Extraglomerular Vascular Involvement of Glomerulopathy with Fibronectin Deposits.

Masayuki Hara, Tetsuro Kusaba, Kenshi Ono, Naoko Masuzawa, Itaru Nakamura, Noriko Urata, Hirokazu Shiraishi, Shigeo Hara, Eiichi Konishi, Satoaki Matoba, Yayoi Shiotsu, Keiichi Tamagaki

Internal medicine (Tokyo, Japan) 2021 Jul 01

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Glomerulopathy with fibronectin deposits (GFND) is a rare hereditary kidney disease with autosomal dominant inheritance. A 21-year-old woman who had been diagnosed with GFND 10 years ago was admitted for investigation of a rapid decline in her renal function, hemolytic anemia, and cardiac dysfunction. A renal biopsy showed GFND accompanied by extraglomerular vascular lesions. Comprehensive treatments against hypertension and anemia improved the renal function. Although there have been few reports of vascular lesions in GFND, we suspect that endothelial hyperpermeability resulting from hypertension caused the fibronectin deposition and narrowing of the extraglomerular vascular lumens, thereby accelerating hypertension and inducing hemolytic anemia.

Citation

Masayuki Hara, Tetsuro Kusaba, Kenshi Ono, Naoko Masuzawa, Itaru Nakamura, Noriko Urata, Hirokazu Shiraishi, Shigeo Hara, Eiichi Konishi, Satoaki Matoba, Yayoi Shiotsu, Keiichi Tamagaki. Extraglomerular Vascular Involvement of Glomerulopathy with Fibronectin Deposits. Internal medicine (Tokyo, Japan). 2021 Jul 01;60(13):2103-2107