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Liver transplantation for biliary atresia: a systematic review

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Abstract

Biliary atresia (BA) is an idiopathic neonatal cholangiopathy characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts. Although the Kasai operation has dramatically improved the outcomes in children with BA, most patients with BA eventually require liver transplantation (LT) even after undergoing a successful Kasai procedure. The Japanese LT Society (JLTS) was established in 1980 to characterize and follow trends in patient characteristics and the graft survival among all liver transplant patients in Japan. The 1-, 5-, 10-, 15- and 20-year survival rates for the patients and grafts undergoing living donor LT were 91.6, 91.5, 87.1, 85.4 and 84.2 and 90.5, 90.4, 84.6, 82.0 and 79.9%, respectively. LDLT was able to be performed even in patients weighing less than 5 kg with early liver failure following a Kasai operation using a reduced left lateral segments. As LT has been revealed to increase the donor pool and decrease the waiting list mortality with an excellent long-term graft survival, early referral to a transplant center should be considered when at least one complication of cirrhosis occurs during its natural history, especially in adolescents.

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Abbreviations

BA:

Biliary atresia

BMI:

Body mass index

DDLT:

Deceased donor liver transplantation

LDLT:

Living donor liver transplantation

LT:

Liver transplantation

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Acknowledgements

The authors thank all of the members of Organ Transplantation Center at the National Center for Child Health and Development, Tokyo, Japan. This work was supported in part by grants from the Scientific Research Fund of the Ministry of Education and a Research Grant for Immunology, Allergy and Organ Transplant, Rare and Intractable Disease from the Ministry of Health, Labor and Welfare, Japan (H27-1, H28-3).

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Authors and Affiliations

  1. Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan

    Mureo Kasahara, Seisuke Sakamoto & Akinari Fukuda

  2. Department of Surgery, Osaka University, Osaka, Japan

    Koji Umeshita

  3. Department of Surgery, Asahikawa University, Asahikawa, Japan

    Hiroyuki Furukawa

  4. Department of HPB and Transplantation Surgery, Kyoto University, Kyoto, Japan

    Shinji Uemoto

Authors
  1. Mureo Kasahara
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  2. Koji Umeshita
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  3. Seisuke Sakamoto
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  4. Akinari Fukuda
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  5. Hiroyuki Furukawa
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  6. Shinji Uemoto
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Contributions

MK: study design, writing of the paper, KU, SS, AF, HF, SU: study design, critical revision of the article for clinical content.

Corresponding author

Correspondence to Mureo Kasahara.

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The authors declare no conflicts of interest in association with the present study.

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Kasahara, M., Umeshita, K., Sakamoto, S. et al. Liver transplantation for biliary atresia: a systematic review. Pediatr Surg Int 33, 1289–1295 (2017). https://doi.org/10.1007/s00383-017-4173-5

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  • DOI: https://doi.org/10.1007/s00383-017-4173-5

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