and lactate, has a major influence on the metabolic response of children during cardiac operations and may be detrimental neurologically. It is possible to cleanse the priming fluid of these excess substrates by ultrafiltration followed by the addition of a balanced electrolyte solution. We have used this technique in the bypass management of nine children over 1 year of age and weighing less than 18 kg who were studied from induction of anesthesia to 6 hours postoperatively. Frequent blood samples were taken for measurement of electrolytes, intermediary metabolites, and stress-related hormones. Throughout the study period electrolyte concentrations were maintained within the physiologic range and changes in blood glucose and lactate were minimized compared with those of matched historical controls from previous studies. During the period of cardiopulmonary bypass mean serum glucose concentrations varied between 4.4 and 7.7 mmol/L and peaked at 10.7 mmol/L just before skin closure. The mean serum lactate concentration did not exceed 2.3 mmol/L during the period studied. Thus ultrafiltration of the priming fluid and replacement with a balanced electrolyte solution results in the delivery to the patient of a reasonably physiologic substrate load, which is reflected in improved control of mean plasma intermediary metabolite concentrations. The method may form the basis of further clinical studies to determine specific aspects of the metabolic stress response in children.

Cystic medionecrosis of the coronary arteries and fatal coronary vasospasm. The histopathological basis of coronary vasospasm is not well defined. We report a patient with directly observed coronary artery spasm in whom cystic medionecrosis of the coronary arteries and great vessels and myxomatous degeneration of the mitral valve were evident. We suggest that myxoid connective tissue lesions of the heart may be linked to coronary vasospasm.

Cognitive and behavioural impairment among elderly people in institutions providing different levels of care. OBJECTIVE: To compare the prevalence and degree of cognitive and behavioural impairment in elderly patients in institutions providing different levels of care. DESIGN: Prevalence study. SETTING: A nursing home, a home for the aged and psychogeriatric wards in a provincial psychiatric hospital. PATIENTS: Only subjects 65 years of age or older were eligible for inclusion. A random sample was selected comprising 25% of the residents in the nursing home and the home for the aged; of the 119 asked to participate 95 agreed (44 in the nursing home and 51 in the home for the aged). All 50 on the psychogeriatric wards agreed to participate. MAIN OUTCOME MEASURES: The Mini-Mental State Examination (MMSE) and the Kingston Dementia Rating Scale (KDRS). RESULTS: An MMSE score of less than 24 (cognitive impairment) was given to 37 (84%) of the residents in the nursing home, 43 (84%) of those in the home for the aged and 48 (96%) of the patients in the psychiatric hospital; the corresponding numbers for a KDRS score of more than 0 (cognitive impairment) were 41 (93%), 48 (94%) and 50 (100%). The seven patients receiving the highest level of care at the home for the aged (special care) had more behavioural problems than those in the psychiatric hospital did (p less than 0.001). CONCLUSIONS: Cognitive and behavioural impairment was widespread in the three institutions regardless of the level of care. When planning services and allocating resources government funding agencies should consider the degree and prevalence of such impairment among elderly people in institutions.

Mapping of the locus for X-linked cardioskeletal myopathy with neutropenia and abnormal mitochondria (Barth syndrome) to Xq28. X-linked cardioskeletal myopathy with neutropenia and abnormal mitochondria is clinically characterized by congenital dilated cardiomyopathy, skeletal myopathy, recurrent bacterial infections, and growth retardation. We analyzed linkage between the disease locus and X-chromosomal markers in a family with seven carriers, four patients, and eight unaffected sons of carriers. Highest lod scores obtained by two-point linkage analysis were 2.70 for St14.1 (DXS52, TaqI) at a recombination fraction of zero and 2.53 for cpX67 (DXS134) at a recombination fraction of zero. Multipoint linkage analysis resulted in a maximum lod score of 5.24 at the position of St35.691 (DXS305). The most distal recombination detected in this family was located between the markers II-10 (DXS466) and DX13 (DXS15). These data indicate the location of the mutated gene at Xq28.

Overdose reperfusion of blood cardioplegic solution. A preventable cause of postischemic myocardial depression. Reperfusion of warm blood cardioplegic solution is useful in minimizing reperfusion damage after ischemia. This study tests the hypothesis that overzealous administration of blood cardioplegic solution at reperfusion counteracts these benefits and can lead to a prevalence of depressed ventricular performance and mortality similar to that seen after normal blood reperfusion. Thirty-one dogs underwent 45 minutes of 37 degrees C global ischemia on vented bypass. Six received normal blood reperfusion and 25 were reperfused with a warm aspartate/glutamate-enriched blood cardioplegic solution; of these, eight received high-dose (3600 +/- 600 ml) and 17 received limited-dose (1180 +/- 120 ml) blood cardioplegic reperfusion over 10 to 20 minutes. High-dose blood cardioplegic perfusion (5100 +/- 200 ml) without prior ischemia was tested in an additional five dogs. High-dose blood cardioplegia without preceding ischemia did not alter ventricular function (peak stroke work index 96% of control). After ischemia, normal blood reperfusion (no cardioplegia) resulted in marked left ventricular dysfunction (peak stroke work index 36% of control, p less than 0.05 versus control) and a 33% mortality rate (2/6 died). High-dose cardioplegic reperfusion yielded marginal recovery of stroke work index (40% of control, p less than 0.05 versus control) and a 25% mortality rate (2/8 died). In contrast, limited-dose reperfusion of blood cardioplegic solution allowed 100% survival (17/17) and restored stroke work index to 90% of control (1.3 versus 1.45 gm.m/kg). We conclude that reperfusion damage can be avoided by initial reoxygenation with limited doses of substrate-enriched blood cardioplegic solution. Conversely, high-dose reperfusion of blood cardioplegic solution offsets this benefit, reduces recovery substantially, and may be lethal.

Primary intratemporal tumours of the facial nerve: diagnosis and treatment. Benign primary tumours of the facial nerve are uncommon. A slowly progressive facial palsy should be considered the result of a nerve tumour until proven otherwise. Improvements in diagnostic imaging techniques of the temporal bone have increased the possibility of a correct pre-operative diagnosis but facial nerve tumours remain a frequently ignored or misdiagnosed entity as a consequence of their subtle and protean clinical manifestations. A series of 12 cases of primary facial nerve tumours is presented. The clinical features, diagnostic modalities and treatment are discussed in relation to a review of the literature.

Palliation of malignant esophageal obstruction. Progressive dysphagia is common in patients with advanced esophageal carcinoma. Multiple nonsurgical techniques are available to provide palliation and improved nutrition. Simple dilatation is the oldest technique and newer methods may offer greater efficacy. Laser therapy now provides an excellent opportunity to treat obstructing tumors. Placement of an esophageal prosthesis may become particularly useful when dilatation must be performed too frequently or has become ineffective or in the patient with an esophageal-pulmonary fistula. Newer techniques including BICAP tumor probe, intracavitary radiotherapy, or absolute alcohol injection offer promise. This review discusses the advantages and disadvantages of these different palliative techniques for patients with the extremely poor prognosis associated with esophageal cancer.

