Crigler-Najjar syndrome

Crigler-Najjar syndrome (CNS) is a hereditary disorder of bilirubin metabolism characterized by unconjugated hyperbilirubinemia due to a hepatic deficit of bilirubin glucuronosyltransferase (GT) activity. Two types have been described, CNS types 1 and 2 (see these terms). CNS1 is characterized by a complete deficit of the enzyme and is unaffected by phenobarbital induction therapy, whereas the enzymatic deficit is partial and responds to phenobarbital in CNS2.

Cholestasis

Impairment of bile flow due to obstruction in bile ducts.


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(per page)
PMID (PMCID)
6872808
MALE Adult
Effect of phenobarbital on serum and biliary parameters in a patient with Crigler-Najjar syndrome, type II and acquired cholestasis.
Trotman BW, Shaw L, Roy-Chowdhury J, Malet PF, Rosato EF.
Dig Dis Sci. 1983;28(8):753-62.
The effect of phenobarbital treatment on bilirubin metabolism and bile secretion was studied in a patient with Crigler-Najjar syndrome, type II and acquired cholestasis.