Extraglomerular Vascular Involvement of Glomerulopathy with Fibronectin Deposits

被引：4

作者：

Hara, Masayuki ^{[1
,2
]}

Kusaba, Tetsuro ^{[1
]}

Ono, Kenshi ^{[3
]}

Masuzawa, Naoko ^{[4
,5
]}

Nakamura, Itaru ^{[1
]}

Urata, Noriko ^{[1
]}

Shiraishi, Hirokazu ^{[3
]}

Hara, Shigeo ^{[6
]}

Konishi, Eiichi ^{[4
]}

Matoba, Satoaki ^{[3
]}

Shiotsu, Yayoi ^{[1
]}

Tamagaki, Keiichi ^{[1
]}

机构：

[1] Kyoto Prefectural Univ Med, Dept Nephrol, Kyoto, Japan

[2] Omihachiman Community Med Ctr, Dept Nephrol, Omihachiman, Japan

[3] Kyoto Prefectural Univ Med, Dept Cardiovasc Med, Kyoto, Japan

[4] Kyoto Prefectural Univ Med, Dept Surg Pathol, Kyoto, Japan

[5] Otsu City Hosp, Dept Diagnost Pathol, Otsu, Shiga, Japan

[6] Gen Hosp, Kobe City Med Ctr, Dept Diagnost Pathol, Kobe, Hyogo, Japan

来源：

INTERNAL MEDICINE | 2021年 / 60卷 / 13期

关键词：

glomerulopathy with fibronectin deposits (GFND); vascular lesion; endothelial damage; hemolytic anemia; hypertension; MUTATIONS;

D O I：

10.2169/internalmedicine.6558-20

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：2103 / 2107

页数：5

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