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Living donor liver transplantation (LDLT) has been applied for the treatment of OTCD. This case series retrospectively reviewed two OTCD patients who experienced hyper\u2010NH<jats:sub>3<\/jats:sub> following LDLT. The first case was a 5\u2010year\u2010old girl who had onset of OTCD at 2\u00a0years of age. Ornithine transcarbamylase (OTC) enzyme activity was 62% for the donor and 15% for the recipient. The patient suffered from recurrence of hyper\u2010NH<jats:sub>3<\/jats:sub> within 2\u00a0months following LDLT. The second case was a 5\u2010year\u2010old girl who had onset of OTCD at 3\u00a0years of age. OTC enzyme activity was 42.6% for the donor and 9.7% for the recipient. The patient suffered hyper\u2010NH<jats:sub>3<\/jats:sub> for 12\u00a0days starting on the date of surgery. Both of the patients transiently required continuous veno\u2010venous hemodialysis; however, they are currently doing well without intensive medical treatment. The use of asymptomatic OTCD heterozygous donors in LDLT has been accepted with careful examination. However, an OTCD heterozygous carrier donor should be avoided if there is another donor candidate, due to the potentially fatal condition of hyper\u2010NH<jats:sub>3<\/jats:sub> following LDLT.<\/jats:p>","DOI":"10.1111\/petr.12848","type":"journal-article","created":{"date-parts":[[2016,11,28]],"date-time":"2016-11-28T07:16:45Z","timestamp":1480317405000},"source":"Crossref","is-referenced-by-count":20,"title":["Hyperammonemia in ornithine transcarbamylase\u2010deficient recipients following living donor liver transplantation from heterozygous carrier donors"],"prefix":"10.1111","volume":"21","author":[{"given":"Tri Hening","family":"Rahayatri","sequence":"first","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hajime","family":"Uchida","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kengo","family":"Sasaki","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takanobu","family":"Shigeta","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshihiro","family":"Hirata","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroyuki","family":"Kanazawa","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vidyadhar","family":"Mali","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akinari","family":"Fukuda","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seisuke","family":"Sakamoto","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mureo","family":"Kasahara","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo 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