Coronary trapping of a complement activation product (C3a des-Arg) during myocardial reperfusion in open-heart surgery. Accumulation of complement factors has been found to occur in the myocardium after infarction. We studied the possibility that the complement activation product C3a des-Arg is trapped within the coronary circulation during reperfusion of the ischemic myocardium. In 11 patients undergoing routine coronary artery bypass grafting, arterial blood was sampled before, during and after cardiopulmonary bypass. Blood was drawn from the coronary sinus concomitantly with arterial blood sampling 5 and 30 min after release of the aortic cross-clamp (n = 10). From a preoperative value of 92 +/- 13 ng/ml, C3a des-Arg rose during CPB to a maximum of 1816 +/- 393 at the end of CPB. Following reperfusion for 5 min, C3a des-Arg was 1284 +/- 232 ng/ml in arterial and 1106 +/- 100 in coronary sinus blood, a significant difference (p less than 0.05). The amount of C3a des-Arg trapped in the heart at 5-min reperfusion showed positive correlation with its arterial concentration (p less than 0.05). No significant difference was found after 30 min of reperfusion. Complement activation products trapped in the heart in the early reperfusion period may play a pathogenetic role in myocardial ischemia-reperfusion injury.

Preventing colorectal cancer. Knowledgeable patients should not die of colorectal cancer. Increasing the intake of dietary fiber, decreasing fat consumption, and increasing the use of modern technology to detect adenomatous polyps and early cancer can greatly decrease the mortality associated with colorectal cancer.

Male breast cancer. Male breast cancer is uncommon but important. The diagnosis is easily made by breast biopsy, and patients are presenting earlier in the course of the disease than in the past. Despite this, patients are often first seen with tumors that have metastasized to the axillary nodes, which markedly decreases the survival rate. Therapy of localized disease includes simple excision, modified radical mastectomy, and radical mastectomy, but there is no consensus for which operation is appropriate. Radiation therapy should be strongly considered in patients with metastases to the axillary nodes, but the role of adjuvant hormonal therapy or chemotherapy is unclear. For treatment of disseminated disease, tamoxifen seems to be replacing orchiectomy. The favorable response rate, especially in patients with estrogen-receptor-positive tumors, the lack of side effects, and the high level of patient acceptability make it an attractive therapeutic choice.

Alterations of muscarinic acetylcholine receptors in the nasal mucosa of allergic patients in comparison with nonallergic individuals. Cholinergic nasal hyperresponsiveness in nasal allergy may be due to changes of the characteristics in muscarinic cholinergic receptors. Radioligand receptor binding and in vitro autoradiographic studies of nasal mucosa in nonallergic (NA) and allergic patients were performed to investigate this hypothesis. The heterogeneous NA group was subdivided into control individuals and patients with chronic sinusitis and vasomotor rhinitis. The 3H-(-)-Quinuclidinylbenzilate binding to muscarinic receptors in human nasal mucosa membranes was saturable and of high affinity in all groups. No significant differences could be demonstrated between the subgroups of the NA patients. In allergic patients the dissociation constants and receptor densities were significantly decreased in comparison with those of NA and with those of control individuals. No differences in agonist binding or coupling of the muscarinic receptor to the effector system via the G protein could be observed in allergic patients. In vitro autoradiographic experiments demonstrated specific 3H-(-)-Quinuclidinylbenzilate labeling of the glandular acini in NA and allergic patients. No specific labeling could be observed in the epithelium, blood vessels, or connective tissue. In conclusion, the increased sensitivity and decreased muscarinic receptor number may reflect the cholinergic-induced hypersecretion in nasal allergy but are probably too small to explain the complex allergic reaction.

Ischemic colitis. An ever-changing spectrum? Ischemic colitis, or more properly colonic ischemia, became a clear clinical entity in the past 25 years. Yet, early diagnosis of this disease with its various presentations remains a difficult task. A 10-year review at our hospital identified 38 patients with colonic ischemia for comparison with the authors' previous experience and with data from the literature. Several important factors emerge: (1) Twice as many cases occurred after operations (34% in this series vs. 16% in the past), probably because fewer and fewer spontaneous cases were hospitalized. (2) Sixteen patients required operative intervention for colonic ischemia with a mortality of 62 per cent, while those treated nonoperatively had a mortality of 14 per cent. Seven of eight postoperative patients who required a second operative procedure for their colonic ischemia died. A high clinical suspicion is necessary in the postoperative patient, as colonic ischemia appears to be more severe among these patients. Moreover, the high incidence of associated cardiovascular disease indicates that early diagnosis, as well as monitoring of the "at-risk" patient, is needed for improvement in survival to occur. New monitoring methods, such as tonometry, may help accomplish this goal.

Observations on the pathophysiology of Nelson's syndrome: a report of three cases. Nelson's syndrome is generally regarded as an unusual sequela of primary bilateral adrenalectomy when performed for Cushing's disease. It is classically defined by cutaneous hyperpigmentation, considerably elevated adrenocorticotropic hormone (ACTH) levels, and an enlarged sella turcica. In this report, we present three cases initially treated by transsphenoidal sellar exploration for Cushing's disease. In two of these cases, remission of hypercortisolism did not occur after the initial pituitary exploration. A microadenomectomy was performed in one case and, in the other, no microadenoma was found. In both, Nelson's syndrome occurred after adrenalectomy. A second transsphenoidal operation and radiotherapy were required to control tumor growth. In another case, transsphenoidal adenomectomy of an ACTH-secreting tumor initially led to a remission of hypercortisolism for 4 years, but recurrent Cushing's disease necessitated adrenalectomy, and again Nelson's syndrome occurred. The documentation of a pre-existing ACTH-secreting basophilic pituitary microadenoma before adrenalectomy, as seen in two of our cases, has not been previously reported, and these observations of "non-classical" courses have major implications for the pathophysiology of Nelson's syndrome.

Coronary angioplasty of diffuse coronary artery disease. From January 1983 through December 1987, 98 patients underwent angioplasty of at least one diffusely diseased coronary artery. Diffuse coronary disease was described as: group I, narrowing greater than or equal to 50% that involved the entire vessel (40 patients), group II, long lesions greater than or equal to 2 cm in length (39 patients), group III, three or more lesions in the same vessel (19 patients). There were 65 men and 33 women, with a mean age of 60 years; 64 patients (65%) had unstable angina, 23 patients (23%) were diabetic, 31 (32%) had prior myocardial infarctions, and 12 had prior bypass surgery. Multivessel disease was present in 89% of patients. Angioplasty of only the diffusely diseased vessel was performed in 41 patients, and additional vessels were dilated in 57 patients. Overall, of 396 lesions (four per patient) and 197 vessels (two per patient) attempted, success was achieved in 382 lesions (96%) and 187 vessels (95%); angiographic success was achieved in 112 of 120 diffusely diseased vessels (93%). Clinical success was achieved in 91 patients (93%). The overall complication rate (death, myocardial infarction, urgent bypass surgery) was 8% (8 of 98): six patients (6%) had myocardial infarction (one Q wave, five non-Q wave), one patient (1%) had urgent bypass surgery, and two patients (2%) died (one during bypass surgery). The majority of complications (7 of 8 or 87%), including the two deaths, occurred in group I patients, with a 17.5% rate, versus 2.5% in group II and 0% in group III, p less than 0.002.

