Abstract

An additional case is reported of a new clinicopathologic entity which has recently been described by Dubin and Johnson, and Sprinz and Nelson as “chronic idiopathic jaundice with unidentified pigment in liver cells.” The evolution of the literature on the subject is traced, and a comparison with Gilbert's disease is offered. The disorder is attributed to an error in liver metabolism. Alcoholism and intercurrent illness may act as precipitating or aggravating factors. Clinical features include persistent, low grade, non-hemolytic jaundice, lassitude, upper abdominal pain and slight enlargement and tenderness of the liver. Direct reacting bilirubin is elevated and forms a major portion of the total. Bilirubinuria, abnormal serocoagulation tests, delayed bromsulphthalein excretion and failure to see the gallbladder on cholecystography may be demonstrated on occasion. The patient's general health is not impaired, and prognosis appears to be good. A constant and diagnostic histologie finding is the presence of coarse, brown lipofuscin pigment within pericentrally placed liver cells. Histochemical reactions which permit characterization of this pigment are outlined and discussed.

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