The purpose of the study was to evaluate the course and outcome in chronic kidney disease of congenital anomalies of the kidneys and urinary tract (CAKUT) associated with rare hereditary syndromes in children and adolescents, and to compare the results with literature data. The results of a follow-up study of the course and outcome in chronic kidney disease with syndromal congenital anomalies of the kidneys and urinary tract in rare hereditary syndromes (Pierson, Fraser 1 type, Renal hypodysplasia/ aplasia 3 type, Schuurs– Hoeómakers, CHARGE, Lowe, Renal-Coloboma, VACTERL association) and chromosomal abnormalities (Shereshevsky—Terner monosomia 45‡) are presented. In 4 out of 9 children and adolescents with congenital anomalies of the kidneys and urinary tract with rare hereditary syndromes, the formation of chronic kidney disease was established.

The article presents the history of World Kidney Day 20062023. The World Kidney Day, which started in 2006, has become an annual successful initiative of the International Society of Nephrologists, the International Federation of Kidney Foundations to inform the world community and politicians, healthcare about the importance of kidneys for general health, to raise awareness about the significance and consequences of congenital, hereditary and acquired kidney diseases, chronic kidney disease, to adopt a strategy, aimed at reducing the frequency, severity and impact of kidney diseases on health and life. The global nature of the problem of kidney disease in children and adults is evidenced by the fact that in 2011 the Political Declaration on Non-infectious diseases, approved at the summit of the World Healthcare Organization, included kidney disease under paragraph 19. Chronic kidney disease is the 6th cause of death in the world. The annual campaign of World Kidney Day 20062023 poses medical and socio-economic problems of global importance and reminds that kidney diseases affecting children and adults are common, dangerous and curable. World Kidney Day 2023, under the motto: Kidney Health for all preparedness for the unexpected, support for the vulnerable discusses the impact of disasters, in particular the COVID-19 pandemic, on the provision of specialized nephrological care to the population and its resources around the world.

The review of the literature presents the clinical phenotype and genotype pathogenesis, renal prognosis of isolated and extra-renal manifestation form of hereditary nephrotic syndrome in children. The clinical and genetic features of hereditary steroid-sensitive and steroid-resistant nephrotic syndrome in children caused by mutations of genes encoding the main components of the slit diaphragm, glomerular basement membrane, structural and functional proteins of the podocyte are highlighted. Literature data demonstrate an unfavorable renal prognosis in children with hereditary steroid-resistant nephrotic syndrome with focal segmental glomerulosclerosis and diffuse mesangial sclerosis with clinical manifestation at the age of 0–17 years with progression to terminal renal failure at the age of 0.4–18 years. Renal replacement therapy with dialysis and kidney transplantation improves the prognosis, survival, and quality of life of children with hereditary nephrotic syndrome.