Crigler-Najjar syndrome type Ii in a 17-year-old girl
Schwegler, U.; May, B.; Müller, K.M.
Zeitschrift für Gastroenterologie 31(Suppl 2): 83-84
1993
ISSN/ISBN: 0044-2771 PMID: 7483726 Document Number: 408755
The Crigler-Najjar-Syndrome type II is characterised by the decreased activity of the bilirubin-UDP-glucuronyltransferase. This fact leads to an increase of the indirect bilirubin to 6-10 mg/dl. The mode of hereditary transmission is autosomal dominant with variable penetrance. The age of manifestation varies from the first year to the second decade of life. The Crigler-Najjar-Syndrome type II of a 17-year-old girl is reported. The diagnosis was made on the basis of the pattern of laboratory parameters in combination with an uneventful morphology of the liver. Under treatment with Chlofibrat the decrease of the serum total bilirubin from 10.4 mg/dl to 2 mg/dl was achieved.