Successful living domino liver transplantation in a child with protein C deficiency

Masatoshi Matsunami, Akira Ishiguro, Akinari Fukuda, Kengo Sasaki, Hajime Uchida, Takanobu Shigeta, Hiroyuki Kanazawa, Seisuke Sakamoto, Motoki Ohta, Hisaya Nakadate, Reiko Horikawa, Atsuko Nakazawa, Mika Ishige, Koichi Mizuta, Mureo Kasahara

Research output: Contribution to journalArticlepeer-review

16 Citations (Scopus)


PC is produced in the liver and inhibits blood coagulation by catalyzing active factors V and VIII. PC deficiency causes abnormal blood clotting that is difficult to regulate by anticoagulative treatments. Four reports of PC deficiency treated with LTx have been published; however, no report of DLT as a therapy for PC deficiency is available. We describe a case of a 23-month-old girl who received DLT for compound heterozygous PC deficiency. Her PC activity was below 5%. She developed intracranial lesion and frequent refractory purpura fulminans. Both her parents had heterozygous mutations of PC genes and were excluded as living donors. Furthermore, she was a low priority on the waiting list of deceased-donor transplantation. We performed living DLT using the liver from a patient with MSUD. Activated PC concentrate safely supported the perioperative period. After DLT, she maintained normal PC activities and BCAA levels. This is the first case of PC deficiency successfully treated by living DLT with MSUD. We propose that DLT using liver from patients with MSUD is a treatment option for PC deficiency.

Original languageEnglish
Pages (from-to)E70-E74
JournalPediatric Transplantation
Issue number3
Publication statusPublished - 2015 May 1
Externally publishedYes


  • domino liver transplantation
  • maple syrup urine disease
  • neonatal purpura fulminans
  • protein C concentrate
  • protein C deficiency

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation


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