title: Abnormal fatty acid metabolism in spinal muscular atrophy may predispose to perioperative risks.
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.
authors.affiliation: Division of Neurology, Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
authors.forename: Zarazuela
authors.initials: Z
authors.lastname: Zolkipli
authors.forename: Mary
authors.initials: M
authors.lastname: Sherlock
authors.forename: William D
authors.initials: WD
authors.lastname: Biggar
authors.forename: Glenn
authors.initials: G
authors.lastname: Taylor
authors.forename: Jamie S
authors.initials: JS
authors.lastname: Hutchison
authors.forename: Arie
authors.initials: A
authors.lastname: Peliowski
authors.forename: Benjamin A
authors.initials: BA
authors.lastname: Alman
authors.forename: Simon C
authors.initials: SC
authors.lastname: Ling
authors.forename: Ingrid
authors.initials: I
authors.lastname: Tein
mesh_terms: D000140:Acidosis, Lactic / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D000293:Adolescent; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D022124:Hyperammonemia / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D007003:Hypoglycemia / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D008099:Liver / Q000378:metabolism* / Q000473:pathology; D017093:Liver Failure / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D008297:Male; D008928:Mitochondria / Q000378:metabolism; D013123:Spinal Fusion / Q000009:adverse effects*; D014897:Spinal Muscular Atrophies of Childhood / Q000378:metabolism / Q000473:pathology / Q000601:surgery*
chemical_list: D005227:Fatty Acids