Extraglomerular Vascular Involvement of Glomerulopathy with Fibronectin Deposits
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- Hara Masayuki
- Department of Nephrology, Kyoto Prefectural University of Medicine, Japan Department of Nephrology, Omihachiman Community Medical Center, Japan
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- Kusaba Tetsuro
- Department of Nephrology, Kyoto Prefectural University of Medicine, Japan
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- Ono Kenshi
- Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine, Japan
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- Masuzawa Naoko
- Department of Surgical Pathology, Kyoto Prefectural University of Medicine, Japan Department of Diagnostic Pathology, Otsu City Hospital, Japan
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- Nakamura Itaru
- Department of Nephrology, Kyoto Prefectural University of Medicine, Japan
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- Urata Noriko
- Department of Nephrology, Kyoto Prefectural University of Medicine, Japan
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- Shiraishi Hirokazu
- Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine, Japan
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- Hara Shigeo
- Department of Diagnostic Pathology, Kobe City Medical Center, General Hospital, Japan
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- Konishi Eiichi
- Department of Surgical Pathology, Kyoto Prefectural University of Medicine, Japan
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- Matoba Satoaki
- Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine, Japan
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- Shiotsu Yayoi
- Department of Nephrology, Kyoto Prefectural University of Medicine, Japan
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- Tamagaki Keiichi
- Department of Nephrology, Kyoto Prefectural University of Medicine, Japan
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説明
<p>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. </p>
収録刊行物
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- Internal Medicine
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Internal Medicine 60 (13), 2103-2107, 2021-07-01
一般社団法人 日本内科学会