Angiotropic intravascular large-cell lymphoma (malignant angioendotheliomatosis): report of a case and review of the literature. We present a case of angiotropic large-cell lymphoma and review the literature in order to define the neurologic features of this rare disorder. This is the first report of gadolinium-DTPA imaging in angiotropic large-cell lymphoma that demonstrates infarcts of multiple ages, as well as striking meningeal enhancement. Angiotropic large-cell lymphoma should be suspected in patients with clinical evidence of small and large cerebral vessel disease and diagnosis requires skin, liver, renal, meningeal, or brain biopsy. Single modality treatment, using either radiation therapy or steroids, has been ineffective, and new findings of a lymphomatous origin of this neoplasm suggest that combination chemotherapy may be indicated.

Phenobarbital and cerebral blood flow during hypertension in the newborn beagle. Phenobarbital sodium has been used in anticonvulsant concentrations (15 to 40 micrograms/mL serum) in premature newborns in attempts to prevent periventricular and intraventricular hemorrhages. Although its clinical usefulness in this regard is controversial, phenobarbital treatment has been shown to reduce periventricular and intraventricular hemorrhages after hypertensive insult in newborn beagles. In this study cerebral blood flow values in steady state and during phenylephrine-induced hypertension with and without phenobarbital pretreatment were measured in newborn beagles. At anticonvulsant dosage, phenobarbital sodium decreased mean arterial blood pressure transiently during steady state and significantly reduced total cerebral blood flow during phenylephrine-induced hypertension without reducing mean arterial blood pressure. This phenobarbital sodium effect on cerebral blood flow was not as great in the presence of acidosis, and the initial hypotensive effect of phenobarbital sodium was sustained for a longer period of time during acidosis. Phenobarbital sodium may reduce the incidence of hemorrhages in the newborn brain by providing protection against isolated hemodynamic stresses characterized by acute increases in cerebral blood flow, with or without increased mean arterial blood pressure.

Endosonography of peri-anal and peri-colorectal fistula and/or abscess in Crohn's disease. Transcolorectal endosonography (ES) was performed in 36 patients with Crohn's disease suspected clinically to have a fistula or abscess. A hypoechoic or anechoic duct-like lesion immediately adjacent to the anorectal lumen compatible with a fistula was found in 32 patients. A communication between the fistulous tract and adjacent structures such as the skin, anal canal, or vagina was detected in all 32 patients. An anechoic cavity adjacent to or communicating with the fistula was visualized in 29 of the 36 patients. A fistula was visualized in the remaining seven patients with no evidence of an abscess. This anechoic cavity compatible with an abscess was surgically confirmed in 14 of 17 patients. We judged the extent and configuration of the abnormalities to be more clearly visualized by ES when results were compared with fistulography in five patients. There were no ES complications, and we conclude that ES is the preferred diagnostic procedure in patients with peri-rectal pathology because of the low risk of bacterial dissemination and low incidence of patient discomfort. Utilizing ES after non-surgical treatment was successful in 19 patients for documentation of the response to therapy.

Acceleration of luteinizing hormone pulse frequency in functional hypothalamic amenorrhea by dopaminergic blockade. A constellation of neuroendocrine secretory aberrations, including reduced LH pulse frequency and PRL concentrations, has been documented in women with functional hypothalamic amenorrhea (FHA). As pituitary function was preserved, these aberrations were attributed to an alteration in hypothalamic neuromodulation. To investigate the participation of the dopaminergic system in the genesis of the reduced LH pulse frequency and suppressed PRL levels in FHA, we studied six women with FHA and six cyclic women in the early follicular phase by obtaining blood samples at 15-min intervals for 48 h during sequential 24-h infusions of saline and a dopamine receptor blocker, metoclopramide (MCP). A hypothalamic vs. pituitary site of action was inferred from the pulsatility characteristics. MCP consistently elicited an increase in the LH pulse frequency in the women with FHA [7.3 +/- 1.2 (+/- SE) to 10.5 +/- 1.3 pulses/24 h; P less than 0.005]. In contrast, the eumenorrheic women did not show a significant change in LH pulse frequency in response to MCP (15.2 +/- 1.0 to 14.3 +/- 0.9 pulses/24 h). While the PRL concentrations were significantly lower in the FHA group during the infusion of saline (P less than 0.001) and MCP (P less than 0.005), the relative increases in PRL during MCP were similar in both groups. The acceleration of LH pulse frequency by blockade of dopamine receptors implies that there is increased hypothalamic dopaminergic inhibition of GnRH pulse frequency in women with FHA.

Early and frequent detection of HBxAg and/or anti-HBx in hepatitis B virus infection. To clarify the significance of the X gene of hepatitis B virus, we have tested for anti-HBx in the serum and HBxAg in the liver at different stages of the natural history of hepatitis B virus infection. Sera were screened by enzyme-linked immunosorbent assay and positive results confirmed by immunoblot. Purified recombinant MS2 Pol-HBx fusion protein was used as target for both assays. Among serial sera of patients with nonfulminant acute hepatitis, 24 of 64 patients (37.5%) were positive for anti-HBx. In fulminant cases, 15 of 36 patients (42%) had anti-HBx. In chronic hepatitis patients with high rates of hepatitis B virus replication, we found a significantly (p less than 0.01) higher prevalence of anti-HBx, 14 of 25 patients (56%), than in those with low replication, 14 of 66 patients (21%), or among asymptomatic HBsAg carrier blood donors (20 of 126 = 16%) without detectable hepatitis B virus replication (p less than 0.0001). The highest prevalence of anti-HBx was found in HBsAg carriers with cirrhosis (41 of 54 patients = 76%) and/or with hepatocellular carcinoma (18 of 33 patients = 54%). The findings suggest that anti-HBx appears as a common and early marker of hepatitis B virus infection, transient in self-limited hepatitis but persisting with progression to chronicity. In chronic hepatitis, the prevalence of anti-HBx correlated with the intensity and duration of hepatitis B virus replication but neither with the severity of the liver disease nor with malignant transformation per se.

Immunogenetic heterogeneity in rheumatoid disease as illustrated by different MHC associations (DQ, Dw and C4) in articular and extra-articular subsets. Genetic variants at DRB1 (Dw subtypes), DQB, and C4 loci were compared in rheumatoid disease subjects with or without the extra-articular feature of Felty's syndrome or major vasculitis. DR4 positive subjects with rheumatoid arthritis alone showed no preferential associations with DQB or Dw variants or with C4 null alleles. Felty's subjects showed associations with the DQB encoded DQw7 allele and with the C4B null allele but no preferential associations with any Dw subtype of DR4. By contrast DR4 +ve rheumatoid-vasculitic subjects showed associations with the Dw14 as well as with DQw7 and the C4A null allele. These different MHC associations in different clinical disease subsets show that rheumatoid disease is immunogenetically heterogeneous and suggest that MHC genes outside the DRB1 locus may also influence susceptibility or modify expression of the rheumatoid disease process.

Skin and epidural recording of spinal somatosensory evoked potentials following median nerve stimulation: correlation between the absence of spinal N13 and impaired pain sense. A clinical lesion study and intraoperative epidural recordings were made to test the origin and clinical significance of the spinal N13 and P13 of somatosensory evoked potentials (SEP) that follow median nerve stimulation. Intraoperatively, the respective peak latencies of spinal P13 and N13 coincided with those of the N1 component of the dorsal cord potential and its phase reversed positivity. On both the ventral and dorsal sides of the cervical epidural space, maximal amplitude was at the C5 vertebral level to which nerve input from the C6 dermatome is the main contributor. The modality of sensory impairment in the hand dermatome was examined in selected patients with cervical lesions, who showed such normal conventional SEP components as Erb N9, far-field P9, P11, P14, N18 and cortical N20, with or without loss of spinal N13. Statistically, the loss of spinal N13 was associated with decrease of pain sensation in the C6 dermatome. This was interpreted as being due to damage to the central grey matter of the cord, including the dorsal horn. Our results suggest the spinal N13 and P13 originate from the same source in the C6 spinal cord segment and that they are good indicators for the detection of centromedullary cervical cord damage.

