Abstract
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 directreacting 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 jauncice, epomediol, 200 mg tid, was administered orally. There was a marked decrease of serum bilirubin with an increase in the δ bilirubin/(conjugated bilirubin + δ 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 syndrom. 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.
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References
Roy-Chowdhury J, Wolkoff AW, Arias IM. Hereditary jaundice and disorders of bilirubin metabolism. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds.) The metabolic basis of inherited disease, 6th ed. New York: McGraw-Hill, 1989;1367–1408.
Arias IM, Gartner LM, Cohen M, et al. Chronic nonhemolytic unconugated hyperbilirubinemia with glucuronyl transferase deficiency: Clinical, biochemical, pharmacologic, and genetic evidence for heterogeneity. Am J Med 1969;47:395–409.
Adachi Y, Yamamoto T. Hepatic bilirubin conjugating enzymes of man in the normal state and in liver disease. Gastroenterol Jpn 1982;17:235–240.
Gambino RS, Schreiber H. The measurement and fractionation of bilirubin on the auto analyzer by the method of Jendrassik and Grof. In: Automation in analytical chemistry, Technicon Symposia. New York: Mediad, 1964;54-1–54-11.
Adachi Y, Inufusa H, Yamashita M, et al. Clinical application of serum bilirubin fractionation by simplified liquid chromatography. Clin Chem 1988;34:385–388.
Sundberg MW, Lauff JL, Weiss JS, et al. Estimation of unconjugated, conjugated, and “delta” bilirubin fractions in serum by use of two coated thin films. Clin Chem 1984;30:1314–1317.
Yamashita M, Adachi Y, Yamamoto T. Analysis of bilirubin conjugates in human bile by column liquid chromatography. J Chromat 1986;375:386–391.
Yamashita M, Adachi Y, Yamamoto T. Analysis of bilirubin conjugates in human bile by column liquid chromatography. Changes in their composition in hepatobiliary diseases. J Gastroenterol Hepatol 1987;2:181–190.
Sherker AH, Heathcote J. Acute hepatitis in Crigler-Najjar syndrome. Am J Gastroenterol 1987;82:883–885.
Miccio M, Orzes N, Lunazzi GC, et al. Reversal of ethinylestradiol-induced cholestasis by epomediol in rat. Biochem Pharmacol 1989;38:3559–3563.
Rodriguez JV, Torres AM, Lunazzi G, et al. Effect of ethinylestradiol and epomediol on bile flow and biliary lipid composition in rat. Biochem Pharmacol 1992;43:1289–1293.
Adachi Y, Inufusa H, Yamashita M, et al. Serum bilirubin fractionation using multilayer film method in hepatobiliary diseases in comparison with high performance liquid chromatography. Gastroenterol Jpn 1987;22:633–639.
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Kagita, A., Adachi, Y., Kambe, A. et al. Type II Crigler-Najjar syndrome with intrahepatic cholestasis. J Gastroenterol 29, 214–217 (1994). https://doi.org/10.1007/BF02358686
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DOI: https://doi.org/10.1007/BF02358686