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 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.
Yorifuji
T
T
Department of Pediatrics, Kyoto University Hospital, 54 Shogoin Sakyo, Kyoto 606-8507, Japan. yorif@kuhp.kyoto-u.ac.jp
Kawai
M
M
Mamada
M
M
Kurokawa
K
K
Egawa
H
H
Shigematsu
Y
Y
Kohno
Y
Y
Tanaka
K
K
Nakahata
T
T
D000592:Amino Acid Metabolism, Inborn Errors / Q000378:metabolism* / Q000601:surgery*; D002648:Child; D002657:Child Development; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D011183:Postoperative Complications; D011422:Propionates / Q000378:metabolism*; D056693:Propionic Acidemia
D011422:Propionates; C029658:propionic acid