Assay of serum cardiac myosin heavy chain fragments in patients with acute myocardial infarction: determination of infarct size and long-term follow-up. To evaluate the correlation between myosin heavy chain release and the necrosis mass, serum levels of myosin heavy chain fragments were determined serially in 55 patients with acute myocardial infarction. Eight of these patients were successfully treated with thrombolytic agents: the others were not treated. The same myosin titration was applied to the sera of 25 dogs with an experimental myocardial infarction. Six of the dogs were successfully treated with thrombolytic agents. The time courses of the myosin concentrations are typical and monophasic for all patients with a noncomplex myocardial infarction. The values for the kinetic parameters of myosin release are comparable to those previously reported. We have now determined that cumulative myosin release significantly correlates with cumulative creatine kinase (CK), CK-MB, and lactate dehydrogenase release, as well as with thallium-201 distribution, as determined for different patient groups. Thrombolytic treatment does not seem to qualitatively upset myosin kinetics. The results obtained in dogs with or without thrombolysis conclusively indicate that myosin release is a quantitative index of the necrosis mass. From a practical point of view, a few serial determinations of serum levels of myosin heavy chains are enough to estimate the necrosed mass in patients with acute myocardial infarction. More generally, serum myosin titration could be useful in detecting any cardiac disturbance involving myocardial injury resulting in membrane leakage of cardiac cells.

Systemic hemodynamic and cardiac function changes in patients undergoing orthotopic liver transplantation. The objective of this study was to determine the changes in systemic hemodynamics (systemic vascular resistance [SVR], cardiac output [CO], systemic blood pressure [SBP]) and cardiac function (pulmonary artery pressure [PAP] and pulmonary wedge pressure [PWP]) during the 96 hours following orthotopic liver transplantation (OLT) and correlate these with changes in hepatic and renal function and patient outcome. The study took place in a 12-bed medical respiratory intensive care unit in a large teaching hospital. Twenty-one patients had OLT performed over a 21.5-month period (January 1988 to October 15, 1989) for end stage liver disease (ESLD) from a variety of causes. A flow-directed right heart catheter and an indwelling arterial cannula were inserted for hemodynamic monitoring over a 96-hour postoperative period. Liver and renal function studies, total serum calcium, serum albumin, and fluid balance were determined daily. The SVR increased significantly to 12.8 +/- 0.6 U at 48 hours compared with immediate (less than 8 hours) postoperative levels (p less than 0.05) and remained elevated for 96 hours. The CO fell progressively and was significantly lower than baseline values from 64 to 96 hours. There was significant inverse correlation between the increase in SVR and the fall in CO (r = .85, p less than 0.01). The SBP was stable except for a small, but significant fall at 16 and 24 hours postoperatively. The PWP increased significantly from a baseline value of 12.5 +/- 0.9 mm Hg to 15 +/- 0.9 mm Hg at 32 hours and remained elevated through 96 hours (p less than 0.05). The serum bilirubin level fell progressively postoperatively and the prothrombin time and partial thromboplastin time (PTT) shortened significantly. Bile flow increased progressively from 107 +/- 120 ml/24 hours at the end of the first 24 hours to 188 +/- 125 ml/24 hours by 96 hours postoperatively. Five patients died from nine to 43 days postoperatively. These patients' hemodynamic parameters were not significantly different from the patients who survived. Successful OLT is associated with a rapid increase in SVR and a fall in CO without changes in SBP. These findings tend to parallel the improvement found in results of liver function tests. However, there is no correlation between the improvement in the hemodynamic state and long-term survival.

Urinary excretion of bile acid glucosides and glucuronides in extrahepatic cholestasis. Recently the formation of bile acid glucosides has been described as a novel conjugation mechanism in vitro and in vivo. In 10 patients with extrahepatic cholestasis caused by carcinoma of the head of the pancreas we investigated excretion rates and profiles of urinary bile acid glucosides. Urinary bile acid glucosides and, for comparison, bile acid glucuronides were extracted and characterized according to established methods. In controls total urinary bile acid glucoside excretion was 0.22 +/- 0.03 mumol/24 hr (mean +/- S.E.M.)-in the range of bile acid glucuronide excretion (0.41 +/- 0.06 mumol/24 hr; mean +/- S.E.M.). A gas chromatography-mass spectrometry-characterized trihydroxy bile acid glucoside of still-unknown hydroxyl positions accounted for 65% of total urinary bile acid glucosides. In extrahepatic cholestasis total urinary bile acid glucoside excretion was 0.52 +/- 0.13 mumol/24 hr (mean +/- SEM), yet significantly lower than bile acid glucuronide excretion (1.53 +/- 0.13 mumol/24 hr; mean +/- SEM; p less than 0.001). In cholestasis the primary bile acid derivatives cholic and chenodeoxycholic acid glucosides amounted to 90%, whereas the trihydroxy bile acid glucoside had decreased to 5% of total bile acid glucoside excretion, indicating its alteration during enterohepatic circulation. The data establish the composition and quantity of urinary bile acid glucosides in healthy controls and cholestasis and constitute a quantitative comparison with another glycosidic conjugation reaction, bile acid glucuronidation.

The De Vega tricuspid annuloplasty. Perioperative mortality and long term follow-up. One hundred and fifty-three patients undergoing De Vega tricuspid annuloplasty, with or without other associated cardiac procedures between January, 1979, and June, 1987, were evaluated. There were 136 hospital survivors. The follow-up was 98.1% complete for a mean of 3.7 years/patient. Operative mortality was 11.1%; preoperative NYHA class and length of CPB were significant risk factors of perioperative mortality. The actuarial survival of operative survivors at 9 years was 73.5 +/- 11.8%. There were 7 late cardiac deaths among a total of 12 late deaths. Eleven patients required reoperation (2.1 +/- 0.6% patient-year). In seven patients it was necessary for recurrence of tricuspid regurgitation; six of these had also a mitral prosthesis malfunction or a periprosthetic leak. Residual tricuspid regurgitation was judged as mild, moderate or severe in 29.9%, 11.9% and 4.3% of the patients respectively. De Vega tricuspid annuloplasty is the method of choice for mild and moderate tricuspid insufficiency; in selected cases, with a more severe degree of regurgitation, better results could be achieved with a different surgical approach.

Arterial oxygen desaturation during gastrointestinal endoscopy This prospective study evaluated the incidence and severity of arterial oxygen desaturation during gastrointestinal endoscopy. Following pulmonary function testing, 115 male patients underwent esophagogastroduodenoscopy (EGD), colonoscopy, or colonoscopy followed by EGD, with continuous recording of arterial oxygen saturation (SaO2). Most patients (80/115, 70%) showed arterial oxygen desaturation (greater than 4% decrease from baseline SaO2); severe arterial oxygen desaturation (SaO2 less than or equal to 85%) reflecting hypoxemia (PaO2 less than or equal to 50 mm Hg) was noted in one-third of patients overall (37/115, 32%). Severe arterial oxygen desaturation occurred in 9/62 EGD patients (15%), 23/46 colonoscopy patients (50%), and 4/7 patients having colonoscopy followed by EGD (57%). Arterial oxygen desaturation occurs frequently during gastrointestinal endoscopy and is often severe. These data support the concept that continuous monitoring of SaO2 should be standard procedure during all gastrointestinal endoscopic procedures.

