title: Liver transplantation in a patient with propionic acidemia requiring extra corporeal membrane oxygenation during severe metabolic decompensation.
abstract: LDLT is an effective treatment modality in patients with congenial metabolic liver disease. PA is a rare autosomal recessive disorder caused by deficiency in propionyl-CoA carboxylase. The present study demonstrates a two-yr-old girl with PA who was admitted for metabolic decompensation and immediately treated with CHD and protein intake restriction at 46 days of age. Two yr later, the patient was readmitted for severe metabolic decompensation with complete atrioventricular block and ventricular fibrillation. CHDF and ECMO were indicated because of progressive metabolic and cardiac deterioration. After full recovery of the ejection fraction, planned LDLT was performed to prevent further metabolic decompensation and fatal cardiac insufficiency. No significant events occurred after the operation and the condition of the patient is stable with continued protein restriction and carnitine supplementation.
authors.affiliation: Department of Transplant Surgery, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Shuichi
authors.initials: S
authors.lastname: Sato
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
authors.forename: Akinari
authors.initials: A
authors.lastname: Fukuda
authors.forename: Koichi
authors.initials: K
authors.lastname: Mizuguchi
authors.forename: Satoshi
authors.initials: S
authors.lastname: Nakagawa
authors.forename: Takashi
authors.initials: T
authors.lastname: Muguruma
authors.forename: Osamu
authors.initials: O
authors.lastname: Saito
authors.forename: Chiaki
authors.initials: C
authors.lastname: Karaki
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakagawa
authors.forename: Keisuke
authors.initials: K
authors.lastname: Yoshii
authors.forename: Reiko
authors.initials: R
authors.lastname: Horikawa
mesh_terms: D054537:Atrioventricular Block / Q000628:therapy; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D043586:Methylmalonyl-CoA Decarboxylase / Q000235:genetics / Q000378:metabolism; D010100:Oxygen / Q000737:chemistry / Q000378:metabolism*; D056693:Propionic Acidemia / Q000628:therapy*; D014693:Ventricular Fibrillation / Q000628:therapy
chemical_list: D043586:Methylmalonyl-CoA Decarboxylase; D010100:Oxygen