TY - JOUR
T1 - A pediatric patient with classical citrullinemia who underwent living-related partial liver transplantation
AU - Ban, Kyoko
AU - Sugiyama, Naruji
AU - Sugiyama, Kohachiro
AU - Wada, Yoshiro
AU - Suzuki, Tatsuya
AU - Hashimoto, Takashi
AU - Kobayashi, Keiko
N1 - Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.
PY - 2001/5/27
Y1 - 2001/5/27
N2 - Patients with inborn errors of metabolism undergo liver transplantation, but the effect of transplanting the liver of healthy carriers of these conditions has not been fully clarified. A 6-year-old girl with classical citrullinemia, who repeatedly suffered from hyperammonemia, underwent living-related liver transplantation by using a segment of the liver of her mother, who was a heterozygote carrier for classical citrullinemia. Hyperammonemia alleviated in the patient after the transplantation, thereby dramatically improving her quality of life. Although the levels of plasma and urinary citrulline remained high postoperatively, there was no marked difference in the level of plasma citrulline up to 1 month after surgery when compared with that of previously reported orthotopic liver transplantation cases with classical citrullinemia.
AB - Patients with inborn errors of metabolism undergo liver transplantation, but the effect of transplanting the liver of healthy carriers of these conditions has not been fully clarified. A 6-year-old girl with classical citrullinemia, who repeatedly suffered from hyperammonemia, underwent living-related liver transplantation by using a segment of the liver of her mother, who was a heterozygote carrier for classical citrullinemia. Hyperammonemia alleviated in the patient after the transplantation, thereby dramatically improving her quality of life. Although the levels of plasma and urinary citrulline remained high postoperatively, there was no marked difference in the level of plasma citrulline up to 1 month after surgery when compared with that of previously reported orthotopic liver transplantation cases with classical citrullinemia.
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U2 - 10.1097/00007890-200105270-00026
DO - 10.1097/00007890-200105270-00026
M3 - Article
C2 - 11391244
AN - SCOPUS:0035958107
VL - 71
SP - 1495
EP - 1497
JO - Transplantation
JF - Transplantation
SN - 0041-1337
IS - 10
ER -