{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T00:14:28Z","timestamp":1790208868528,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2015,2,25]],"date-time":"2015-02-25T00:00:00Z","timestamp":1424822400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2015,5]]},"abstract":"Abstract<\/jats:title>PC<\/jats:styled-content> is produced in the liver and inhibits blood coagulation by catalyzing active factors V and VIII<\/jats:styled-content>. PC<\/jats:styled-content> deficiency causes abnormal blood clotting that is difficult to regulate by anticoagulative treatments. Four reports of PC<\/jats:styled-content> deficiency treated with LTx have been published; however, no report of DLT<\/jats:styled-content> as a therapy for PC<\/jats:styled-content> deficiency is available. We describe a case of a 23\u2010month\u2010old girl who received DLT<\/jats:styled-content> for compound heterozygous PC<\/jats:styled-content> deficiency. Her PC<\/jats:styled-content> activity was below 5%. She developed intracranial lesion and frequent refractory purpura fulminans. Both her parents had heterozygous mutations of PC<\/jats:styled-content> genes and were excluded as living donors. Furthermore, she was a low priority on the waiting list of deceased\u2010donor transplantation. We performed living DLT<\/jats:styled-content> using the liver from a patient with MSUD<\/jats:styled-content>. Activated PC<\/jats:styled-content> concentrate safely supported the perioperative period. After DLT<\/jats:styled-content>, she maintained normal PC<\/jats:styled-content> activities and BCAA levels. This is the first case of PC<\/jats:styled-content> deficiency successfully treated by living DLT<\/jats:styled-content> with MSUD<\/jats:styled-content>. We propose that DLT<\/jats:styled-content> using liver from patients with MSUD<\/jats:styled-content> is a treatment option for PC<\/jats:styled-content> deficiency.<\/jats:p>","DOI":"10.1111\/petr.12446","type":"journal-article","created":{"date-parts":[[2015,2,25]],"date-time":"2015-02-25T05:41:49Z","timestamp":1424842909000},"source":"Crossref","is-referenced-by-count":25,"title":["Successful living domino liver transplantation in a child with protein C deficiency"],"prefix":"10.1111","volume":"19","author":[{"given":"Masatoshi","family":"Matsunami","sequence":"first","affiliation":[{"name":"Transplantation Center National Center for Child Health and Development Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Ishiguro","sequence":"additional","affiliation":[{"name":"Division of Hematology National Center for Child Health and Development Tokyo 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