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Biomedical subjects

R H Wiesner

Publications and source records attributed to R H Wiesner.

At least 199 records · Page 11Linked to original sources
PubMed1992

Primary sclerosing cholangitis: refinement and validation of survival models.

The natural history of primary sclerosing cholangitis was studied in 426 patients from five medical centers. The median follow-up time was 3.0 years (range, 0.01-16.6 years); 100 patients had died by the time of last follow-up. Survival analysis (Cox proportional-hazards regression) was used to identify the variables most useful in predicting survival of patients with primary sclerosing cholangitis. Serum bilirubin concentration, histological stage on liver biopsy, age, and the presence of splenomegaly were independent predictors of a high risk of dying. A mathematical model to predict survival of patients with primary sclerosing cholangitis (based on referral values of those predictors) was statistically validated using two methods. Confidence intervals for predicting patient-specific survival probabilities are also presented. This model to predict survival could be used to stratify participants in therapeutic trials, counsel patients and their families, decide on candidacy for and timing of liver transplantation, and provide mathematical controls for evaluating the efficacy of therapies for primary sclerosing cholangitis, including transplantation.

Adult↗
PubMed1992

Management of ascites in patients with cirrhosis. What to do when diuretics fail.

Resistant or refractory ascites is unusual in cirrhotic patients who comply with dietary sodium restriction and optimal diuretic therapy. Patients unresponsive to medical therapy often have end-stage liver disease and renal insufficiency, although reversible complicating factors must be excluded. For patients with truly refractory ascites, liver transplantation is the only option that improves chances of survival. When this is not feasible, therapeutic paracentesis is the procedure of choice for intractable ascites. Several surgical shunts have been used, but none have been found to be safer and more effective than large-volume paracentesis.

Ascites↗
PubMed1992

A prospective study of pulmonary function and gas exchange following liver transplantation.

Pulmonary function and gas exchange were prospectively studied in 95 patients before and 9 to 15 months following liver transplantation. Pretransplant, the most common PF abnormality was impaired efficiency of gas exchange as measured by Dss. As a group, the mean Dss was 78.0 +/- 16.6 percent predicted and was found to be less than 80 percent predicted in 50 patients. As a group, patients with the most severe liver diseases clinically (Child's C classification) had the lowest mean Dss pretransplant. Posttransplant, three findings were of clinical importance: PaCO2 significantly improved posttransplantation, suggesting a resolution of pretransplant respiratory alkalosis. Expiratory airflow obstruction, measured by a change in the FEV1/FVC, was extremely uncommon posttransplant. Mean Dss improved significantly in patients with Child's C severity of liver disease. The most frequent deteriorations in Dss statistically were associated with posttransplant thoracotomy, ARDS, nonspecific pneumonitis, significant pleural effusions and hepatic retransplantation.

Female↗
PubMed1992

Antibody response to cytomegalovirus (CMV) polypeptides in liver transplant recipients with CMV hepatitis.

Immunoblotting (IB) was used to detect the presence of serum antibody against each individual CMV structural polypeptide and the major nonstructural antigen during CMV hepatitis in 12 orthotopic liver transplant recipients to evaluate the diagnostic utility of this technique. The basic phosphoprotein p150 primarily, and to a much lesser extent p65 (lower matrix protein) and p52 (nonstructural DNA-binding protein) were the most recognized antigens by both IgG and IgM class antibodies. Collectively, IgM antibodies were detected to p150 in 9 of 12 (75%) patients and were detected before (2 cases), at the same time (1 case), or after (6 cases) detection of virus in cell cultures. IB was equal to indirect immunofluorescence (IIF) and enzyme immune assay (EIA) for the detection of IgG and superior to EIA for IgM class antibodies to CMV in OLT patients. Our results indicate that the p150 phosphoroprotein is an essential component of serologic assays, especially for the detection of IgM antibodies to CMV.

Antibodies, Bacterial↗
PubMed1992

Selection and timing of liver transplantation in primary biliary cirrhosis and primary sclerosing cholangitis.

