Living donor liver transplantation from an asymptomatic mother who was a carrier for ornithine transcarbamylase deficiency

Pediatric Transplantation
T WakiyaKoichi Mizuta

Abstract

Liver transplantation (LT) has been adopted as a radical treatment for ornithine transcarbamylase deficiency (OTCD), yielding favorable outcomes. Despite the fact that it is an inheritable disease, a blood relative who is heterozygous for the disorder must sometimes be used as a liver donor for living donor LT. There is ongoing discussion regarding the use of heterozygous donors, however, to our knowledge, no cases where donation was determined based on the Ornithine transcarbamylase (OTC) activity before LT have been reported. Between May 2001 and April 2011, 17 patients were indicated for living donor LT because of OTCD at our facility. There were three cases with heterozygous donor candidate (17.6%). All heterozygous candidates underwent a liver biopsy to measure their OTC activity before LT and made efforts to secure the safety of the both donor and recipient. Two of 3 candidates had headaches sometimes, and their activity was less than 40%, and thus they were not employed as the donor. One candidate with 104.4% activity was employed, yielding favorable outcomes. Our current experience supported the effectiveness of our donation criteria, however it is necessary to collect sufficient data on a large number of patients to co...Continue Reading

References

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Mar 13, 2010·Molecular Genetics and Metabolism·Philippe M CampeauBrendan H Lee
May 19, 2011·Pediatric Transplantation·T WakiyaH Kawarasaki

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Citations

Apr 5, 2013·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·A MukhtarM El-Meteini
Oct 19, 2013·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Emily R PeritoPhilip Rosenthal
Dec 17, 2014·Digestive and Liver Disease : Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver·Astrid SchielkeOlivier Scatton
Apr 13, 2013·Journal of Hepatology·Stefano FagiuoliGiuseppe Remuzzi
Jun 8, 2012·Pediatric Transplantation·Derek A Wong
Sep 25, 2020·Journal of Pediatric Gastroenterology and Nutrition·Vikrant SoodPatrick J McKiernan

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