Extracapsular cataract surgery with lens implantation in diabetics with and without proliferative retinopathy In a retrospective study we have examined all diabetics (66 operated eyes) and an equal number of non-diabetic matched controls who underwent extracapsular cataract extracation (ECCE) with intraocular lens (IOL) implantation over a two-year period ending in December 1987. Of the diabetic patients' 76% eyes improved by at least two lines of Snellen acuity postoperatively. Of these patients 68% eyes and of the control eyes 83% achieved an acuity of 6/12 or better. In the diabetics the visual outcome depended on the state of the retinopathy and in particular the maculopathy. The diabetic group had a greater incidence of postoperative inflammation, but the major complications were related to continuing neovascularisation. Early postoperative laser photocoagulation may help to prevent these proliferative complications, and, provided a large, adequate capsulotomy is performed for capsular thickening, the presence of an IOL does not interfere with this photocoagulation. We also advise early postoperative assessment, and treatment if necessary, of any maculopathy. Diabetic retinopathy should no longer be regarded as a contraindication to intraocular lens insertion.

Cholesterol screening in 5,719 self-referred elderly subjects. To assess the frequency of hypercholesterolemia as a potential major public health problem in the elderly, we studied 5,719 self-referred subjects greater than age 60 and 11,890 less than or equal to 60 years, whose nonfasting capillary blood cholesterol levels were measured during an 18-month screening in Cincinnati area grocery stores. We followed National Cholesterol Education Program guidelines for serum cholesterol (less than 200 mg/dl "desirable," 200-239 "borderline-high," and greater than or equal to 240 mg/dl "high"). Of 4,011 61-70 year-olds, only 19% had capillary blood total cholesterol less than 200, 38% were 200-239, and 43% greater than or equal to 240 mg/dl. Of 1,493 aged 71-80 years, only 20% had total cholesterol less than 200, 36% had levels 200-239, and 44% were greater than or equal to 240 mg/dl. In 215 subjects, 81 and over, 29% had total cholesterol less than 200, 36% were 200-239, and 35% were greater than or equal to 240 mg/dl. Application of the non-age, non-race, non-sex specific National Cholesterol Education Program guidelines to the elderly may necessitate followup and perhaps therapy in 71-81% of subjects, suggesting that the appropriate intervention approach be general population-oriented, rather than the individual detection, diagnosis, and treatment approach which presents a huge, expensive load for a relatively unprepared health care community.

Molecular pathologic study of human papillomavirus infection in inverted papilloma and squamous cell carcinoma of the nasal cavities and paranasal sinuses. Nasal inverted papilloma is a rare benign tumor occasionally associated with squamous cell carcinoma. To determine the etiological role of human papillomavirus in inverted papilloma, and to clarify the relationship between human papillomavirus and malignant transformation of this benign tumor, we retrospectively analyzed inverted papillomas from 26 patients, 7 of whom had squamous cell carcinoma. We used an immunohistochemical method and molecular pathologic techniques, or dot-blot hybridization of DNA extracted from paraffin-embedded tissues, in situ hybridization, and polymerase chain reaction. Human papillomavirus was detected in 5 of 26 patients (19%), 3 patients with human papillomavirus 11 and 2 patients with human papillomavirus 16. The latter 2 patients had inverted papillomas associated with squamous cell carcinoma. We speculate that human papillomavirus may be related to the malignant transformation of inverted papillomas.

Surgical treatment of brain metastases in malignant melanoma. The authors report the results of a retrospective review of 13 patients who underwent 19 craniotomies for resection of metastatic malignant melanoma at the University of Colorado (Denver, CO) between 1983 and 1989. There was preoperative evidence of extracranial disease in 11 patients. Eight patients had more than one intracranial metastasis at operation. Intraoperative ultrasound was used in 18 of the 19 craniotomies to minimize surgical trauma to the brain. The 30-day mortality was zero. The 30-day morbidity was minimal. No patient acquired a new neurologic deficit as a result of surgery. All patients regained at least their preoperative level of functioning. Six of the patients who were living at the time of review have been followed for 4 to 25 months (median, 7.5 months). The seven patients who were dead at the time of review survived 4 to 18 months (median, 10 months). These results compare favorably with the survival of untreated patients with metastatic melanoma to the brain (median, 1 month), patients treated with radiation therapy alone (median, 2-4 months), and those treated with chemotherapy alone (median, 2-4 months). The excision of metastatic melanoma from the brain, although not curative, may increase survival in patients with this problem with little morbidity and mortality even in the presence of other metastases.

Family recovery after vascular surgery. Twenty-one families were observed in a 3-month study to assess family coping with major vascular surgery and recovery. Analysis of family measures data (the Family APGAR, the Family Inventory of Resource for Management, and the Family Crisis Oriented Personal Evaluation Scales) was combined with grounded theory method to assess family responses over time and recovery outcomes. Containment emerged as the major conceptual category of the grounded theory. Containment refers to a constellation of constructed meanings for events and behavioral responses used by families to regulate the impact of the surgical crisis and reduce family disruption. This "contained" coping pattern was manifested in families' avoidant behaviors and narrow definitions of the problem: that is, they defined their situation in terms of the surgical repair as cure rather than palliative intervention for a chronic, progressive disease. Situational factors such as the insidious development of the illness and the primary focus of care providers in the hospital on surgical care (allowing families' narrow definitions of their situation to remain unchallenged) also contributed to containment. Containment resulted in poor risk factor management as a major recovery outcome. Isolation and family conflict were evident throughout the recovery period. Concerns generated by continued evidence of morbidity during recovery contributed to a developing awareness of underlying disease, and diminishing containment when this growing awareness was openly shared within the family. Significant findings of the family measures analysis were compared with the grounded theory of the qualitative data. Each corroborated the other in key dimensions.

Neuropsychological aspects of learning disabilities in epilepsy. Cognitive impairment is regarded as the link between epileptic conditions and the inability to learn in school. The neuropsychological approach to learning disabilities in epilepsy, therefore, first concentrates on analyzing the differential effects of epileptic factors on cognitive function. The impact of seizure activity, localization of epileptogenic foci, and antiepileptic treatment on cognitive functioning can be evaluated based upon the results of continuous assessment with a computerized neuropsychological test system. Second, learning disabilities may be evaluated on observations made during classroom performance. Three issues seem to predominate in learning studies among disabled children with epilepsy: test-retest variability, deterioration, and the supposed specificity of the learning disabilities.

Reoperative versus conservative management for gastrointestinal fistulas. Development of a fistula is a serious complication. It is necessary to identify its anatomic and pathologic features, as these can influence the outcome of treatment independent of the primary disease. Electrolyte abnormalities should be addressed as volume deficits are being restored. Sepsis, the most common cause of death in patients with fistulas, must be controlled, and the skin must be protected. Reoperation and conservative management are not opposing forms of therapy but rather are complementary.

