Rhabdomyolysis in organic acidemia patients manifesting with metabolic decompensation

Abstract

Several metabolic disorders are related to rhabdomyolysis, but their association with methylmalonic acidemia (MMA) and propionic acidemia (PA) is unclear. Eleven patients with MMA and four patients with PA were treated and/or followed up in Kumamoto University Hospital between January 2009 and December 2018. Three patients with MMA and one patient with PA developed rhabdomyolysis at 1–2 weeks after onset of metabolic crisis. Cases 1 and 4 initially developed rhabdomyolysis after withdrawal from continuous hemodiafiltration (CHDF), and cases 2 and 3 developed rhabdomyolysis at the time of onset and had recurrent rhabdomyolysis during the recovery phase after withdrawal from CHDF. Mitochondrial dysfunction is associated with rhabdomyolysis. The rhabdomyolysis in patients with MMA and PA may have been attributed to a defect in energy production because of a secondary mitochondrial disorder. Therefore, physicians should closely follow patients with MMA and PA, especially after withdrawal of hemodialysis therapy, and provide supportive care for their mitochondrial function.