The anesthetic implications of Crigler-Najjar syndrome.
Crigler-Najjar syndrome is a hereditary condition of unconjugated hyperbilirubinemia due to a deficiency of the enzyme, uridine diphosphate glucuronosyltransferase. Exacerbations of the disease can occur whenever there is either an increase in free serum bilirubin and/or a decrease in serum albumin. The exacerbations can lead to bilirubin encephalopathy and severe brain damage. The goal of anesthetic management in these patients is to prevent an imbalance in the serum bilirubin to serum albumin molar ratio, thereby avoiding neurologic sequelae.
Robards
Christopher
C
Department of Anesthesiology, Mayo Clinic College of Medicine, Mayo Clinic, Jacksonville, Flourida 32224, USA.
Brull
Sorin J
SJ
D000328:Adult; D018685:Anesthetics, Inhalation / Q000009:adverse effects; D000779:Anesthetics, Local / Q000009:adverse effects; D001663:Bilirubin / Q000097:blood; D003414:Crigler-Najjar Syndrome / Q000097:blood*; D006801:Humans; D006932:Hyperbilirubinemia / Q000097:blood / Q000139:chemically induced / Q000517:prevention & control; D008297:Male
D018685:Anesthetics, Inhalation; D000779:Anesthetics, Local; D001663:Bilirubin