Delayed rupture of a pseudoaneurysm of the costocervical trunk: treatment with therapeutic embolization. Fourteen days after removal of an internal jugular catheter inserted prior to renal transplantation, a patient presented with brisk arterial hemorrhage from the insertion site. The hemorrhage, caused by a ruptured pseudoaneurysm of the costocervical trunk, was controlled by transcatheter embolization with gelatin particles. One year later the patient reported no problems related to the pseudoaneurysm or its management.

Effect of microwave and ionizing radiation in patients with recurrent laryngeal carcinoma. This study describes the treatment and survival of 54 patients with gross recurrent laryngeal carcinoma after radiotherapy. Twenty-six patients were treated with local ultra-high frequency hyperthermia in combination with radiotherapy and chemotherapy (HRCH). Twenty-eight control patients were treated with radiotherapy and chemotherapy (RCh). Eight patients (33 per cent) in HRCh showed a three year survival but no patient in the RCh group. At two years 18 patients (75 per cent) in HRCh and two (7 per cent) in RCh survived.

Magnetic resonance imaging of congenital heart disease in the pediatric patient. Current state-of-the-art MR imaging offers a high resolution evaluation of cardiac anatomy in the child with congenital heart disease. Although echocardiography remains the standard initial evaluation modality, MR imaging plays a key role in supplementing this information in the pediatric thorax. Although applications for this modality continue to be defined, some indications have become clear. The future of MR imaging promises better spatial resolution, faster image acquisition times, and in vivo spectroscopic capabilities.

Soluble histocompatibility antigen class I in breast cancer patients in relation to tumor burden. Serum beta-2 microglobulin (B-2M) levels were studied in 365 breast cancer patients and 210 age-matched controls. The patients were divided into three groups: Group A, new patients at diagnosis; Group B, patients at follow-up; and Group C, metastatic patients. The mean B-2M of all breast cancer patients plus or minus one standard deviation (3.5 +/- 1.2; range, 1.1 to 5.9) was significantly higher than normal controls (1.29 +/- 0.49; range, 0.3 to 2.3; P less than 0.005). When the three patient groups were compared with each other, the mean B-2M level of Group A (3.0 +/- 1.5; range, 0.9 to 6.9) was similar to that of Group C (4.22 +/- 1.1; range, 2.0 to 6.4). The mean B-2M of both Groups A and C was significantly higher than that of Group B (2.38 +/- 1.02, range, 0.4 to 5.4; P less than 0.001). In Group A the mean B-2M decreased significantly after a 12-month period and reached the mean level of Group B but not that of normal controls. When patients in Group B were analyzed by their stage of disease at diagnosis, there was no significant difference between Stages I and II. There was a significant difference in the mean B-2M levels between Stages I and III. In relapsing patients, mean B-2M levels increased. These findings suggest that serum B-2M levels may reflect tumor burden, and even in patients at follow-up, occult tumor cells may activate the immune system.

Effect of indomethacin on pain relief after thoracotomy. The effect of indomethacin on postoperative pain was studied in 60 adult patients undergoing thoracotomy in a prospective, randomized, double-blind manner. Patients receiving indomethacin required significantly less opioid after operation and had significantly lower pain scores compared with the control group. Pain on movement and on coughing were reduced also. No major adverse effects were encountered.

Intravital detection of skin capillary aneurysms by videomicroscopy with indocyanine green in patients with progressive systemic sclerosis and related disorders. Conventional capillaroscopy and infrared fluorescence videomicroscopy with indocyanine green were performed at the nailfold in 12 healthy controls and 38 patients with microangiopathy due to systemic sclerosis or related disorders. Saccular aneurysms featuring head and neck (type 1) and aneurysmatic enlargements (type 2) were defined. Microaneurysms were located at the apex or near the apex of capillary loops and were significantly more common in patients than in controls (p less than 0.02 for type 1 and p less than 0.001 for type 2). Combination of the two lesions was found only in patients and appears to be a valuable new diagnostic sign for the presence of microangiopathy. In comparison with conventional capillaroscopy, about twice as many microaneurysms were detected by videomicroscopy with indocyanine green coupling almost completely to plasma proteins. The new technique allows visualization of capillary aneurysms even when filled only by plasma.

Isotretinoin for refractory lupus erythematosus. We describe six patients with cutaneous manifestations of lupus erythematosus who were treated with isotretinoin, 1 mg/kg/day. In each case the cutaneous lesions had proved resistant to systemic corticosteroids and antimalarial therapy. Treatment with isotretinoin resulted in rapid clinical improvement in all cases. Recurrences were similarly rapid when the drug was discontinued. Side effects were minimal and easily controlled by adjustments in dose or by the use of lubricants.

Expert testimony based on decision analysis: a malpractice case report. OBJECTIVE: Expert testimony in malpractice cases is often subjective and biased. Decision-analytic techniques might provide an objective basis for such testimony. DESIGN: Case report. This article reports the case of a patient with chest pain that resulted in a malpractice suit alleging a delay in diagnosis of coronary artery disease. SETTING: The case occurred in a private practice; the expert witnesses and the decision analysis originated from a university teaching hospital. METHODS: A decision tree and threshold analysis were used to define the thresholds of disease probability at which either testing or treatment should be implemented. The expert testimony of two witnesses that exercise stress testing was the standard of care was compared with the results of the decision analysis. MAIN RESULTS: Decision analysis supported the view that cardiac catheterization would have been the more appropriate test. CONCLUSIONS: Techniques of decision analysis provide a structured and quantitative basis for empirical judgment and may help to minimize current problems with expert testimony.

The effect of alcohol in isolated blunt splenic trauma. The effect of alcohol on trauma patients is controversial, with numerous authors citing no difference in mortality in acutely intoxicated patients. The purpose of our study was to retrospectively investigate the effect of alcohol in adult patients with isolated blunt splenic injury. From 1980 through 1989, 47 adult patients with splenic trauma as the only major injury were admitted to the Trauma Service. There were 37 males and ten females with a mean age of 29 years (range, 15 to 61). Motor vehicle accidents were responsible for 44 (94%) of the injuries. Group 1 consisted of 24 patients with a mean blood alcohol level of 185 mg/dl (range, 15 to 380). In Group 2 there were 23 patients without detectable blood alcohol. There were no statistically significant differences between the two groups in age, Abbreviated Injury Severity Score, initial hematocrit, and grade of splenic injury. Hypotension was present in 13 patients (55%) in Group 1 versus six patients (26%) in Group 2 (p less than 0.05). Significant abnormalities in clotting studies were present on admission in six patients (25%) in the alcohol-detected group versus one in the other group (p less than 0.05). Blood transfusion requirements in the first 24 hours were significantly greater in Group 1 (mean, 3.9 units) versus Group 2 (mean, 0.5 units) (p less than 0.001). If alcohol was present, there was much less chance for splenic conservation, as 18 patients (75%) underwent splenectomy versus seven patients (30%) in the nonalcohol group (p less than 0.05). There was one death and this occurred in a patient acutely intoxicated who suffered a cerebral infarct.

Arterial switch procedure in an adult. The arterial switch procedure has been shown to offer both anatomic and physiologic correction in infants and children with transposition of the great arteries and ventricular septal defect. We believe that adults may also benefit in some circumstances, as evidenced by the case of a 22-year-old man who underwent successful operation after having initially undergone pulmonary artery banding for transposition with ventricular septal defect in 1967 when he was 5 months of age.

