Delayed granuloma formation in a patient with vanishing bile duct syndrome 7 years post-liver transplantation.

A patient was found to have numerous granulomata 7 years after orthotopic liver transplantation for primary sclerosing cholangitis (PSC) on a recent liver biopsy specimen. This histopathologic finding prompted a review of the literature to determine the commonality of this feature in the absence of the usual causes of granulomatous liver disease, none of which were found to be the cause of this patient's liver histopathologic state. The presence of posttransplantation granulomata is rare, and although previously reported to occur shortly after liver transplantation, this finding has not been reported previously with either PSC or vanishing bile duct syndrome. We are not aware of another case of granulomata associated with recurrent PSC or vanishing bile duct syndrome 7 years after liver transplantation.

Dobutamine stress echocardiography for preoperative cardiac risk stratification in patients undergoing orthotopic liver transplantation.

This study attempts to evaluate the efficacy of dobutamine stress echocardiography for preoperative cardiac risk stratification in patients undergoing orthotopic liver transplantation. Two hundred twenty consecutively submitted patients were evaluated in preparation for orthotopic liver transplantation. Dobutamine stress echocardiography was performed in 80 patients with known or suspected coronary artery disease. Follow-up information was available in 40 patients in the form of cardiac catheterization and/or outcome from liver transplantation to validate the dobutamine stress echo findings. The prevalence of coronary artery disease in this cohort was 5% and was closely associated with the presence of diabetes mellitus. Dobutamine stress echocardiography, when interpreted as abnormal in the presence of wall motion abnormalities only, is associated with a sensitivity, specificity, and positive and negative predictive value of 100%. Dobutamine stress echocardiography is highly efficacious and should be the screening study of choice to detect coronary artery disease in patients undergoing orthotopic liver transplantation.

Distinctive clinical features of portopulmonary hypertension.

STUDY OBJECTIVE: To differentiate the cardiopulmonary profile of portopulmonary hypertension (PPHTN) from that of primary pulmonary hypertension and chronic liver disease. DESIGN: Retrospective survey. SETTING: Tertiary care center. PATIENTS: Thirty patients with cardiac catheterization-proven PPHTN were compared to 30 randomly selected patients with primary pulmonary hypertension alone and 30 patients with chronic liver disease alone necessitating consideration of liver transplantation (L-CONT). INTERVENTIONS: All patients underwent right heart catheterization, echocardiography, ECG, chest radiography, pulmonary function tests, ventilation-perfusion scanning, and room air arterial blood gas measurements. RESULTS: Patients with PPHTN exhibited elevated pulmonary pressures (mean pulmonary pressure, 48.6+/-2.1 mm Hg) and pulmonary vascular resistance (11.6+/-1.6 mm Hg/L/min/m2) with simultaneous elevation in the cardiac index (3.8+/-0.3 L/min/m2) and depression of systemic vascular resistance (24.9+/-1.7 mm Hg/L/min/m2). Arterial blood gas measurements indicate that PPHTN exhibits a significant accentuation of the chronic respiratory alkalosis (PCO2, 28.7+/-0.5 mm Hg) usually seen with chronic liver disease and pulmonary hypertension. In addition, patients with PPHTN have an increased alveolar-arterial gradient (27.0+/-2.7 mm Hg) when compared to patients with L-CONT, suggesting impaired gas exchange. CONCLUSIONS: PPHTN is associated with a unique clinical profile that possesses characteristics common to and exclusive of liver disease and primary pulmonary hypertension.

The evolution of a successful liver transplant program in 1996: the clinical and administrative role of the anesthesiologist.

The establishment of a new liver transplant program requires enormous planning and resources. Extensive negotiations must take place to ensure institutional and departmental commitments to obtain the proper equipment, personnel, and other resources. The formation of a well-trained multidisciplinary team of physicians and nurses becomes the next step. Finally, ample time must be provided to adequately deploy resources, lobby referring physicians, recruit patients, and troubleshoot problems as they arise.

Primary biliary cirrhosis.

Primary biliary cirrhosis is a chronic, usually progressive, cholestatic liver disease of presumed autoimmune etiology that affects predominantly young and middle-aged women. It is nearly always associated with an antibody directed against a component of the pyruvate dehydrogenase complex located on the inner wall of the mitochondria. The disease is associated with a number of other associated autoimmune disorders. No totally effective medical treatment has been established for the disease, although urosdeoxycholic acid appears promising. Complications of cholestasis such as fat malabsorption and fat-soluble vitamin deficiency should be excluded or corrected when found. Individual patient prognosis varies. Several models for estimating individual patient survival are available. Liver transplantation is recognized as a procedure to extend and improve the quality of life for patients with advanced disease.

The natural history of abdominal pain associated with primary biliary cirrhosis.

OBJECTIVES: The aim of the present study was to determine the prevalence and natural history of abdominal pain in patients with primary biliary cirrhosis. METHODS: We studied 178 patients with well-defined primary biliary cirrhosis enrolled in a prospective randomized trial of ursodeoxycholic acid. These patients underwent upper endoscopy and upper abdominal ultrasound prior to entry, at 2 yr, and as indicated. Fourteen patients had additional evaluations including abdominal CT (four), colon x-ray (five), colonoscopy (three), endoscopic retrograde cholangiopancreatography (two), and upper gastrointestinal x-ray (two). RESULTS: Patients with abdominal pain generally presented with right upper quadrant discomfort. Thirty-one patients (17%) had pain at study entry: 33% of these had pain persisting at 1 yr, and 20% of these had pain persisting at 2 yr. The resolution of pain was not clearly affected by ursodeoxycholic acid. Evaluation with ultrasound and upper endoscopy found four patients with asymptomatic cholelithiasis, one with esophageal erosions, four with gastric erosions, one with a gastric ulcer, and two with duodenal erosions. Additional tests were unrevealing in 14 patients. Patients with pain were similar to patients without pain with regard to age, histological stage, gender, and liver biochemistries. CONCLUSIONS: We conclude that chronic right upper quadrant pain is not uncommon in patients with primary biliary cirrhosis, that it usually resolves spontaneously, and that upper endoscopy is the most important diagnostic test to use to exclude treatable causes of pain.

Vertebral osteomyelitis caused by Nocardia asteroides: report and review of the literature.

A 65-year-old man with 9 months of radicular lower back pain was admitted for treatment of presumed malignancy of the lumbar spine. Lumbar radiographs showed destruction of the L4-L5 endplates and obliteration of the disc space, which was more compatible with an infectious process. After 2 different closed biopsy specimens failed to reveal an etiologic organism, N. asteroides was cultured from an open biopsy sample of the affected vertebrae. This and 8 other cases in the English literature of vertebral osteomyelitis caused by N. asteroides are reviewed.