In summary, we answer the three questions we have previously posed: (a) Can liver transplantation prolong survival? Evolving data from several centers indicate that liver transplantation indeed prolongs survival in patients with PBC and PSC as compared with estimated survival using disease-specific risk scores based on the natural history of PBC and PSC. (b) Can we optimize timing of liver transplantation? Although many factors enter into the timing of liver transplantation, including when the patient is actually referred for liver transplantation and the individual desires of the patient to pursue liver transplantation, evidence is growing that having patients with chronic liver diseases like PBC and PSC undergo transplantation a little earlier in the course of the disease rather than waiting until the patients have experienced life-threatening complications or are on life-support measures can indeed improve early postliver transplant survival. In patients with PBC and PSC, the survival risk score, which reflects disease severity, can serve as an objective measurement to assess and evaluate the effect of liver disease severity on transplant outcome. Indeed, a number of studies have strongly suggested that optimal timing of liver transplantation may indeed be important to improve outcome, decrease morbidity and decrease cost. (c) Does the present allocation system in the United States allow for optimal use of our scarce donor organ resource?(ABSTRACT TRUNCATED AT 250 WORDS)

Cholangitis, Sclerosing↗
PubMed1992

Progression of primary biliary cirrhosis with ursodeoxycholic acid therapy.

Three patients with symptomatic, noncirrhotic primary biliary cirrhosis who had no evidence of esophageal varices on esophagogastroduodenoscopy and who were treated with ursodeoxycholic acid, 15 mg.kg-1.day-1, for a period of 1-2 years are reported. Initially, all three patients showed improvement in symptoms of fatigue and pruritus, and there was marked improvement or normalization in serum levels of bilirubin, alkaline phosphatase, and alanine aminotransferase. However, after 1-2 years, all three patients progressed histologically to cirrhosis on follow-up liver biopsy, and all had esophageal variceal bleeding documented by esophagogastroduodenoscopy. These three patients represent examples of ursodeoxycholic acid treatment failure despite improvements in symptoms and biochemical liver test results.

Aged↗
PubMed1992

Role of endoscopic retrograde cholangiopancreatography after orthotopic liver transplantation.

We reviewed the records of 20 liver transplant patients who underwent 28 procedures [endoscopic retrograde cholangiopancreatography (ERCP)] to rule out biliary obstruction, treat bile leaks, dilate and/or stent strictures, or remove stones and debris. Three patients (two with abnormal T-tube cholangiograms and one with hyperbilirubinemia) underwent ERCP to rule out obstruction. Therapeutic ERCP (sphincterotomy with balloon dilatation or stone extraction) was successful in 16 of 17 patients, including seven of nine in whom there was resolution of bile leaks without the use of stents or surgery. Mild pancreatitis occurring in one patient was the only complication experienced that was related to ERCP. We conclude that ERCP is a safe and important modality in the medical management of biliary tract complications after orthotopic liver transplantation.

Adult↗
PubMed1991

Current concepts in cell-mediated hepatic allograft rejection leading to ductopenia and liver failure.

Hepatic allograft rejection is presently classified into acute and chronic rejection based on histological features, timing and reversibility. However, because features of both types of rejection can occur at any time, and in many combinations, the terms "acute" and "chronic" seem inappropriate in some instances. Thus the term "cellular rejection" better defines the histological features of portal hepatitis, nonsuppurative destructive cholangitis and endotheliitis, which are independent of time and response to therapy. Similarly, because progressive bile duct destruction leading to a decrease in the number of interlobular and septal bile ducts is the major histological feature of "chronic rejection," the term "ductopenic rejection," defined as the loss of bile ducts in 50% or more of portal tracts independent of time and reversibility, seems more appropriate. The pathogenesis of cell-mediated rejection has not been completely explained; however, direct immunocytic attack on small bile ducts and small arteries appear to be the major feature. The process may lead to bile duct loss ("ductopenia"). The pathogenetic role of foam-cell arteritis resulting in ischemic bile duct injury and the role of humoral mechanisms in causing ductopenic rejection awaits further clarification. In the past, irreversible ductopenic rejection occurred in approximately 10% of all patients who underwent their first liver transplantation; this figure, however, appears to be decreasing. The clinical features of irreversible rejection include persistent and progressive cholestasis; rising serum levels of bilirubin, alkaline phosphatase and gamma-glutamyltransferase; and a decrease in hepatic synthetic function. Ductopenic rejection can occur early (2 to 5 wk after liver transplantation) but most often develops between 6 wk and 6 mo after transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1991

Primary sclerosing cholangitis: a progressive disease?