Clinical performance of St. Jude and Medtronic-Hall prostheses: a randomized comparative study. Newer and improved models of mechanical prostheses are regularly added to surgeons' armamentarium. This study was designed to compare the clinical performance of two of the most used current models of mechanical prostheses. From August 1983 through July 1985, 182 white patients were prospectively randomized to implantation of the St. Jude Medical (95 patients) or Medtronic-Hall (87 patients) prostheses. There were 84 mitral, 85 aortic, and 13 double (mitral and aortic) valve replacements. There were no differences between the two groups with regard to sex distribution, age, functional class, emergency operation, and site of implantation. Early mortality was 3.2% for patients with the St. Jude valve and 5.7% for those with the Medtronic-Hall (p = NS). The survivors were followed for 3 to 5 years (mean, 4.2 +/- 0.6 years; cumulative follow-up, 559 patient-years). Late mortality was 7.1%/patient-year for the St. Jude group and 3.2%/patient-year for the Medtronic-Hall group (p less than 0.05). However, the valve-related mortality was equal (1.4%/patient-year) for both groups. Noncardiac causes accounted for most of the difference between the St. Jude and Medtronic-Hall groups (2.5%/patient-year and 0.4%/patient-year, respectively). There were no cases of thrombotic obstruction, whereas serious systemic thromboembolism occurred at the rate of 1.8%/patient-year (5 episodes) for the St. Jude group and 2.5%/patient-year (7 episodes) for the Medtronic-Hall group (p = not significant); there were another nine episodes of systemic embolism that left no sequelae. Three patients (St. Jude, 2; Medtronic-Hall, 1), all of whom had aortic valve replacement, required reoperation (0.5%/patient-year) because of prosthetic endocarditis, with two deaths.

Function in athymic nude mice of parathyroid heterografts from patients with primary hyperparathyroidism and secondary hyperparathyroidism. Heterotransplantation of adenomatous parathyroid glandular tissue from humans with primary hyperparathyroidism into athymic nude mice creates a unique animal model of this disease. The mice manifest high concentrations of both midregion/C-terminal human parathyroid hormone and biologically active intact human parathyroid hormone relative to either mice with no implants or mice that received normal human parathyroid tissue. Secretion of these substances is maintained in most mice for at least 9 to 13 months after implantation. In addition, animals that have experienced implantation exhibit other characteristics associated with human primary hyperparathyroidism including relative hypercalcemia and increased renal 25-hydroxyvitamin D-1 alpha-hydroxylase activity. We also measured these parameters in a group of nude mice that received transplantation of a similar mass of hyperplastic parathyroid tissue that was obtained from patients with uremic secondary hyperparathyroidism. Although we hypothesized that the level of human parathyroid hormone secretion from these implants would fall over time in response to the normal host environment, hormone levels remained as high as those in recipients of adenomatous heterografts, even after 9 to 13 months. Moreover, similar biologic effect of the excess parathyroid hormone (i.e., relative hypercalcemia, hyperphosphatasemia, and increased 1,25-dihydroxyvitamin D biosynthesis) were detected. These animal models should prove extremely useful in supplementing our understanding of hyperparathyroid disorder in man.

Occult cancer in patients with deep venous thrombosis. A systematic approach. The authors prospectively studied 113 consecutive patients with deep venous thrombosis of the lower extremities to determine the most appropriate workup study for searching for a hidden cancer. After a careful physical examination, the following routine tests were performed: erythrocyte sedimentation rate (ESR), whole blood counts, biochemistry, carcinoembryonic antigen (CEA) levels, chest radiograph, upper gastrointestinal endoscopy, abdominal ultrasound and computed tomography (CT) scan. If a malignant lesion was suspected, further appropriate studies were performed. After discharge, periodic follow-up was performed on all patients in the outpatient clinic. A malignant neoplasm was detected in 12 patients. Of these 12 patients, six were asymptomatic with the exception of experiencing thrombophlebitis. Cancer was found more commonly in patients with idiopathic deep vein thrombosis (DVT) (7 of 31 versus 5 of 82 patients with secondary DVT; P = 0.012), and in those patients with abnormal lactic dehydrogenase (LDH) levels (6 of 23 versus 6 of 90; P = 0.007). Abnormal CEA levels allowed diagnosis of two cases of colonic cancer (on colonoscopy). Both ultrasound and CT scan of the abdomen showed two cases of urinary bladder carcinoma at a very early stage. Furthermore, two cases of adenomatous polyps in colon were found, a condition considered by most authors to be a colorectal cancer precursor. In addition, there were five patients with large benign pelvic tumors, and two patients with absent inferior vena cava. The most striking finding was that some cases of cancer were at a very early stage. It was concluded that blood cell counts, LDH, CEA, chest radiograph, and abdominal ultrasonography (or CT scan) should be routinely performed on all patients with deep venous thrombosis (particularly those with idiopathic DVT). Malignancy would not have been recognized in some patients if these tests had not been performed.

The prolonged burner syndrome. Over the course of a single football season, six players evaluated by the medical staff had burners that displayed a prolonged neurologic recovery. These players were examined and subsequently evaluated with isokinetic testing and electrodiagnostic studies to elucidate better the short-term natural history of the prolonged burner syndrome. Evidence of muscular weakness at 72 hours postinjury best correlated with positive electrodiagnostic findings. No correlation was found between the initial physical examination findings and the results of electrodiagnostic testing. Isokinetic strength evaluation demonstrated many relative strength differences that were difficult to discern with manual muscle testing. The return of a player to athletic competition following this injury should largely be based on the clinical examination.

Capillary hemangiomas and treatment with the flash lamp-pumped pulsed dye laser Strawberry, or capillary, hemangiomas are common vascular neoplasms, with an incidence of approximately 2.6% in neonates. They usually develop in the first few weeks of life, so that between 1 month and 1 year the incidence rises to between 8.7% and 10.1%. These lesions may grow quite large in the first year of life, and they may ulcerate or obstruct a vital organ or function. The great majority will spontaneously regress after the first year of life. Parents are often alarmed at the sight of these hemangiomas and need reassurance that the great majority will regress spontaneously. Treatments such as cryosurgery, irradiation, radium instillation, corticosteroid therapy, or surgical excision are often ineffective or cause significant morbidity. We describe 10 children with capillary hemangiomas treated with the flash lamp-pumped pulsed dye laser. The patients ranged in age from 7 weeks to 5.5 years at the beginning of laser therapy. The patients underwent 3.1 +/- 1 (mean +/- SD) laser treatments, with a mean regression of the lesions of 69.9% +/- 4.5%. All patients demonstrated some diminution in the size and color of their hemangiomas after the treatments, and there were no ill effects, such as ulceration, hemorrhage, infection, or scarring. There was no evidence of hyperpigmentation or hypopigmentation. Pulsed dye laser therapy should be considered as an option in the treatment of capillary hemangiomas, preferably prior to their full evolution. It is also a useful therapeutic approach in those hemangiomas that are slow to regress in older children.

