Familial Nonhemolytic Jaundice with Conjugated Bilirubin in the Serum

Abstract

CONGENITAL defects in bilirubin metabolism are a relatively infrequent cause of jaundice. If neonatal jaundice is excluded, they can be divided into two groups of cases, depending on whether the increased plasma bilirubin occurs only in the free or in the free and conjugated forms. The first group comprises patients with Gilbert's disease (constitutional hepatic dysfunction), whose jaundice is relatively mild, and cases of severe nonhemolytic jaundice, which are often accompanied by neurologic damage.^{1 2 3} The extent of the impairment in the hepatic conjugation of bilirubin may govern the degree of icterus that develops⁴ and may be due to a deficiency . . .