Type II crigler-Najjar syndrome with intrahepatic cholestasis.
A 58-year-old Japanese man was admitted to our hospital with appendicitis and marked unconjugated hyperbilirubinemia (11.6 mg/dl). The jaundice worsened following appendectomy, and the direct-reacting bilirubin increased, probably due to the ceftizoxime administered postoperatively. Bilirubin diglucuronide was the main component of the serum direct-reacting bilirubin (51%) in serum measured by liquid chromatography. Because the discontinuation of ceftizoxime did not markedly improve the jaundice, epomediol, 200 mg tid, was administered orally. There was a marked decrease of serum bilirubin with an increase in the delta bilirubin/(conjugated bilirubin + delta bilirubin) ratio. After improvement of jaundice to below the pre-surgical level (4.4 mg/dl), we analyzed the duodenal bile for bilirubin fractions; those showed a marked reduction in bilirubin diglucuronide and a marked increase in bilirubin monoglucuronide, which was consistent with type II Crigler-Najjar syndrome. A marked increase of bilirubin diglucuronide in serum of this patient during cholestasis suggests that bilirubin conjugation proceeds in this syndrome when excretion of conjugated bilirubin decreases.
Second Department of Internal Medicine, Kinki University School of Medicine, Osaka, Japan.
A
A
Kagita
Y
Y
Adachi
A
A
Kambe
T
T
Kamisako
T
T
Yamamoto
D015296:Ceftizoxime / Q000009:adverse effects; D002780:Cholestasis, Intrahepatic / Q000150:complications*; D003414:Crigler-Najjar Syndrome / Q000150:complications*; D006801:Humans; D006932:Hyperbilirubinemia / Q000139:chemically induced / Q000150:complications; D008297:Male; D008875:Middle Aged
D015296:Ceftizoxime