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Liver transplantation in a patient with propionic acidemia requiring extra corporeal membrane oxygenation during severe metabolic decompensation
Author(s) -
Sato Shuichi,
Kasahara Mureo,
Fukuda Akinari,
Mizuguchi Koichi,
Nakagawa Satoshi,
Muguruma Takashi,
Saito Osamu,
Karaki Chiaki,
Nakagawa Atsuko,
Yoshii Keisuke,
Horikawa Reiko
Publication year - 2009
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2008.01029.x
Subject(s) - medicine , decompensation , ejection fraction , propionic acidemia , cardiology , liver transplantation , carnitine , metabolic disorder , surgery , heart failure , gastroenterology , transplantation
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.