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Successful pediatric living donor liver transplantation from carrier to carrier of hereditary butyrylcholinesterase variant
Authors:Kawano Youichi  Mizuta Koichi  Hisikawa Shuji  Saito Takeshi  Egami Satoshi  Takatsuka Yuka  Sanada Yukihiro  Fujiwara Takehito  Yasuda Yoshikazu  Ohmori Masami  Sakamoto Koichi  Liu Weidong  Nishiguchi Shuhei  Hada Toshikazu  Kawarasaki Hideo
Affiliation:Department of Transplant Surgery, Jichi Medical University, Tochigi, Japan. y-kawano@jichi.ac.jp
Abstract:Hypocholinesterasemia is often observed clinically, especially in various liver diseases. Not well known, however, is the fact that some patients have a hereditary BChE variant. Little has been reported on liver transplants associated with this hereditary BChE variant. Furthermore, no cases have been reported of a LDLT involving hereditary BChE variant that had been diagnosed preoperatively. A 23-month-old girl who had had a failed Kasai operation for biliary atresia underwent a liver transplant using as a graft her father's lateral segment. Preoperatively, she had been diagnosed with hypocholinesterasemia. As the donor, her father had undergone a preoperative examination, during which he was found to also have hypocholinesterasemia. DNA sequencing revealed that both had the hereditary BChE variant. The unique mutation caused a frame-shift mutation. Variant K was also detected. The patient was discharged 143 days after the operation and has had no problems with immunosuppression since. In conclusion, we report that the hereditary BChE variant is not a contraindication for either transplantation or living liver donation.
Keywords:living donor liver transplantation    pediatric liver transplantation    hereditary butyrylcholinesterase (BChE) variant    hypocholinesterasemia    DNA sequencing
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