title: Citric acid as the last therapeutic approach in an acute life-threatening metabolic decompensation of propionic acidaemia.
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.
authors: Department of Women and Child Health, University Hospital, University of Leipzig, Leipzig, Germany.
authors: Manuela
authors: M
authors: Siekmeyer
authors: Stefanie
authors: S
authors: Petzold-Quinque
authors: Friederike
authors: F
authors: Terpe
authors: Skadi
authors: S
authors: Beblo
authors: Rolf
authors: R
authors: Gebhardt
authors: Franziska
authors: F
authors: Schlensog-Schuster
authors: Wieland
authors: W
authors: Kiess
authors: Werner
authors: W
authors: Siekmeyer
mesh_terms: D000208:Acute Disease; D000925:Anticoagulants / Q000627:therapeutic use; D002648:Child; D019343:Citric Acid / Q000627:therapeutic use*; D002952:Citric Acid Cycle / Q000187:drug effects*; D016638:Critical Illness; D004734:Energy Metabolism / Q000187:drug effects; D005260:Female; D006801:Humans; D056693:Propionic Acidemia / Q000188:drug therapy* / Q000378:metabolism*; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D019343:Citric Acid