An unusual presentation of primary sclerosing cholangitis.

This case report describes the unusual presentation of a patient who had findings which were initially suggestive of a type IV choledochal cyst. Her liver biopsy demonstrated biliary cirrhosis. She was treated with endoscopic retrograde cholangiopancreatography and biliary stent exchanges over one year. Her cholangiogram one year later demonstrated resolution of the biliary cystic dilation which led to her initial diagnosis, with beading and stricturing of the hepatic ducts consistent with primary sclerosing cholangitis. Liver-associated enzymes and physical findings also improved. A liver biopsy one year later demonstrated a marked improvement in hepatic fibrosis with no evidence of cirrhosis.

False positive acetaminophen concentrations in patients with liver injury.

BACKGROUND: Acetaminophen toxicity is the most common form of acute liver failure in the U.S. After acetaminophen overdoses, quantitation of plasma acetaminophen can aid in predicting severity of injury. However, recent case reports have suggested that acetaminophen concentrations may be falsely increased in the presence of hyperbilirubinemia. METHODS: We tested sera obtained from 43 patients with acute liver failure, mostly unrelated to acetaminophen, utilizing 6 different acetaminophen quantitation systems to determine the significance of this effect. In 36 of the 43 samples with bilirubin concentrations ranging from 1.0-61.5 mg/dl no acetaminophen was detectable by gas chromatography-mass spectroscopy. These 36 samples were then utilized to test the performance characteristics of 2 immunoassay and 4 enzymatic-colorimetric methods. RESULTS: Three of four colorimetric methods demonstrated 'detectable' values for acetaminophen in from 4 to 27 of the 36 negative samples, low concentration positive values being observed when serum bilirubin concentrations exceeded 10 mg/dl. By contrast, the 2 immunoassay methods (EMIT, FPIA) were virtually unaffected. The false positive values obtained were, in general, proportional to the quantity of bilirubin in the sample. However, prepared samples of normal human serum with added bilirubin showed a dose-response curve for only one of the 4 colorimetric assays. CONCLUSIONS: False positive acetaminophen tests may result when enzymatic-colorimetric assays are used, most commonly with bilirubin concentrations >10 mg/dl, leading to potential clinical errors in this setting. Bilirubin (or possibly other substances in acute liver failure sera) appears to affect the reliable measurement of acetaminophen, particularly with enzymatic-colorimetric assays.

Hemodialysis for hyperammonemia associated with ornithine transcarbamylase deficiency.

Acute hyperammonemia is a medical emergency requiring rapid recognition and treatment to prevent devastating neurologic sequelae. Its varying etiologies include primary hepatic failure, drug toxicity, infection, and inherited disorders of metabolism. Ornithine transcarbamylase (OTC) deficiency is the most common inherited urea cycle disorder and can result in hyperammonemic encephalopathy and coma, often presenting in the newborn or early childhood. Partial deficiencies of the enzyme can present later in adulthood with protean neuropsychiatric signs and symptoms. Early recognition and management of metabolic encephalopathy is crucial to avoid neurologic damage, and may require hemodialysis for rapid removal of ammonia, with adjunctive medications and dietary modifications to decrease endogenous nitrogen production and activate alternate pathways of nitrogen excretion. We present the case of an adult patient with partial OTC deficiency who presented with encephalopathy, coma, and seizures, accompanied by hyperammonemia and treated acutely with hemodialysis.

The cost-effectiveness of CT colonography in screening for colorectal neoplasia.

BACKGROUND: We examined the cost-effectiveness of 2- and 3-dimensional computerized tomography (CT) colonography as a screening test for colorectal neoplasia. METHODS: We created a Markov model of the natural history of colorectal cancer. Effectiveness of screening was based upon the diagnostic accuracy of tests in detecting polyps and cancer. RESULTS: CT colonography every 5 or 10 yr was effective and cost-effective relative to no screening. Optical colonoscopy dominates 2-dimensional CT colonography done every 5 or 10 yr. Optical colonoscopy is weakly dominant over 3-dimensional CT colonography done every 10 yr. 3-D CT colonography done every 5 yr is more effective than optical colonoscopy every 10 yr, but costs an incremental 156,000 dollars per life-year gained. Sensitivity analyses show that test costs, accuracy, and adherence are critical determinants of incremental cost-effectiveness. 3-D CT colonography every 5 yr is a dominant strategy if optical colonoscopy costs 1.6 times more than CT colonography. However, optical colonoscopy is a dominant strategy if the sensitivity of CT colonography for 1 cm adenomas is 83% or lower. CONCLUSIONS: CT colonography is an effective screening test for colorectal neoplasia. However, it is more expensive and generally less effective than optical colonoscopy. CT colonography can be reasonably cost-effective when the diagnostic accuracy of CT colonography is high, as with primary 3-dimensional technology, and if costs are about 60% of those of optical colonoscopy. Overall, CT colonography technology will need to improve its accuracy and reliability to be a cost-effective screening option.

Impact of diabetes and hepatitis after kidney transplantation on patients who are affected by hepatitis C virus.

Complications associated with use of donor hepatitis C-positive kidneys (DHCV+) have been attributed primarily to posttransplantation liver disease (as a result of hepatitis C disease). The role of posttransplantation diabetes has not been explored in this setting. With the use of the United States Renal Data System database, 28,942 Medicare KT recipients were studied from January 1, 1996, through July 31, 2000. Cox proportional hazards regression models were used to calculate adjusted hazard ratios (AHR) for the association of sero-pairs for HCV (D+/R-, D+/R+, D-/R+ and D-/R-) with Medicare claims for de novo posttransplantation HCV and posttransplantation diabetes. The peak risk for posttransplantation HCV was in the first 6 mo after transplantation. The incidence of posttransplantation HCV after transplantation was 9.1% in D+/R-, 6.3% in D+/R+, 2.4% in D-/R+, and 0.2% in D-/R-. The incidence of posttransplantation diabetes after transplantation also peaked early and was 43.8% in D+/R-, 46.6% in D+/R+, 32.3% in D-/R+, and 25.4% in D-/R-. Associations for both complications were significant in adjusted analysis (Cox regression). Both posttransplantation HCV (AHR, 3.36; 95% confidence interval, 2.44 to 4.61) and posttransplantation diabetes (AHR, 1.81; 95% confidence interval, 1.54 to 2.11) were independently associated with an increased risk of death, but posttransplantation diabetes accounted for more years of life lost, particularly among recipients of DHCV+ kidneys. Posttransplantation diabetes may contribute substantially to the increased risk of death associated with use of DHCV+ kidneys and accounts for more years of life lost than posttransplantation HCV. Because HCV infection acquired after transplantation is so difficult to treat, methods that have been shown to reduce viral transmission warrant renewed attention.