Liver transplantation in a patient with propionic acidemia requiring extra corporeal membrane oxygenation during severe metabolic decompensation.
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.
Sato
Shuichi
S
Department of Transplant Surgery, National Center for Child Health and Development, Tokyo, Japan.
Kasahara
Mureo
M
Fukuda
Akinari
A
Mizuguchi
Koichi
K
Nakagawa
Satoshi
S
Muguruma
Takashi
T
Saito
Osamu
O
Karaki
Chiaki
C
Nakagawa
Atsuko
A
Yoshii
Keisuke
K
Horikawa
Reiko
R
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
D043586:Methylmalonyl-CoA Decarboxylase; D010100:Oxygen