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Eur J Paediatr Neurol. 2012 Sep;16(5):549-53. doi: 10.1016/j.ejpn.2012.01.004. Epub 2012 Jan 20.

Abnormal fatty acid metabolism in spinal muscular atrophy may predispose to perioperative risks.

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  • 1Division of Neurology, Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Abstract

A 15 year old boy with SMA type II underwent spinal fusion and suffered a mitochondrial Reye-like catabolic crisis 4 days postop with hypoketotic hypoglycemia, lactic acidaemia, hyperammonemia and liver failure, with 90% coagulative necrosis and diffuse macro- and microvesicular steatosis, requiring orthotopic liver transplantation. This crisis responded in part to mitochondrial therapy and anabolic rescue. He made a dramatic sustained neurological recovery, though his post-transplant liver biopsies revealed micro- and macrosteatosis. We hypothesize that a combination of surgical stress-catecholamine induced lipolysis, prolonged general anaesthesia with propofol and sevoflurane, and perioperative fasting on a background of decreased β-oxidation were potential risk factors for the mitochondrial decompensation.

Copyright © 2012 European Paediatric Neurology Society. All rights reserved.

PMID:
22264649
[PubMed - indexed for MEDLINE]
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