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Anesth Analg 2007;104:435-436
© 2007 International Anesthesia Research Society
doi: 10.1213/01.ane.0000252462.70451.0e

GENERAL ARTICLES

The Anesthetic Implications of Crigler-Najjar Syndrome

Christopher Robards, MD, and Sorin J. Brull, MD

From the Department of Anesthesiology, Mayo Clinic College of Medicine, Mayo Clinic, Jacksonville, Flourida.

Address correspondence and reprint requests to Sorin J. Brull, MD, Mayo Clinic, JAB 4035, 4500 San Pablo Rd., Jacksonville, FL 32224, (904) 296-5688. Address e-mail to brull.sorin{at}mayo.edu.

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.






Lippincott, Williams & Wilkins Anesthesia & Analgesia® is published for the International Anesthesia Research Society® by Lippincott Williams & Wilkins with the assistance of Stanford University Libraries' HighWire Press®. Copyright 2006 by the International Anesthesia Research Society. Online ISSN: 1526-7598   Print ISSN: 0003-2999 HighWire Press
Copyright © 2007 by the International Anesthesia Research Society.