Research progress of benign familial hematuria
10.3760/cma.j.issn.2095-428X.2017.05.001
- VernacularTitle:良性家族性血尿研究进展
- Author:
Zihua YU
;
Zheng LI
- Keywords:
Benign familial hematuria;
Thin basement membrane nephropathy;
COL4A3 gene;
COL4A4 gene
- From:
Chinese Journal of Applied Clinical Pediatrics
2017;32(5):321-323
- CountryChina
- Language:Chinese
-
Abstract:
Benign familial hematuria,also called thin basement membrane nephropathy,is caused by a heterozygous mutation in the COL4A3 or COL4A4 gene.The prognosis of the patients with benign familial hematuria,who present isolated hematuria without associated with proteinuria and normal renal function,is good in childhood.However,the prognosis of part of the patients with benign familial hematuria,who appear proteinuria,hypertension,chronic renal failure and end-stage kidney disease,is poor in adulthood.Therefore,the patients with benign familial hematuria should be carried on the long-term follow-up,and may be reviewed every 1-2 years for hypertension,proteinuria,and renal impairment.Treatment for benign familial hematuria should include an angiotensin converting enzyme inhibitor to delay the onset of renal failure.
