Successful living-donor liver transplantation from an asymptomatic carrier mother in ornithine transcarbamylase deficiency.
A liver transplantation from an asymptomatic mother, who was a carrier of ornithine transcarbamylase deficiency, to her daughter, who had severe manifestation, was successfully performed. One-year monitoring of plasma amino acid and urinary orotate/orotidine levels revealed no abnormality in the urea cycle in either subject.
Department of Pediatrics, Hokkaido University School of Medicine, Sapporo, Japan.
H
H
Nagasaka
T
T
Yorifuji
H
H
Egawa
H
H
Kikuta
K
K
Tanaka
K
K
Kobayashi
D000328:Adult; D000596:Amino Acids / Q000097:blood; D002648:Child; D005260:Female; D006579:Heterozygote; D006801:Humans; D016031:Liver Transplantation; D019520:Living Donors; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000601:surgery*; D009963:Orotic Acid / Q000652:urine; D016896:Treatment Outcome; D014529:Uridine / Q000031:analogs & derivatives* / Q000652:urine
D000596:Amino Acids; D009963:Orotic Acid; C008714:orotidine; D014529:Uridine