A case with chronic nonhemolytic unconjugated hyperbilirubinemia (Crigler-Najjar syndrome Type II) with special reference of pathogenesis and classification of unconjugated hyperbilirubinemia

• Published in: Kanzo Vol. 19; no. 1; pp. 22 - 32
• Main Authors: NAMBU, Masaji, YAMASHIRO, Yuji, OIKAWA, Yoko, KOBAYASHI, Norio, NAMIHISA, Toshihiko
• Format: Journal Article
• Language: Japanese
• Published: The Japan Society of Hepatology 25.01.1978
• ISSN: 0451-4203; 1881-3593
• DOI: 10.2957/kanzo.19.22

A patient, 40 year-old woman, with nonhemolytic unconjugated hyperbilirubinemia was reported. Jaundice was demonstrated in 9 of 11 siblings and all of them died at age three or four with kernicterus. Plasma disappearance of ICG and BSP showed normal initial disappearance. The serum bilirubin concentration was decreased and the bile bilirubin concentration was increased after an administration of bucolome. Most of the increased bilirubin in bile was ester form of direct bilirubin. Hepatic glucuronyltransferase revealed low activity. The elution pattern of liver supernate mixed with ICG or BSP remained the same as that of normal subject. These results suggest that the increased unconjugated bilirubin in the plasma is the results of a defect in hepatic bilirubin conjugation and of increased back flow of unconjugated bilirubin from the liver to the plasma. We classified unconjugated hyperbilirubinemias into four groups as follow: I, 1) Serum bilirubin concentration 10-50mg/dl, 2) The patient dies in infancy with kernicterus, 3) Familial occurrence is usual, 4) Effect of phenobarbital or bucolome on serum bilirubin is none. II, 1) 5-20mg/dl, 2) The patient survives into adult life. Kernicterus sometimes, 3) usual, 4) effective, III, 1) 2-5mg/dl, 2) The patient is well for life. Kernicterus never, 3) usual, 4) effective. IV, 1) 2-5mg/dl, 2) The patient is well for life. Kernicterus never, 3) never (?), 4) effective.