What are the nonsurgical options for Crigler-Najjar syndrome type 2?

Updated: May 21, 2019
  • Author: Hisham Nazer, MBBCh, FRCP, DTM&H; Chief Editor: BS Anand, MD  more...
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Answer

Phenobarbital therapy has been shown to be effective in reducing plasma bilirubin levels in patients with Crigler-Najjar syndrome type 2. Administration of 60-180 mg/day of the drug (in divided doses) can reduce serum bilirubin levels by at least 25%. A response should be expected within 2-3 weeks. A similar benefit can be observed with clofibrate, which is associated with fewer adverse effects. (Clofibrate is no longer on the US market.) However, patients with the type 2 syndrome often do well even without therapy.

In rare cases, however, patients with Crigler-Najjar syndrome type 2 require exchange transfusions or long-term phototherapy.


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