A randomized clinical trial of ursodeoxycholic acid as adjuvant treatment to prevent liver transplant rejection.

Acute rejection following orthotopic liver transplantation is a common problem despite current immunosuppressive regimens. Ursodeoxycholic acid (UDCA) has been shown in small, open-labeled studies to prevent rejection episodes, although its effects on complications such as infections, length of hospital stay, and survival have not been evaluated. We conducted a randomized, placebo-controlled, double-blind trial to determine if UDCA (10-15 mg/kg/d) added to a cyclosporine-based immunosuppressive regimen was associated with a decrease in the incidence of at least one episode of acute cellular rejection.

The Child-Pugh classification as a prognostic indicator for survival in primary sclerosing cholangitis.

Clinical decisions in primary sclerosing cholangitis (PSC) depend upon understanding its variable natural history. Several prognostic models for survival have been developed. We explored the Child-Pugh Classification (CPC) to determine if it predicts survival as well as a disease-specific model (DSM).

First-trimester diagnosis of Smith-Lemli-Opitz syndrome.

We report here the prenatal diagnosis of Smith-Lemli-Opitz (SLO) syndrome in the first trimester by direct measurement of 7-dehydrocholesterol (7-DHC) in a chorionic villus (CV) biopsy. The proband was diagnosed clinically at birth and the diagnosis was confirmed biochemically by demonstrating elevated 7-DHC in plasma. The family pursued prenatal diagnosis in their fourth, fifth, and sixth pregnancies.

Performance characteristics and results of a large-scale screening program for viral hepatitis and risk factors associated with exposure to viral hepatitis B and C: results of the National Hepatitis Screening Survey. National Hepatitis Surveillance Group.

Chronic viral hepatitis frequently goes undetected until cirrhosis develops. Although the effect of interferon on the natural history of hepatitis B virus (HBV) or hepatitis C virus (HCV) infection in asymptomatic persons is unknown, treatment may modify the course of the infection, producing cures in some. In September 1992, screening for HBV and HCV was offered in 40 centers throughout the United States.

Management and outcome of patients with invasive carcinoma arising in colorectal polyps.

Background & Aims: Treatment for invasive adenocarcinoma in colorectal polyps (malignant polyps) is controversial. The aim of this study was to evaluate our institutional treatment strategy for malignant polyps.

Methods: Malignant polyps were designated as having favorable histology (grade I or II carcinoma with at least a 2-mm free margin) or unfavorable histology (grade III invasive adenocarcinoma, invasive adenocarcinoma with an unassessable margin, or a margin of < 2 mm).

Transmission of Toxoplasma gondii infection by liver transplantation.

Toxoplasmosis is an important disease in immunocompromised hosts, particularly in patients with AIDS and in heart transplant recipients. Infection with Toxoplasma is less commonly seen in recipients of other solid organ transplants. We report a case of fulminant disseminated infection with Toxoplasma after liver transplantation.

Prevalence and reversibility of the hepatopulmonary syndrome after liver transplantation. The Cleveland Clinic experience.

To ascertain the prevalence and reversibility of the hepatopulmonary syndrome, we reviewed the cases of 98 patients undergoing liver transplantation at the Cleveland (Ohio) Clinic Foundation from June 1988 through July 1992 and identified 4 patients with clinically recognized hepatopulmonary syndrome (prevalence 4%). All 4 patients ultimately had complete reversal of their disorder. As reviewed herein, the prevalence of the hepatopulmonary syndrome in the current series is lower than in previous reports, possibly reflecting a dependence on its clinical recognition in this series rather than the use of routine screening tests.

The prevalence of coronary artery disease in liver transplant candidates over age 50.

The prevalence of angiographically proven coronary artery disease (CAD) in adults with end-stage liver disease who undergo evaluation for liver transplantation is unknown; also it is unclear if cholestatic liver disease represents an independent risk factor. Patients with end-stage liver disease over age 50 having liver transplantation were studied using coronary angiography. Arterial stenosis was graded as normal, mild (< 30%), moderate (30 to 70%), or severe (> 70%).

Risk of extraesophageal malignancy in patients with adenocarcinoma arising in Barrett's esophagus.

Objectives: It has been suggested that the presence of Barrett's mucosa is a marker for potential malignancy in other organs. Our objective was to study subjects with adenocarcinoma of the esophagus arising in Barrett's epithelium.

Methods: We reviewed the medical records of patients with esophageal adenocarcinoma, with esophageal squamous cell carcinoma, and with no esophageal pathology and recorded the occurrence of extraesophageal malignancies and the heavy use of tobacco and alcohol.

Dysplastic nevi as a melanoma risk factor in patients with familial melanoma.

Background: Familial melanoma has been associated with "clinically atypical moles" or "dysplastic nevi," (DN) which are markers for increased melanoma risk. In addition, melanomas in these kindreds present at a younger age, and tend to be multiple.

Methods: Melanoma incidence rates were determined for 710 members of 311 melanoma families, defined as kindreds in which melanoma had occurred in two or more blood relatives.

Pitfalls in the prenatal diagnosis of peroxisomal beta-oxidation defects by chorionic villus sampling.

Variability in the level of expression of very long chain fatty acids (VLCFAs) is documented in cultured chorionic villus (CV) cells derived from two fetuses, one at risk for an unusual peroxisomal fatty acid beta-oxidation defect, and the other at risk for the X-linked form of adrenoleucodystrophy (ALD). Cells from early subcultures of chorionic cells from both cases gave normal values for VLCFA ratios. The results for the fetus at risk for the beta-oxidation defect were interpreted to indicate that the fetus was not affected; however, at birth, the infant was clinically and biochemically affected.

Neonatal screening for cystic fibrosis using immunoreactive trypsinogen and direct gene analysis: four years' experience.

Objective: To assess the performance and impact of a two tier neonatal screening programme for cystic fibrosis based on an initial estimation of immunoreactive trypsinogen followed by direct gene analysis.

Design: Four year prospective study of two tier screening strategy. First tier: immunoreactive trypsinogen measured in dried blood spot samples from neonates aged 3-5 days.

Anticardiolipin antibody-associated cerebral infarction in cirrhosis: clearance of anticardiolipin antibody after liver transplantation.

We report a 47-yr-old woman with a 30-yr history of cryptogenic cirrhosis who sustained a cerebral infarction shortly before undergoing liver transplantation. Anticardiolipin antibody titers were positive prior to transplantation (IgG = 24 GPL; IgM > 80 MPL; IgA < 10 APL). After liver transplantation, however, the titers dropped to normal (< 10 PL units).