Jaundice of the familial type is almost invariably considered to be hemolytic. Congenital or familial hemolytic (acholuric) icterus is a well defined disease entity. That there are instances of familial icterus in which all the evidences of excessive blood destruction are lacking has only occasionally been recognized. We have recently observed two families in which, although bilirubinemia of the "indirect" type was present, there were the signs neither of hepatic disease nor of splenomegaly, increased fragility of the red cells or reticulocytosis. The urine was free of bilirubin and did not contain increased quantities of urobilinogen, and the results of hematologic examinations were uniformly normal. Final proof of the nonhemolytic character of the icterus in these families was obtained from study of the urobilinogen content of the feces, which was either normal or slightly decreased.
From 1900 to 1907, Gilbert and his associates1 described a series of cases of
DAMESHEK W, SINGER K. FAMILIAL NONHEMOLYTIC JAUNDICE: CONSTITUTIONAL HEPATIC DYSFUNCTION WITH INDIRECT VAN DEN BERGH REACTION. Arch Intern Med (Chic). 1941;67(2):259–285. doi:10.1001/archinte.1941.00200020021002
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