ホシノ　ジユンイチ   HOSHINO Jiyun'ichi   星野　純一    所属   医学部 医学科（東京女子医科大学病院）    職種   教授・基幹分野長
論文種別	原著
言語種別	英語
査読の有無	査読なし
表題	Overexpression of plasmalemmal vesicle-associated protein-1 in patient with cyanotic nephropathy: a case report.
掲載誌名	正式名：BMC nephrology 略　 称：BMC Nephrol ISSNコード：14712369/14712369
掲載区分	国外
巻・号・頁	26(1),pp.109
著者・共著者	Yusuke Ushio, So Hirata, Shun Manabe, Mayuko Suyama, Ayano Tanaka, Momoko Seki, Haruka Kato, Kana Nomura, Anna Nakai, Hitoko Sumori, Yuki Kawaguchi, Shizuka Kobayashi, Shiho Makabe, Hiroshi Kataoka, Naoko Itoh, Sekiko Taneda, Kazuho Honda, Junichi Hoshino
担当区分	最終著者,責任著者
発行年月	2025/03
概要	BACKGROUND:Cyanotic nephropathy (CN) is a known complication of cyanotic congenital heart disease (CCHD). However, many aspects of its pathophysiology remain unclear.CASE PRESENTATION:We report the case of a 29-year-old male with a history of tetralogy of Fallot. Renal biopsy revealed glomerular hypertrophy and focal segmental glomerulosclerosis. Electron microscopy revealed extensive endothelial cell damage. To investigate the etiology of endothelial cell damage, PAL-E staining was conducted, revealing staining along the glomerular capillary wall.CONCLUSION:This is the first report of PAL-E staining in CN, suggesting potential overexpression of PV-1. The association of PV-1 expression with endothelial cell damage indicates its role in the pathogenesis of CN.
DOI	10.1186/s12882-025-04046-x
PMID	40033218