Abnormal fatty acid metabolism in spinal muscular atrophy may predispose to perioperative risks Journal Articles uri icon

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  • 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.


  • Zolkipli, Zarazuela
  • Sherlock, Mary
  • Biggar, William D
  • Taylor, Glenn
  • Hutchison, Jamie S
  • Peliowski, Arie
  • Alman, Benjamin A
  • Ling, Simon C
  • Tein, Ingrid

publication date

  • September 2012