Connective tissue dysplasia affects the course of pyelonephritis.Purpose. The study aimed at identifying the features of chronic pyelonephritis in children with different dysplastic phenotypes.Material and methods. The study included 108 children aged 6–17 years with chronic pyelonephritis and connective tissue dysplasia. They were divided into 3 groups with Marfan-like, Ehlers-like, and unclassifiable phenotypes.Results. It was shown that the groups differed in the frequency of occurrence and types of structural disorders of urinary system, which affected the need for surgical treatment.Conclusion. Considering the dysplastic phenotype allows for a personalized approach to the patient.

BACKGROUND: There is information in the literature about the effect of connective tissue dysplasia (СTD) on the course of many somatic diseases. The role of СTD in the development of chronic pyelonephritis (СhPN) is presented differently to different authors, which is why it became necessary to conduct this study. AIM: The aim of the work is to evaluate the effect of СTD on the course and manifestations of chronic secondary pyelonephritis in children. MATERIALS AND METHODS: 142 children from 6 to 17 years old with СhPN were examined. They were divided into 2 groups: the 1st group included 108 children with manifestations of СTD, including 41 people with СTD of the 3rd degree and 67 people with СTD of the 2nd degree; group 2 34 patients without СTD. The groups were comparable in gender and age. The examination was carried out according to the standards for patients with ChPN. Signs of СTD were evaluated by the method of T.I. Kadurina. RESULTS: Obstructive and mixed variants of СhPN are typical for children with СTD. In most patients, the disease manifested at 12 years of life; at school age the first signs of the disease occurred mainly in children without СTD with dysmetabolic disorders. Relapses of СhPN in children with СTD occurred 1.5 times more often during the year and 2,1 times more often during 5 years of follow-up. Patients with СTD are characterized by a wider range of pathogens, anemia, a high incidence of structural abnormalities and urodynamic disorders, a tendency to form foci of nephrosclerosis. The need for surgical treatment in the presence of СTD increases in patients with СhPN by 4.6 times, including in repeated and radical operations. In children with grade 3 СTD, all these signs are more represented. CONCLUSIONS: СTD affects the course and manifestations of СhPN in children, creating an unfavorable background. It seems promising to study the role of СTD in the occurrence of anemia in children with СhPN.

Systemic lupus erythematosus is a chronic autoimmune disease accompanied by the synthesis of autoantibodies and the deposition of complement-fixing immune complexes in damaged tissues and organs. Systemic lupus erythematosus has many clinical manifestations, with kidney damage, the so-called lupus nephritis, being one of the most dangerous. The problem of lupus nephritis is especially relevant in pediatric practice, since it is believed that in children the disease is more severe than in adults and is often associated with infant mortality. This article presents modern ideas about the pathogenesis, clinical picture, and therapeutic tactics for lupus nephritis, as well as a clinical observation of class I lupus nephritis occurring with lupus podocytopathy.