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Liver transplantation in two cases of propionic acidaemia

✍ Scribed by J. S. Schlenzig; F. Poggi-Travert; J. Laurent; D. Rabier; D. Jan; U. Wendel; A. C. Sewell; Y. Revillon; P. Kamoun; J. M. Saudubray


Publisher
Springer
Year
1995
Tongue
English
Weight
893 KB
Volume
18
Category
Article
ISSN
0141-8955

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Propionic acidemia (PA) is a rare inherited disorder of branched chain amino acid metabolism; despite improvements in conventional medical management, the long-term outcome remains disappointing. Liver transplantation (LT) has been proposed to minimize the risk of further metabolic decompensations a