Two cases of Type Ⅲ collagen glomerulopathy and literature review.
10.11817/j.issn.1672-7347.2020.190130
- Author:
Fang YU
1
,
2
;
Xuejing ZHU
1
,
3
;
Shuguang YUAN
1
,
4
;
Zailiang GONG
5
;
Xiangqing XU
1
;
Hong LIU
1
;
Jun LI
1
;
Lin SUN
1
;
Fuyou LIU
1
Author Information
1. Department of Nephrology, Second Xiangya Hospital, Central South University; Institute of Nephrology, Central South University, Changsha
2. 745623147@qq.com.
3. zhuxuejing5225209@csu.edu.cn.
4. shuguangy@csu.edu.cn.
5. Department of Nephrology, Xiangya Changde Hospital, Changde Hunan 415000, China.
- Publication Type:Journal Article
- Keywords:
collagen Type Ⅲ glomerulopathy;
electron microscopy;
immunohistochemistry;
renal histopathology
- MeSH:
Collagen Type III;
genetics;
Glomerular Mesangium;
Humans;
Kidney Diseases;
Kidney Glomerulus;
Proteinuria
- From:
Journal of Central South University(Medical Sciences)
2020;45(7):869-873
- CountryChina
- Language:English
-
Abstract:
In this paper, 2 cases of collagen Type Ⅲ glomerulopathy were analyzed. The clinical manifestations mainly included nephrotic syndrome, proteinuria, hypertension and renal dysfunction. One patient showed that the complement factor H-related protein 5 (CFHR5) gene was likely a disease-causing mutation. The pathological examination of renal tissues showed hyperplasia of mesangial matrix, sub-endothelial insertion, and double-track formation. Immunohistochemistry of Type III collagen was positive. Electron microscopy revealed that massive collagen fibers (40-70 nm in diameter) deposited in the mesangial matrix and basement membrane. As for the follow-up results, the normal renal function had kept steady and the proteinuria was moderate in 1 case treated with angiotensin Ⅱ receptor blocker. Due to other system disease, another case developed into acute kidney injury and then received hemodialysis. The clinical manifestations of collagen Type Ⅲ glomerulopathy was atypical, the light microscope pathological features were various, and the disease was mainly diagnosed by electron microscopy and immunohistochemistry.
