A pediatric patient with classical citrullinemia who underwent living-related partial liver transplantation

Citation
K. Ban et al., A pediatric patient with classical citrullinemia who underwent living-related partial liver transplantation, TRANSPLANT, 71(10), 2001, pp. 1495-1497
Citations number
10
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Medical Research Diagnosis & Treatment
Journal title
TRANSPLANTATION
ISSN journal
0041-1337 → ACNP
Volume
71
Issue
10
Year of publication
2001
Pages
1495 - 1497
Database
ISI
SICI code
0041-1337(20010527)71:10<1495:APPWCC>2.0.ZU;2-8
Abstract
Patients with inborn errors of metabolism undergo liver transplantation, bu t the effect of transplanting the liver of healthy carriers of these condit ions has not been fully clarified. A 6-year-old girl with classical citrull inemia, who repeatedly suffered from hyperammonemia, underwent living-relat ed liver transplantation by using a segment of the liver of her mother, who was a heterozygote carrier for classical citrullinemia. Hyperammonemia all eviated in the patient after the transplantation, thereby dramatically impr oving her quality of life. Although the levels of plasma and urinary citrul line remained high postoperatively, there was no marked difference in the l evel of plasma citrulline up to 1 month after surgery when compared with th at of previously reported orthotopic liver transplantation cases with class ical citrullinemia.