MISC

2009年9月

Liver transplantation in a patient with propionic acidemia requiring extra corporeal membrane oxygenation during severe metabolic decompensation

PEDIATRIC TRANSPLANTATION
  • Shuichi Sato
  • ,
  • Mureo Kasahara
  • ,
  • Akinari Fukuda
  • ,
  • Koichi Mizuguchi
  • ,
  • Satoshi Nakagawa
  • ,
  • Takashi Muguruma
  • ,
  • Osamu Saito
  • ,
  • Chiaki Karaki
  • ,
  • Atsuko Nakagawa
  • ,
  • Keisuke Yoshii
  • ,
  • Reiko Horikawa

13
6
開始ページ
790
終了ページ
793
記述言語
英語
掲載種別
DOI
10.1111/j.1399-3046.2008.01029.x
出版者・発行元
WILEY-BLACKWELL PUBLISHING, INC

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.

Web of Science ® 被引用回数 : 16

リンク情報
DOI
https://doi.org/10.1111/j.1399-3046.2008.01029.x
PubMed
https://www.ncbi.nlm.nih.gov/pubmed/19207227
Web of Science
https://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=JSTA_CEL&SrcApp=J_Gate_JST&DestLinkType=FullRecord&KeyUT=WOS:000268793800023&DestApp=WOS_CPL
URL
http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=68849086378&origin=inward
ID情報
  • DOI : 10.1111/j.1399-3046.2008.01029.x
  • ISSN : 1397-3142
  • PubMed ID : 19207227
  • SCOPUS ID : 68849086378
  • Web of Science ID : WOS:000268793800023

エクスポート
BibTeX RIS