What is the therapeutic response to nonsurgical correction of bilirubin levels in Crigler-Najjar syndrome type 1?

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

Therapeutic response varies according to the type of Crigler-Najjar syndrome being treated. Crigler-Najjar syndrome type 1 does not respond to phenobarbital therapy, and patients may require repeated exchange transfusions followed by long-term phototherapy to prevent neurologic complications.

Other therapies include plasmapheresis, hemoperfusion, and the administration of cholestyramine, calcium phosphate, oral agar, and orlistat. [3] An approach to therapy using Sn-protoporphyrin, a heme oxygenase inhibitor, was introduced to prevent an increase in serum bilirubin levels. [4] In patients with Crigler-Najjar syndrome type 1, however, liver transplantation remains the only guaranteed form of therapy. This surgery should occur prior to the onset of kernicterus.


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