Abstract:
:Phenobarbital response, bile pigment composition, and the fractional biliary excretion ratio of bilirubin were studied in nine children with Crigler-Najjar disease. In five children, serum bilirubin levels decreased during phenobarbital treatment by 26% or more and the pigment composition in bile changed with a decrease in the proportion of unconjugated bilirubin from 33% +/- 12% to 13% +/- 1% and an increase in monoconjugates and diconjugates from 57% +/- 14% and 10% +/- 2%, respectively, to 72% +/- 4% and 16% +/- 3%. In four children, serum bilirubin levels did not change significantly during phenobarbital treatment. In these patients, bile pigments comprised 91% +/- 10% unconjugated bilirubin, 9% +/- 11% monoconjugates, and 1% +/- 1% diconjugates. On the basis of these differences, the former group can be classified as having type 2 Crigler-Najjar disease and the latter, type 1. Bile pigment analysis in parents of patients with Crigler-Najjar disease showed an increased proportion of monoconjugates in at least one of the partners in three of four couples tested, despite normal serum bilirubin levels. Serum bilirubin levels were about the same in type 1 and 2 patients and amounted to 236 +/- 62 mumol/L and 214 +/- 82 mumol/L, respectively. In addition the fractional bilirubin excretion ratio, calculated as the ratio ([bilirubin in bile]/[bilirubin in serum])/([bile acid in bile]/[bile acid in serum]) could not differentiate between these two groups. However, there was a 10-fold and 100-fold difference of this ratio between patients with Crigler-Najjar disease and those with Gilbert's syndrome and between patients with Crigler-Najjar disease and controls. The fractional bilirubin excretion ratio proved an excellent tool to differentiate between Gilbert's syndrome and Crigler-Najjar disease, whereas Crigler-Najjar disease types 1 and 2 could be differentiated on the basis of bile pigment analysis.
journal_name
Gastroenterologyjournal_title
Gastroenterologyauthors
Sinaasappel M,Jansen PLdoi
10.1016/0016-5085(91)80026-6subject
Has Abstractpub_date
1991-03-01 00:00:00pages
783-9issue
3eissn
0016-5085issn
1528-0012pii
0016-5085(91)80026-6journal_volume
100pub_type
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