Patients with PSC should be viewed as having a disease that frequently progresses to liver failure if followed long enough. The rate of disease progression is variable and can occur without changes in symptoms or signs. With the advent of ERCP and widespread use of liver tests on routine examinations, the diagnosis of PSC is being made earlier and survival estimates have lengthened. Varying methods of survival analysis have also been responsible for differing opinions regarding the natural history of this disease. Nevertheless, it is probable that with close prospective, long-term follow-up, evidence of disease progression and increased mortality becomes apparent. Challenges for the future include being able to predict accurately the rate of disease progression and timing of liver failure in the individual patient. Preliminary studies using statistical survival modeling have been developed; however, further refinement of these models will be needed prior to application to clinical trials and liver transplantation.

Cholangitis, Sclerosing↗
PubMed1991

Cholangiocarcinoma complicating primary sclerosing cholangitis.

Cholangiocarcinoma is more likely to develop in patients with primary sclerosing cholangitis. Our aims were to describe the clinical presentation, course, and management of patients afflicted with both cholangiocarcinoma and primary sclerosing cholangitis and to estimate the prevalence of cholangiocarcinoma in patients with primary sclerosing cholangitis. A retrospective analysis was conducted of 30 patients with both primary sclerosing cholangitis and cholangiocarcinoma managed at our institution during an 8-year period. Development of cholangiocarcinoma was heralded by rapid clinical deterioration with jaundice, weight loss, and abdominal discomfort. Cholangiocarcinoma complicating primary sclerosing cholangitis often was detected at an advanced tumor stage, which precluded effective therapy, and overall median survival was 5 months. Earlier recognition and treatment of cholangiocarcinoma in such patients will be necessary to increase survival rates. Seventy patients with primary sclerosing cholangitis were followed prospectively in a clinical trial of medical therapy for an average of 30 months. Twelve patients died and five were found at autopsy to have cholangiocarcinoma. The potential for cholangiocarcinoma to develop in patients with primary sclerosing cholangitis may indicate that liver transplantation should be considered earlier in the course of the disease.

Adenoma, Bile Duct↗
PubMed1991

Elevation of plasma endothelin associated with systemic hypertension in humans following orthotopic liver transplantation.

Endothelin (ET) is a 21-amino-acid peptide of endothelial origin, is a potent systemic and renal vasoconstrictor associated with sodium retention and modulation of the renin-angiotensin-aldosterone system. The present study was designed to determine if plasma ET is elevated in humans with cirrhosis (n = 12), a state characterized by sodium retention and increased plasma renin activity (PRA) and plasma aldosterone (PA), and to determine the effect of orthotopic liver transplantation (OLT) upon plasma ET, PRA, and PA at 1, 3, and 7 days after transplantation. Plasma ET before OLT was 1.62 +/- 0.23 pg/ml, which was not different as compared with normal controls. Plasma ET significantly increased to 4.18 +/- 0.66, 3.87 +/- 0.58, and 4.07 +/- 0.61 pg/ml, respectively following OLT. PRA remained elevated throughout the postoperative course, in contrast to PA that decreased following OLT. Mean arterial pressure increased significantly from 82 +/- 4 pre-OLT to 98 +/- 4 and 103 +/- 2 mmHG on days 3 and 7 respectively.

Aldosterone↗
PubMed1991

Persistent bile leak after liver transplantation: biloma drainage and endoscopic retrograde cholangiopancreatographic sphincterotomy.

Persistent bile leaks after liver transplantation were successfully treated in three patients with percutaneous or surgical drainage of bilomas and endoscopic retrograde ampullary sphincterotomy. In all three patients, biliary anastomoses were achieved by means of choledochocholedochostomies, with a T-tube stent in the anastomosis. The bile leaks led to subhepatic bilomas that had persistently high catheter outputs (greater than 200 mL per 24 hours) for longer than 1 month. Biloma drainage decreased shortly after sphincterotomy.

Ampulla of Vater↗
PubMed1991

Primary sclerosing cholangitis: value of cholangiography in determining the prognosis.

