期刊
EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY
卷 16, 期 5, 页码 549-553出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.ejpn.2012.01.004
关键词
Fatty acid oxidation; Liver failure; Spinal muscular atrophy; Reye-like syndrome; Mitochondria
资金
- Department of Paediatrics, Hospital for Sick Children, Toronto
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, hyper-ammonemia 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 beta-oxidation were potential risk factors for the mitochondrial decompensation. (c) 2012 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
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