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

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

The rate of urinary tract abnormalities and the functional state of kidneys in relation to the degree of connective tissue dysplasia in children

The paper is devoted to the study of the rate of urinary tract abnormalities and kidney functions in children with mild, moderate, and severe connective tissue dysplasia. Severe connective tissue dysplasia was found to prevail in children with urinary tract abnormalities and to be characterized by a variety of urodynamic urinary tract abnormalities. Urinary system infection occurred equally frequently in both patient groups and its rate did not depend on the degree of the dysplasia. Some children with severe connective dysplasia were noted to have diminished renal filtration function. High-grade vesicoureteral reflux, tubular disorders as nocturias, and lowered urine osmolarity were more common in children with severe dysplasia. Hypertension was seen equally often in both patient groups, no matter what the degree of connective tissue dysplasia

Vesicoureteral reflux and its complications in children in relation to the degree of connective tissue dysplasia

The paper deals with an association of connective tissue dysplasia with vesicoureteral reflux and its complications in children. Highgrade vesicoureteral reflux was found to be typical of children with severe connective tissue dysplasia; moreover, its repeated endoscopic correction is required in 27% of cases. The frequency of vesicoureteral reflux complications as hypertension and focal nephrosclerosis did not depend on the degree of connective tissue dysplasia. Urinary tract abnormalities were more specific to children with severe connective tissue dysplasia. The children, mainly girls, with severe connective tissue dysplasia showed a propensity to have recurrent urinary tract infection