A new type of constitutional hyperbilirubinemia with unconjugated bilirubin-A case of nonhemolytic hyperbilirubinemia with marked retention of sulfobromophthalein test

Abstract

We reported a case of 29 year-old male with nonhemolytic unconjugated hyperbilirubinemia (continued since 15 year old) and with marked retention of BSP test without retention of ICG test.
The plasma disappearance rate of BSP showed 0.030 and plasma retention rate at 45 minutes was 25.6%, whereas ICG plasma disappearance rate revealed 0.154 and plasma retention rate at 15 minutes was 9.8%.
The serum bilirubin concentration was ranges between 2.1mg/dl to 3.0mg/dl, and unconjugated bilirubin was dominant. The bilirubin levels were elevated after the intake of low calorie diet (400Cal/day) for 3 days, and lowered after the administration of phenobarbital (100mg/day) for 7 days.
Bilirubin composition in bile showed decrease of molar ratio of glucuronic acid to ester form of bilirubin. This result suggested the decreased activity of UDP glucuronyl transferase in liver cells.
The hepatic uptake of ^99mTc-HIDA was retarded and the excretion rate was within normal. Hepatic uptake and excretion of ^99mTc-PI were almost normal. The secondary rise was observed at 180 minutes on the plasma disappearance curve of UDCA following an oral administration of UDCA (300mg).
The elution pattern of serum protein conjugated with ICG or BSP using column chromatography (Sephadex G-200) showed the same result as of healthy subjects.
From above mentioned results it was concluded that this case was a new type of constitutional hyperbilirubinemia with marked retention of BSP.