Benign Recurrent Cholestasis

Abstract
The literature on benign recurrent cholestasis is reviewed, and three personal illustrative cases are briefly described. Benign recurrent cholestasis was familial in 31 % of the total of 39 known cases, and there was a significant male preponderance. The disease consisted in attacks of jaundice and pruritus separated by symptom-free intervals. The age at the time of the first episode ranged from 8 months to 59 years, but in 79% of cases it was under 20 years. The duration of the attacks ranged from 2 days to 24 months. The shortest asymptomatic interval recorded was 1 month, and the longest was 20 years (mean 15 months). During the asymptomatic periods the clinical, laboratory, and morphologic findings were normal. The cause is not known. Treatment is purely symptomatic: it consists in relieving or eliminating pruritus (chiefly by administration of cholestyramine) and in combating the effects of malabsorption. The prognosis for life is good, but there is no known way of shortening the attacks or of reducing their frequency. There is as yet no evidence that any permanent morphologic changes occur in the liver.