Criggler-Najjar, syndrome
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Autosomal recessive transmission of a mutation of the UGT1A 1gene (2q37). Jaundice due to unconjugated bilirubin due to complete bilirubin-glucuronyl transferase deficiency.
Types:
- type I (severe form) [MIM 218 800]: unconjugated hyperbilirubinemia that does not or poorly responds (< 20 % decrease) to the administration of phenobarbital (5-10 mg/kg/day). Phototherapy should be continued while waiting for a liver transplantion; risk of kernicterus
- type II [MIM 616785]: mild form; phenobarbital is effective but the bilirubin level increases in case of fasting or infection.
Anesthetic implications:
avoid substances that may compete with bilirubin to bind to albumin. Paracetamol is metabolized in part by this glucuronyl transferase: an increased risk of toxicity is therefore theoretically possible. Morphine is metabolized by a different glucuronyl transferase.
References :
- Robards C, Brull SJ.
The anesthetic implications of Crigler-Najar syndrome.
Anesth Analg 2007; 104: 435-6.
- Watchko JF.
Genetics and pediatric unconjugated hyperbilirubinemia.
J Pediatr 2013; 162;: 1092-4.
Updated: September 2019