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Title: An unusual case of Crigler-Najjar disease in the adult. Classification into types I and II revisited
Authors: Duhamel, G ×
Blanckaert, Norbert
Metreau, J M
Préaux, A M
Bouvry, M
Fevery, Johan
Berthelot, P #
Issue Date: Dec-1985
Series Title: Journal of hepatology vol:1 issue:1 pages:47-53
Abstract: We present the case of a 25-year-old man with Crigler-Najjar disease who had since birth a marked unconjugated hyperbilirubinemia without bilirubin overproduction, without any neurological involvement and in whom phenobarbital administration failed to produce any effect. Analysis of his biliary bile pigments on two occasions showed (i) a decrease excretion of bilirubin, as indirectly suggested by a high ratio of biliary bile acids over total bilirubin; (ii) an increase in unconjugated bilirubin IX alpha quantitated by thin-layer chromatography (TLC) following alkaline methanolysis and by direct extraction and TLC of the tetrapyrroles; (iii) a high proportion of bilirubin monoconjugates whereas the excretion of diconjugates was very low. Classification of the present patient into Crigler-Najjar disease type I or II was not possible. The most striking and practical difference among the various cases of Crigler-Najjar disease remains the response to phenobarbital. Among cases of Crigler-Najjar disease which respond to enzyme induction and Gilbert's syndrome, the continuous spectrum suggests a common defect.
ISSN: 0168-8278
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Laboratory of Clinical Bacteriology and Mycology
Department of Imaging & Pathology - miscellaneous
× corresponding author
# (joint) last author

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