Successful pediatric living donor liver transplantation from carrier to carrier of hereditary butyrylcholinesterase variant

:  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...

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Bibliographic Details
Published in:Pediatric transplantation 2007-09, Vol.11 (6), p.694-697
Main 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
Format: Article
Language:English
Subjects:
Biological and medical sciences
Butyrylcholinesterase - classification
Butyrylcholinesterase - genetics
DNA sequencing
Female
General aspects
hereditary butyrylcholinesterase (BChE) variant
Heterozygote
Humans
hypocholinesterasemia
Infant
Liver Transplantation
living donor liver transplantation
Living Donors
Medical sciences
pediatric liver transplantation
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
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Summary::  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.
ISSN:1397-3142
1399-3046
DOI:10.1111/j.1399-3046.2007.00750.x