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Cholestatic jaundice associated with primary amyloidosis

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Abstract

Hepatic involvement in amyloidosis is common1–3 and the clinical manifestations vary. A normal or enlarged liver and normal liver function tests are compatible with extensive hepatic infiltration by amyloid. The most sensitive tests of this condition4 are bromsulphalein excretion and serum alkaline phosphatase. Rarely amyloidosis of the liver can present features of portal hypertension with esophageal varices4, 5 or ascites.6 However, the usual stigmata of chronic liver disease such as spider nevi, gynecomastia, and palmar erythema are absent.4 Jaundice is uncommon.4 Only 4.7% of 490 patients with hepatic amyloidosis were jaundiced; serum bilirubin generally did not exceed 5 mg/100 ml.7 However, severe obstructive jaundice caused solely by hepatic amyloidosis presents a difficult differential diagnostic problem. Only five cases of obstructive jaundice due to amyloidosis have been reported to date.7–11 We report an additional case of severe cholestatic jaundice caused by primary amyloidosis and discuss the pertinent diagnostic problems.

Case report

A 65-year-old man was referred to the Cleveland Clinic in November 1971 for evaluation. Two years before admission he had an episode of abdominal pain diagnosed as “gallbladder colic.” He did well for one year, when right-sided Bell’s palsy developed. A persistent elevation of serum alkaline phosphatase of twice the normal value was found at this time. Roentgenograms disclosed no evidence of Paget’s disease or metastatic neoplasm. An oral cholecystogram did not show the gallbladder. In February 1971 the patient began to feel weak and nauseous and became anorectic. On hospitalization in July 1971 the left lobe of . . .


 

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