A multicenter study of primary liver transplantation for biliary atresia in Japan
- 120 Downloads
Kasai portoenterostomy (KP) is the primary procedure for biliary atresia (BA). However, due to reports of poor outcomes of KP, primary liver transplantation (LT) is preferred in selected cases. In Japan, primary LT is limited with no study reports details, particularly regarding the indications. We conducted the first nationwide survey to assess the status of primary LT in Japan.
Questionnaires were sent to institutions where procedures other than KP were performed as the initial procedure for BA and registered to the Japan Biliary Atresia Registry. Parameters related to procedure selection were analyzed.
Of 2895 patients registered (1989–2013), primary LT (n = 15) or exploratory laparotomy (EL) followed by LT (n = 9) without KP was performed in 24 cases (0.8%). The main reason for primary LT was late diagnosis, and for EL followed by LT, lack of fibrous tissue at the porta hepatis (ο-type). The prognoses of LT without KP was good.
Non-KP initial procedures were limited. Clear criteria regarding the timing of diagnosis or patient condition could not be determined. Reasons for not initially selecting KP varied, but late diagnosis was predominant. Further study is needed to create guidelines for the initial treatment of BA patients.
KeywordsBiliary atresia Kasai portoenterostomy Liver transplantation Primary liver transplantation Exploratory laparotomy
We would like to express our sincere gratitude to the Japan Biliary Atresia Society for supplying the registry data. In addition, we are deeply thankful for the cooperation of the institutions and the pediatric surgeons in gathering the patient data; Dr. Tatsuya Suzuki from Fujita Health University, Dr. Shigeru Ono from Jichi Medical University, Dr. Masato Shinkai from Kanagawa Children's Medical Center, Dr. Hideaki Okajima from Kyoto University, Dr. Taizo Furukawa from Kyoto Prefectural University of Medicine, Dr. Toshiharu Matsuura from Kyushu University, Dr. Mikihiro Inoue from Mie University, Dr. Akihide Tanano from Nagoya University, Dr. Satoshi Kondo from Nagoya City University, Dr. Mureo Kasahara from National Center for Child Health and Development, Dr. Kengo Nakaya from Niigata University, Takehisa Ueno from Osaka University, Dr. Hiroaki Kitagawa from St. Marianna University School of Medicine, Dr. Hiromu Tanaka from Tohoku University. We would like to thank Editage (https://www.editage.com) for English language editing.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee (Kumamoto University Hospital IRB #827) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
- 11.Japanese Biliary Atresia Society Workshop (2017) Biliary atresia national registration 2015 count result. J Jpn Soc Pediatr Surg 53:319–325 (in Japanese) Google Scholar
- 13.Eurotransplant International Foundation. Statistics Report Library. Liver transplants in 2018, by country, by donor type, by organ combination. https://statistics.eurotransplant.org/. Accessed 10 Aug 2019
- 14.Japanese Biliary Atresia Society Workshop (2016) Biliary atresia national registration 2014 count result. J Jpn Soc Pediatr Surg 54:307–313 (in Japanese) Google Scholar
- 15.Japanese Biliary Atresia Society Workshop (2000) Biliary atresia national registration 1998 count result. J Jpn Soc Pediatr Surg 36:348–353 (in Japanese) Google Scholar
- 16.Japanese Biliary Atresia Society Workshop (2015) Biliary atresia national registration 2013 count result. J Jpn Soc Pediatr Surg 51:268–274 (in Japanese) Google Scholar