Living‐donor liver transplantation for propionic acidaemia

Three patients with the severe form of propionic acidaemia were treated with living‐donor liver transplantation (LDLT). The procedure was successful for all patients and the incidence of metabolic decompensation was reduced dramatically even without protein restriction. Biochemically, however, the i...

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Bibliographic Details
Published in:Journal of inherited metabolic disease 2004-03, Vol.27 (2), p.205-210
Main Authors: Yorifuji, T., Kawai, M., Mamada, M., Kurokawa, K., Egawa, H., Shigematsu, Y., Kohno, Y., Tanaka, K., Nakahata, T.
Format: Article
Language:English
Subjects:
Amino Acid Metabolism, Inborn Errors - metabolism
Amino Acid Metabolism, Inborn Errors - surgery
Biological and medical sciences
Child
Child Development
Child, Preschool
Feeding. Feeding behavior
Female
Fundamental and applied biological sciences. Psychology
Genetics of eukaryotes. Biological and molecular evolution
Humans
Infant
Liver Transplantation
Living Donors
Male
Molecular and cellular biology
Postoperative Complications
Propionates - metabolism
Propionic Acidemia
Vertebrates: anatomy and physiology, studies on body, several organs or systems
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Summary:Three patients with the severe form of propionic acidaemia were treated with living‐donor liver transplantation (LDLT). The procedure was successful for all patients and the incidence of metabolic decompensation was reduced dramatically even without protein restriction. Biochemically, however, the improvement was not significant and the patients continued to excrete large amounts of propionic acid metabolites. One of the patients experienced a severe acidaemic episode 3 years after transplantation. LDLT has a beneficial effect on the care of severely affected patients since it reduces the risk of metabolic decompensation and improves the quality of life with less strict dietary control. Adequate protein restriction and medication need to be maintained even after successful transplantation.
ISSN:0141-8955
1573-2665
DOI:10.1023/B:BOLI.0000028778.54210.13