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.
Kagita
A
A
Second Department of Internal Medicine, Kinki University School of Medicine, Osaka, Japan.
Adachi
Y
Y
Kambe
A
A
Kamisako
T
T
Yamamoto
T
T
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