Specific features of urinary system diseases in children with connective tissue dysplasia

Rossijskij Vestnik Perinatologii i Pediatrii

T. A. Kryganova¹ 

[1] Acad. Yu.E. Veltishchev Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Ministry of Health of the Russian Federation, Moscow;

关键词: children;    connective tissue dysplasia;    congenital anomalies of the kidney and urinary tract;    alport syndrome;    ehlers-danlos syndrome;    marfan's syndrome;    matrix metalloproteinases;    transforming growth factor-p.;   

DOI  :  

来源: DOAJ

【 摘 要 】

Urinary system diseases in children with differentiated connective tissue dysplasia have not been adequately investigated and the available information is extremely scarce. The diseases represent mainly minimal change disease as orthostatic proteinuria, microscopic hematuria, metabolic disturbances, structural (nephroptosis) and vascular abnormalities (aneurysms). Undifferentiated connective tissue dysplasia is an abnormality that is being actively explored by Russian investigators. Multiple organ dysfunctions attract the attention of physicians in many specialties, including nephrologists. Urinary system diseases as recurrent urinary tract infections, renal and calicopeMc malformations, bladder diseases, and severe congenital anomalies of the kidney and urinary tract (CAKUT) are often accompanied by the manifestations of connective tissue dysplasia. A number of authors have identified connective tissue disease markers (matrix metalloproteinases, tissue inhibitors of matrix metalloproteinases, transforming growth factor-p etc.) to evaluate sclerotic processes in the kidneys. There are single studies of these markers in Alport syndrome or au-tosomal dominant polycystic kidney disease while such data on the differentiated types of dysplasia (Ehlers-Danlos syndrome, Marfan's syndrome) are unavailable.

【 授权许可】

Unknown