Living-donor liver transplantation for liver hemorrhaging due to peliosis hepatis in X-linked myotubular myopathy: Two cases and a literature review

Seiichi Shimizu, Seisuke Sakamoto, Akinari Fukuda, Yusuke Yanagi, Hajime Uchida, Masahiro Takeda, Yohei Yamada, Noriyuki Nakano, Takako Yoshioka, Mureo Kasahara

研究成果: Article査読

5 被引用数 (Scopus)

抄録

X-linked myotubular myopathy (MTM) (OMIM 310400) is a severe neuromuscular disorder caused by mutations in the myotubularin (MTM1) gene. Liver hemorrhaging due to peliosis hepatis (PH) is a fatal complication. We herein report 2 successful cases of living-donor liver transplantation (LDLT) for MTM patients due to liver hemorrhaging caused by PH and review previous reports. A boy who was 9 years and 4 months old initially underwent left lateral segmentectomy due to massive hepatic and intraperitoneal hemorrhaging. As bleeding from the remnant liver continued after hepatectomy, this patient emergently underwent LDLT using a left lateral segment graft from his father. Another boy who was 1 year and 7 months old underwent transcatheter arterial embolization due to hepatic hemorrhaging and was referred to our hospital for LDLT using a left lateral segment graft from his father. The pathological findings in both cases showed sinusoidal dilatation with degenerative changes in reticular fiber and hematoma in the explanted liver, which were consistent with PH associated with MTM. LT should be considered as a treatment option for patients with episodes of hepatic hemorrhaging due to MTM in order to protect against fatal bleeding.

本文言語English
ページ(範囲)2606-2611
ページ数6
ジャーナルAmerican Journal of Transplantation
20
9
DOI
出版ステータスPublished - 2020 9 1
外部発表はい

ASJC Scopus subject areas

  • 免疫アレルギー学
  • 移植
  • 薬理学(医学)

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