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Chronic Idiopathic Jaundice with Unidentified Pigment in Liver Cells

AMA Arch Intern Med. 1957;99(2):294-297. doi:10.1001/archinte.1957.00260020130019.
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In 1954 Dubin and Johnson 1 reported 12 cases of idiopathic jaundice with an unidentified pigment in the centrilobular liver cells. Clinically, these patients could have been classified as cases of "familial hyperbilirubinemia" or chronic hepatitis. However, with the unusual liver biopsy finding of a previously undescribed pigment, a new clinical entity was suggested. Since the original description, four additional cases have been recorded.2-5 It is our purpose to record an additional case of this entity.

Clinically, in addition to mild recurring icterus of unexplainable cause, these cases are characterized by (1) vague abdominal discomfort, (2) dark urine which is positive for bilirubin, (3) hepatomegaly, (4) poor visualization of the gall bladder by oral dye methods, (5) one-minute bilirubin values which are elevated to at least 50% of the total bilirubin and an otherwise benign course with a good prognosis for life.

The diagnosis of these cases can only


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