Introduction. Congenital anomalies of the kidneys and urinary tract cause chronic kidney disease in 7% of cases. The zone of the pyeloureteral segment (PUS) is affected most often. The kidney and ureter originate from two different rudiments, the violation of their connection under the influence of teratogenic or genetically determined causes determines the obstruction of the pelvic-ureteral junction.
Materials and methods. The search, analysis and systematization of publications in PubMed databases and eL ibrary.ru using the keywords «hydronephrosis», «etiology», «pathogenesis». As a result, 27 publications were selected, on the basis of which this narrative literature review was prepared.
Results and discussion. Among the many causes of pathology of the PUS, there is a violation of the recanalization of this zone, a vicious interaction of the ureteral rudiment and mesanephros, from which the pelvis is differentiated. Genetic studies provide new data for understanding pathogenesis. Pathology of angiotensin receptors, bone morphogenetic protein, TSHZ3, SHH, TBX18, calceneurin genes can lead to PUS stenosis. In the future, genetic discoveries may be the key to preventing the disease. Of all the existing mechanisms for the formation of an obstruction of the PUS, the conflict with the aberrant vessel is proven. Diagnosis, observation, and the timing of surgical treatment are left to the discretion of the specialist. Timely plastic surgery of the PUS zone prevents the formation of nephrosclerosis and chronic kidney disease. Along with the lesion of vascular structures, nephrons in the pathology of PUS, changes occur in the walls of the abdominal system. Retention of urine leads to ulceration, atrophy of the transitional epithelium, hypertrophy of muscle fibers, hyalinosis and overgrowth of connective tissue, which inhibits the contractility of the upper urinary tract and may worsen the result of surgery.
Conclusion. Currently, the mechanisms of PUS obstruction formation are not fully disclosed. Information about embryogenesis, the course of pathological processes allows the clinician to optimally approach the treatment of pathology
