Recurrence of Progressive Familial Intrahepatic Cholestasis Type 2 Phenotype After Living-donor Liver Transplantation: A Case Report

Masahata, K.; Uehara, S.; Ibuka, S.; Nakahata, K.; Hasegawa, Y.; Kondou, H.; Kubitz, R.; Ueno, T.

doi:https://doi.org/10.1016/j.transproceed.2016.02.067

Recurrence of Progressive Familial Intrahepatic Cholestasis Type 2 Phenotype After Living-donor Liver Transplantation: A Case Report

Masahata, K.; Uehara, S.; Ibuka, S. et al.

Transplantation Proceedings, 2016, 48(9), 3156-3162

アクセス数:751件（2025-07-26 12:30 集計）

固定URL: https://hdl.handle.net/11094/100009

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タイトル	Recurrence of Progressive Familial Intrahepatic Cholestasis Type 2 Phenotype After Living-donor Liver Transplantation: A Case Report
著者	NRID 1000040588381 1000040588381 著者名 Masahata, K.
	NRID 1000000448060 1000000448060 著者名 Uehara, S.
	著者名 Ibuka, S.
	NRID 1000050643532 1000050643532 researchmap permalink nakahatakengo nakahatakengo 著者名 Nakahata, K.
	NRID 1000010730369 1000010730369 著者名 Hasegawa, Y.
	NRID 1000010373515 1000010373515 著者名 Kondou, H.
	著者名 Kubitz, R.
	NRID 1000010456957 1000010456957 ORCID 0000-0002-4639-8814 0000-0002-4639-8814 researchmap permalink ueno-pedsurg ueno-pedsurg Scopus Author ID 21743992300 21743992300 著者名 Ueno, T.
内容	Masahata K., Uehara S., Ibuka S., et al. Recurrence of Progressive Familial Intrahepatic Cholestasis Type 2 Phenotype After Living-donor Liver Transplantation: A Case Report. Transplantation Proceedings 48, 3156 (2016); https://doi.org/10.1016/j.transproceed.2016.02.067.
抄録	Background: Progressive familial intrahepatic cholestasis 2 (PFIC2) is the result of mutations in the ABCB11, which encodes for bile salt export pump (BSEP). An absence of BSEP in the canalicular membrane causes cholestasis and leads to the development of end-stage liver disease in the first decade of life. Liver transplantation (LT) has been considered curative for BSEP disease. However, patients with PFIC2 having undergone LT have recently been reported to develop recurrence of cholestasis together with the clinical and histological features of primary BSEP disease. Case Report: We herein present a rare case of a patient with PFIC2 who developed post-transplantation recurrence of progressive intrahepatic cholestasis due to antibodies against BSEP after living-donor LT, which mimicked primary BSEP disease. The patient had mutations in the ABCB11 gene, resulting in the complete absence of BSEP in the native liver, explaining the lack of tolerance. Immunofluorescence staining of normal human liver sections with the patient's serum and using an anti-human immunoglobulin G antibody to detect serum antibodies showed reactivity to the BSEP epitope in the canalicular membrane. We suggest that the patients having undergone LT had been associated with a risk of autoantibody formation against the BSEP protein. The absence of primary tolerance for the BSEP epitopes may explain the formation of the anti-BSEP antibodies after LDLT.
出版者	Elsevier USA
収録物名	Transplantation Proceedings
巻(号)	48(9)
ページ	3156-3162
刊行年月	2016-11-01
言語	英語
ハンドルURL	https://hdl.handle.net/11094/100009
PISSN	00411345
eISSN	18732623
関連情報 (isVersionOf)	DOI (出版社版) https://doi.org/10.1016/j.transproceed.2016.02.067
アクセス権	open access
権利情報	© 2016. This manuscript version is made available under the CC-BY-NC-ND 4.0 license https://creativecommons.org/licenses/by-nc-nd/4.0/
出版タイプ	Accepted Manuscript
カテゴリ	学術雑誌論文 Journal Article

資源タイプ	学術雑誌論文
ローカル資源タイプ	学術雑誌論文
DCTERMS.bibliographicCitation	Transplantation Proceedings. 2016, 48(9), p. 3156-3162
DC.title	Recurrence of Progressive Familial Intrahepatic Cholestasis Type 2 Phenotype After Living-donor Liver Transplantation: A Case Report
DC.creator	Masahata, K.
	Uehara, S.
	Ibuka, S.
	Nakahata, K.
	Hasegawa, Y.
	Kondou, H.
	Kubitz, R.
	Ueno, T.
DC.publisher	Elsevier USA
DC.language' scheme='DCTERMS.RFC1766	英語
DCTERMS.issued' scheme='DCTERMS.W3CDTF	2016-11-01
DC.identifier	https://doi.org/10.1016/j.transproceed.2016.02.067
DC.description	Masahata K., Uehara S., Ibuka S., et al. Recurrence of Progressive Familial Intrahepatic Cholestasis Type 2 Phenotype After Living-donor Liver Transplantation: A Case Report. Transplantation Proceedings 48, 3156 (2016); https://doi.org/10.1016/j.transproceed.2016.02.067.
DCTERMS.abstract	Background: Progressive familial intrahepatic cholestasis 2 (PFIC2) is the result of mutations in the ABCB11, which encodes for bile salt export pump (BSEP). An absence of BSEP in the canalicular membrane causes cholestasis and leads to the development of end-stage liver disease in the first decade of life. Liver transplantation (LT) has been considered curative for BSEP disease. However, patients with PFIC2 having undergone LT have recently been reported to develop recurrence of cholestasis together with the clinical and histological features of primary BSEP disease. Case Report: We herein present a rare case of a patient with PFIC2 who developed post-transplantation recurrence of progressive intrahepatic cholestasis due to antibodies against BSEP after living-donor LT, which mimicked primary BSEP disease. The patient had mutations in the ABCB11 gene, resulting in the complete absence of BSEP in the native liver, explaining the lack of tolerance. Immunofluorescence staining of normal human liver sections with the patient's serum and using an anti-human immunoglobulin G antibody to detect serum antibodies showed reactivity to the BSEP epitope in the canalicular membrane. We suggest that the patients having undergone LT had been associated with a risk of autoantibody formation against the BSEP protein. The absence of primary tolerance for the BSEP epitopes may explain the formation of the anti-BSEP antibodies after LDLT.
DC.rights	© 2016. This manuscript version is made available under the CC-BY-NC-ND 4.0 license https://creativecommons.org/licenses/by-nc-nd/4.0/
DC.format	100009
citation_title	Recurrence of Progressive Familial Intrahepatic Cholestasis Type 2 Phenotype After Living-donor Liver Transplantation: A Case Report
citation_author	Masahata, K.
	Uehara, S.
	Ibuka, S.
	Nakahata, K.
	Hasegawa, Y.
	Kondou, H.
	Kubitz, R.
	Ueno, T.
citation_publisher	Elsevier USA
citation_language	英語
citation_date	2016-11-01
citation_journal_title	Transplantation Proceedings
citation_volume	48
citation_issue	9
citation_firstpage	3156
citation_lastpage	3162
citation_doi	https://doi.org/10.1016/j.transproceed.2016.02.067
citation_public_url	https://hdl.handle.net/11094/100009

Recurrence of Progressive Familial Intrahepatic Cholestasis Type 2 Phenotype After Living-donor Liver Transplantation: A Case Report

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