What is the prognosis of 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

Kernicterus in infancy or later in life is the main cause of death in Crigler-Najjar syndrome type 1. This disease can also result in permanent neurologic sequelae, due to bilirubin encephalopathy. Even with treatment, most, if not all, patients with Crigler-Najjar (CN) syndrome type 1 eventually develop some neurologic deficit.

Unless treated vigorously (ie, orthotopic liver transplant, segmental transplantation), most patients with Crigler-Najjar syndrome type 1 die by age 15 months. Fortunately, more patients are surviving to adulthood because of advances in the treatment of hyperbilirubinemia.

Neonates of mothers with Crigler-Najjar syndrome type 1 develop adverse outcomes, including developmental delay, hearing loss, and cerebellar syndrome. [49]


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