We studied cholangiograms in 129 patients with primary sclerosing cholangitis (PSC) to determine if there was a correlation between any of the findings and the prognosis of the disease. The grade, length, and extent of strictures, the degree of bile duct dilatation, and the distribution of lesions were evaluated. Survival curves were generated to test the association of these radiologic signs with subsequent survival. High-grade intrahepatic duct strictures (greater than 75% narrowing) were associated with a 19% decrease in 3-year survival (p = .05) compared with lower-grade strictures. Diffuse intrahepatic strictures (involving greater than 25% of the ducts) were associated with a 16% decrease in 3-year survival (p = .012) compared with localized strictures. Statistically insignificant (p greater than .05) but measurable decreases in survival were observed with high-grade extrahepatic duct strictures, diffuse involvement of the extrahepatic ducts, long confluent strictures anywhere in the biliary tree, and marked dilatation of the intrahepatic ducts. In general, intrahepatic duct disease was found to have greater prognostic significance than extrahepatic duct disease. High-grade strictures and diffuse strictures of the intrahepatic ducts were found to be indicators of a poor prognosis in PSC and were more predictive of a poor prognosis than was extrahepatic duct disease.

Adult↗
PubMed1991

Epstein-Barr virus and persistent graft dysfunction after liver transplantation.

Epstein-Barr virus infection has been associated with a broad spectrum of clinical manifestations, depending on the immune status of the host. In this report, we describe two liver transplant patients who received hepatic allografts from donors serologically positive for Epstein-Barr virus and who experienced primary infection with Epstein-Barr virus associated with prolonged liver graft dysfunction. In both patients, Epstein-Barr serologies converted within 3 mo of liver transplantation, and hepatic histological study revealed mononuclear infiltration of the sinusoids evolving to pronounced immunoblastic features suggestive of evolving lymphoma. In both cases, in situ hybridization studies confirmed the presence of Epstein-Barr virus genome in the liver. Furthermore, polymerase chain reaction analysis suggested that high levels of Epstein-Barr virus DNA were present in biopsy specimens obtained during the episode of acute hepatitis that followed Epstein-Barr virus seroconversion. The degree of Epstein-Barr virus DNA estimated by polymerase chain reaction appeared to increase in parallel with the progression of parenchymal lymphocytic infiltrates. In one patient, a biopsy sample from a cervical node also revealed high levels of Epstein-Barr virus DNA estimated using the polymerase chain reaction technique. Furthermore, in these patients, Epstein-Barr virus DNA levels appeared to decrease dramatically after discontinuing azathioprine administration and beginning treatment with acyclovir. These two cases illustrate the dynamics of Epstein-Barr virus immune regulation and confirm chronic hepatic allograft dysfunction related to Epstein-Barr viral infection.

Adult↗
PubMed1991

The metabolic bone disease of primary sclerosing cholangitis.

The incidence and severity of osteopenic bone disease in primary sclerosing cholangitis is poorly defined. Clinical, biochemical and radiographic assessment and bone mineral density measurements of the lumbar spine were carried out in two groups of patients. Group 1 consisted of 30 patients with advanced primary sclerosing cholangitis; group 2 consisted of 18 patients with newly diagnosed primary sclerosing cholangitis. Only one patient had bone pain. All patients were normocalcemic; two had elevated serum parathormone levels. Fourteen patients (47%) from group 1 but no patients from group 2 had low serum 25-hydroxyvitamin D levels. Mean bone mineral density was significantly reduced in group 1 patients (0.97 +/- 0.04 gm/cm2) compared with age-matched and sex-matched controls (1.25 +/- 0.01 gm/cm2, p less than 0.0001), and in 15 patients (50%) bone mineral density was below the fracture threshold (0.98 gm/cm2). The bone mineral density in group 2 was not significantly different from controls, and no patient was below the fracture threshold. In neither group did bone mineral density correlate with serum bilirubin, 25-hydroxyvitamin D, fecal fat excretion, previous drug therapy or the presence of chronic ulcerative colitis. Histomorphometrical examination of bone from four group 1 patients showed increased bone resorption, reduced bone formation, moderate-to-severe osteopenia and no osteomalacia. In conclusion, severe osteopenic bone disease is common in advanced primary sclerosing cholangitis and, like that seen in other cholestatis diseases, is consistent with osteoporosis.

Adult↗