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 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.
Kawano
Youichi
Y
Department of Transplant Surgery, Jichi Medical University, Tochigi, Japan. y-kawano@jichi.ac.jp
Mizuta
Koichi
K
Hisikawa
Shuji
S
Saito
Takeshi
T
Egami
Satoshi
S
Takatsuka
Yuka
Y
Sanada
Yukihiro
Y
Fujiwara
Takehito
T
Yasuda
Yoshikazu
Y
Ohmori
Masami
M
Sakamoto
Koichi
K
Liu
Weidong
W
Nishiguchi
Shuhei
S
Hada
Toshikazu
T
Kawarasaki
Hideo
H
D002091:Butyrylcholinesterase / Q000145:classification* / Q000235:genetics*; D005260:Female; D006579:Heterozygote; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D019520:Living Donors
D002091:Butyrylcholinesterase