PEDIATRICS Vol. 45 No. 5 May 1970, pp. 812-820
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IDIOPATHIC, RECURRENT CHOLESTASIS

Frederick B. Ruymann Maj., M.C.1, Akio Takeuchi M.D.1, and H. Worth Boyce LTC., M.C.1

1 The Departments of Hematology and Experimental Pathology, Walter Reed Army Institute of Resarch and The Gastroentrology Service, Walter Reed General Hospital, Washington, D.C.

Idiopathic, recurrent cholestasis is characterized by episodes of obstructive jaundice often preceded by pruritus, steatorrhea, and purpuric rash. Between episodes of jaundice the liver histology is normal. We report a case of idiopathic, recurrent cholestasis occurring in a 14-year-old Caucasian girl who has experienced seven episodes of jaundice since 8 years of age. Serum alkaline phosphatase elevation preceded clinical jaundice. During remission the bromsulphalein excretion was normal. Response to oral steroids occurred when used after 4 weeks of jaundice. Cholestyramine resin and cyproheptadine relieved pruritus and in combination may have prevented progressive jaundice when administered early. Light microscopy during exacerbation showed centrilobular cholestasis with a slight mononuclear infiltration of the portal triad.

The electron microscopic examinations demonstrate dilated bile canaliculi with altered microvilli. Serial liver biopsies over 6 years show no fibrosis or cytoplasmic alterations of hepatocytes. Of the 24 reported cases of idiopathic, recurrent cholestasis, two-thirds have been in males and had onset of jaundice under 20 years of age. This younger age of onset merits a greater awareness by the practicing pediatrician. After the diagnosis is established by clinical, biochemical, and morphologic studies, a benign course can be anticipated.

Submitted on September 19, 1969
Accepted on November 6, 1969