Glucuronide Formation in Patients with Constitutional Hepatic Dysfunction (Gilbert's Disease)

Abstract

IT has been shown that bilirubin has to be converted to its glucuronide or sulfate^{1 2 3 4} before it can be excreted in the bile. Retention of nonconjugated bilirubin in the plasma, associated with an enzymatic defect in glucuronide formation in the liver, has been demonstrated in a strain of rats with congenital icterus.^{5 , 6} A similar defect in glucuronide formation has been found in children with severe congenital nonhemolytic jaundice (Crigler–Najjar syndrome).^{7 8 9} It has been suggested that, in patients with constitutional hepatic dysfunction or so-called Gilbert disease,¹⁰ the mild and intermittent icterus may be the result of a similar but less severe . . .