Citric acid as the last therapeutic approach in an acute life-threatening metabolic decompensation of propionic acidaemia

J Pediatr Endocrinol Metab. 2013;26(5-6):569-74. doi: 10.1515/jpem-2012-0148.

Abstract

The tricarboxylic acid (TCA) cycle represents the key enzymatic steps in cellular energy metabolism. Once the TCA cycle is impaired in case of inherited metabolic disorders, life-threatening episodes of metabolic decompensation and severe organ failure can arise. We present the case of a 6 ½-year-old girl with propionic acidaemia during an episode of acute life-threatening metabolic decompensation and severe lactic acidosis. Citric acid given as an oral formulation showed the potential to sustain the TCA cycle flux. This therapeutic approach may become a treatment option in a situation of acute metabolic crisis, possibly preventing severe disturbance of energy metabolism.

Publication types

  • Case Reports

MeSH terms

  • Acute Disease
  • Anticoagulants / therapeutic use
  • Child
  • Citric Acid / therapeutic use*
  • Citric Acid Cycle / drug effects*
  • Critical Illness
  • Energy Metabolism / drug effects
  • Female
  • Humans
  • Propionic Acidemia / drug therapy*
  • Propionic Acidemia / metabolism*
  • Treatment Outcome

Substances

  • Anticoagulants
  • Citric Acid