Chapter 043. Jaundice (Part 9)

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Choledocholithiasis is the most common cause of extrahepatic cholestasis. The clinical presentation can range from mild right upper quadrant discomfort with only minimal elevations of the enzyme tests to ascending cholangitis with jaundice, sepsis, and circulatory collapse. PSC may occur with clinically important strictures limited to the extrahepatic biliary tree. In cases where there is a dominant stricture, patients can be effectively managed with serial endoscopic dilatations. Chronic pancreatitis rarely causes strictures of the distal common bile duct, where it passes through the head of the pancreas. AIDS cholangiopathy is a condition, usually due to infection of the bile...

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Chapter 043. Jaundice (Part 9) Choledocholithiasis is the most common cause of extrahepatic cholestasis. The clinical presentation can range from mild right upper quadrant discomfort with only minimal elevations of the enzyme tests to ascending cholangitis with jaundice, sepsis, and circulatory collapse. PSC may occur with clinically important strictures limited to the extrahepatic biliary tree. In cases where there is a dominant stricture, patients can be effectively managed with serial endoscopic dilatations. Chronic pancreatitis rarely causes strictures of the distal common bile duct, where it passes through the head of the pancreas. AIDS cholangiopathy is a condition, usually due to infection of the bile duct epithelium with CMV or cryptosporidia, which has a cholangiographic appearance similar to that of PSC. These patients usually present with greatly elevated serum alkaline phosphatase levels (mean, 800 IU/L), but the bilirubin is often near normal. These patients do not typically present with jaundice.
Summary The goal of this chapter is not to provide an encyclopedic review of all of the conditions that can cause jaundice. Rather, it is intended to provide a framework that helps a physician to evaluate the patient with jaundice in a logical way (Fig. 43-1). Simply stated, the initial step is to obtain appropriate blood tests to determine if the patient has an isolated elevation of serum bilirubin. If so, is the bilirubin elevation due to an increased unconjugated or conjugated fraction? If the hyperbilirubinemia is accompanied by other liver test abnormalities, is the disorder hepatocellular or cholestatic? If cholestatic, is it intra- or extrahepatic? All of these questions can be answered with a thoughtful history, physical examination, and interpretation of laboratory and radiologic tests and procedures. Further Readings Bosma PJ: Inherited disorders of bilirubin metabolism. J Hepatol 38:107, 2003 [PMID: 12480568]
Ferenci P: Wilson's disease. Clin Gastroenterol Hepatol 3:726, 2005 [PMID: 16233999] Fox IJ et al: Treatment of the Crigler-Najjar syndrome type I with hepatocyte transplantation. N Engl J Med 338:1422, 1998 [PMID: 9580649] Glasova H, Beuers U: Extrahepatic manifestations of cholestasis. J Gastroenterol Hepatol 9:938, 2002 Pratt DS, Kaplan MM: Laboratory tests, in Schiff's Diseases of the Liver, 9th ed, ER Schiff et al (eds). Philadelphia, Lippincott Williams & Wilkins, 2003 Trauner Met al: Molecular pathogenesis of cholestasis. N Engl J Med
339:1217, 1998 [PMID: 9780343] Bibliography Berg CL et al: Bilirubin metabolism and the pathophysiology of jaundice, in Schiff's Diseases of the Liver, 9th ed, ER Schiff et al (eds). Philadelphia, Lippincott Williams & Wilkins, 2003 Berk PD, Noyer C (eds): Bilirubin metabolism and the hereditary hyperbilirubinemias. Semin Liv Dis 14:321, 1994 [PMID: 7855625] Zimmerman HJ: Hepatoxicity: The Adverse Effects of Drugs and Other Chemicals on the Liver, 2d ed. Philadelphia, Lippincott Williams & Wilkins, 1999