Copper metabolism after living donor liver transplantation for hepatic failure of Wilson's disease from a gene mutated donor

Citation
S. Kobayashi et al., Copper metabolism after living donor liver transplantation for hepatic failure of Wilson's disease from a gene mutated donor, HEP-GASTRO, 48(41), 2001, pp. 1259-1261
Citations number
10
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Gastroenerology and Hepatology","da verificare
Journal title
HEPATO-GASTROENTEROLOGY
ISSN journal
0172-6390 → ACNP
Volume
48
Issue
41
Year of publication
2001
Pages
1259 - 1261
Database
ISI
SICI code
0172-6390(200109/10)48:41<1259:CMALDL>2.0.ZU;2-E
Abstract
There is a genetic problem in living donor liver transplantat,ion, involvin g Wilson's disease, because the majority of donors have a kinship relations hip. Recently, it was reported that the serum ceruloplasmin level is insuff icient in some persons with one allele mutation. The recipient was a 13-year-old male child, and the donor was a 22-year-old woman, who was his sister by a different father. The gene analysis for Wil son's disease (ATP7B gene) was preoperatively carried out by the amplificat ion refractory mutation system-PCR. Homozygous and heterozygous deletion of 2871 cytosine (C) were detected in the recipient and donor, respectively, in the ATP7B gene. Serum. ceruloplas min level was sufficient in the donor. The right hepatic lobe graft was tra nsplanted to the recipient. Immediately after the liver transplantation, th e copper metabolism improved to increase the serum ceruloplasmin levels up to the normal range, and decrease the urinary copper excretion. However, th e serum ceruloplasmin levels gradually decreased below the normal base line , although the urine copper levels continued to be low without any clinical symptoms. We should perform gene analyses and confirm the serum ceruloplasmin levels in donors before living donor liver transplantation for Wilson's disease, t o screen for their impairment of copper metabolism. After living donor live r transplantation for Wilson's disease, we should carefully follow-up the t ransition of serum ceruloplasmin levels in the recipient.