Chronic Idiopathic Nonhemolytic Jaundice (Dubin-Johnson Syndrome)
WILLIAM H. SHAFER, M.D.
Department of Internal Medicine
FRED F. WHITCOMB, M.D.
Department of Gastroenterology
CHRONIC idiopathic nonhemolytic jaundice was independently described by Dubin and Johnson1 and by Sprinz and Nelson2 in 1954. Dubin3 extensively reviewed the clinical features of the 50 cases that had been reported up to 1958. The commonest clinical findings are jaundice, abdominal pain, fatigue, dark urine, and slight hepatomegaly. The jaundice is due to the presence of both free and conjugated bilirubin in the blood. The characteristic pathologic finding is the presence of an intracellular lipochrome pigment in an otherwise normal-appearing liver. The patient described in this report has many of the features of this unusual syndrome.
Report of a Case
A 35-year-old housewife was in good health until November, I960. At that time a severely sore throat, extreme fatigue, bloating, recurrent vomiting, and several episodes of moderately severe pain in the right upper quadrant occurred. On roentgen study at another hospital, the gallbladder was poorly visualized.
The symptoms of fatigue, bloating, and pain in the right upper quadrant continued. In February, 1961, three months after the initial symptoms occurred, a mild jaundice was noted for the first time. She was hospitalized for study. Hepatic function tests showed the total serum bilirubin to be 1.65 mg. per 100 ml., and the direct reacting bilirubin to be 1.15 mg. per 100 ml. The sulfobromophthalein test showed 37 per cent retention in 45 minutes. The serum alkaline phosphatase content, serum glutamic transaminase level, serum protein electrophoretic pattern, serum cholesterol content, and serum cholesterol esters were normal. On hepatic biopsy the gross specimen. . .