Effects of MK-801 on recovery from sensorimotor cortex lesions. Histologic evidence suggests that drugs acting as noncompetitive antagonists at the N-methyl-D-aspartate receptor can have beneficial or pathologic effects on central nervous system neurons. In the present experiments we examined the effects of MK-801 on recovery of behavioral function after unilateral lesions in the rat somatic sensorimotor cortex. In the first experiment, rats with unilateral sensorimotor cortex lesions were given either MK-801 (1 mg/kg) or saline 12-16 hours after surgery. Additional injections were given on postoperative days 2, 4, and 6. Behavioral tests measured somatosensory asymmetries (i.e., bilateral tactile stimulation tests) and forelimb placing. After creation of sensorimotor cortex lesions, rats showed an ipsilateral somatosensory bias and an impairment in placing the contralateral forelimb. Rats treated with MK-801 recovered slightly faster than saline-treated animals as measured by a bilateral tactile stimulation test (p less than 0.05). In contrast, there was no significant difference between the groups in the recovery of forelimb placing. In a second experiment, rats with sensorimotor cortex lesions were treated with a single injection of MK-801 after behavioral recovery. Twenty hours after the MK-801 injection, rats with sensorimotor cortex lesions showed a reinstatement of the placing deficits. The impairment endured for at least 7 days after injection. These behavioral data support the idea that MK-801 can have either beneficial or detrimental effects when administered after brain damage.

Mucoid vasculopathy of unknown etiology. A new vascular disorder with generalized deposition of abnormal amounts of acid mucopolysaccharide (AMPS) material in arteries, veins, and vasanervorum has been observed in a large number of autopsies at the author's institution. It is unlike any of the known vascular diseases and has emerged as a distinct disorder of vascular connective tissue. This has been named "mucoid vasculopathy of unknown etiology." This hitherto unreported entity is described here.

The importance of cytogenetic studies in adult acute lymphocytic leukemia. PURPOSE: The prognostic importance of pretreatment bone marrow cytogenetic studies in adults with acute lymphocytic leukemia treated at a single institution, with an identical treatment program, is described. PATIENTS AND METHODS: A total of 105 patients with a documented morphologic diagnosis of acute lymphocytic leukemia were reviewed for the purpose of this analysis. All patients had an extensive workup at presentation, and cytogenetic analysis was performed in 103 patients, using the Giemsa banding technique with trypsin pretreatment on 24-hour cultured cells. RESULTS: The specific cytogenetic classification in the 103 patients who had the karyotypic analysis was as follows: diploid 27%; Philadelphia chromosome-positive 13%; hyperdiploid 12%; B-cell karyotype 6%; 6q- and 14q+ abnormalities 4%; pseudodiploid 8%; hypodiploid 2%; and insufficient metaphases 28%. B-cell, 6q- or 14q+, and Philadelphia chromosome-positive karyotypes tended to correlate with other known negative prognostic factors. Patients with diploid, hyperdiploid, pseudodiploid, and hypodiploid karyotypes or with insufficient metaphases could be combined into one group with a favorable prognosis. In this group, the remission rate with induction chemotherapy was 89%, the median complete remission duration was 26 months, and the median survival was 25 months, with a 3-year survival rate of 45%. Patients with Philadelphia chromosome-positive, B-cell, and 6q- or 14q+ abnormalities collectively had an unfavorable prognosis. Their response rate to induction chemotherapy was 65%, the median response duration was 7 months, and the median survival was 8 months, with a 3-year survival rate of less than 10%. CONCLUSION: We conclude that the pretreatment bone marrow karyotype is an important part of the evaluation of adults with acute lymphocytic leukemia and provides significant prognostic information.

Smooth muscle cell proliferation and restenosis after stand alone coronary excimer laser angioplasty It has been shown that coronary excimer laser angioplasty can remove atherosclerotic intracoronary tissue. Stand alone coronary excimer laser angioplasty was successfully performed in a 53 year old white man with 90% stenosis of the left anterior descending coronary artery and exertional angina (Canadian Cardiovascular Society class III). The lesion was reduced to a 30% residual stenosis with use of a 1.2 mm and subsequently a 1.8 mm diameter laser catheter. Early follow-up angiography 24 h later revealed persistent patency and unchanged lesion diameter of the target vessel. The patient was free of symptoms during the 2 month follow-up period, but died suddenly while playing in a tennis tournament 63 days after the procedure. Postmortem histologic examination revealed 80% restenosis at the lesion site without plaque disruption or thrombosis. Specific staining of the histologic specimen for smooth muscle cells using alpha-actin revealed significant smooth muscle cell proliferation at the site of coronary excimer laser angioplasty. However, most of the vessel narrowing appeared to be due to underlying fibrotic plaque as a result of insufficient tissue ablation. This was probably related to the size of the currently available catheters, which are too small to create a large channel.

Late effects of treatment for Wilms' tumor. A report from the National Wilms' Tumor Study Group. The National Wilms' Tumor Study (NWTS) was initiated in 1969. One of its objectives was to modulate treatments according to risk factors to minimize the number and severity of treatment-related short-term and long-term iatrogenic complications. The NWTS has therefore incorporated a Long Term Follow-up Study (LTFS) within its framework to monitor late effects. The LTFS is confined to relapse-free survivors alive 5 years or longer after initial surgery, and data are collected using specifically designed forms. A total of 787 patients registered on NWTS-1 or NWTS-2 (1969 to 1979) were eligible, of whom 680 (86%) were available for analysis regarding musculoskeletal, cardiovascular, and neuropsychologic status, and the presence of benign and malignant tumors. Patients with early-stage disease who were treated with radiation had scoliosis reported, along with other musculoskeletal abnormalities (32 versus 2), nearly seven times as often as did the members of the cohort population who did not undergo radiation (35 of 57 versus 5 of 53, respectively). The difference in cardiovascular problems recorded in survivors who did and did not receive Adriamycin (Adria Laboratories, Columbus, OH) (2.4 versus 1.1 per 100-person years at risk) had borderline statistical significance (P = 0.06). No excess in neuropsychologic events was reported for those given the neurotoxin vincristine. When considering patients with disease of all stages, all 5 second malignant tumors occurred in the 623 patients who underwent radiation (RT patients); benign tumors were also more frequent in RT patients than in those patients who did not undergo radiation (41 of 486 or 8% versus 4 of 194 or 2%). Continuing study of this unique body of patients is needed, especially for those given Adriamycin, because of the known long interval needed for latent cardiomyopathy to become clinically manifest in some patients.

Diagnosis of oral hairy leukoplakia by ultrastructural examination of exfoliative cytologic specimens. Lingual exfoliative cytologic specimens (scrapings) were obtained from 18 patients positive for human immunodeficiency virus with clinical oral hairy leukoplakia. Buccal mucosal scrapings were obtained from 12 of these patients. The specimens were processed for examination by transmission electron microscopy (TEM). Sixteen (89%) of the lingual specimens revealed infection of keratinocytes by herpes-type virus. There was no evidence of virus infection in the 12 buccal mucosal scrapings. Fungal hyphae were seen by TEM in 14 (78%) of the lingual scrapings and two (17%) of the buccal scrapings. One exfoliative specimen and two biopsy specimens were stained for Epstein-Barr virus DNA with a DNA probe. The demonstration of herpes-type virions by TEM in keratinocytes from a lesion clinically suspected to be hairy leukoplakia provides direct, objective diagnosis. Furthermore, use of exfoliative cytologic specimens provides a clinically simple, noninvasive technique.