Pathogenesis connective tissue dysplasia (CTD) is an important pediatric issue. CTD is characterized by polymorpfism of phenotypic and visceral signs. Pathogenetic aspect connected with disbalance of collagenic and non-collagenic proteins deserve special attention when children have non-hereditary forms of CTD. The role of elastin and laminin disbalance and the genome «major proteins» that participate in tissue structures’ formation remains an understudied issue.

This paper describes a case report of renocardiac syndrome in a child with non-inherited connective tissue disease (CTD). The importance of CTD is accounted for by its high occurrence (up to 80%) and controversial interpretation of phenotypic and visceral signs. In children, combined diseases more commonly develop under the effects of similar pathogenic factors or one disease impacts the development of another one. It was demonstrated that five organ systems are affected in 20% of children with CTD and three organ systems are affected in 40% of children with CTD. The most common conditions are cardiovascular and osteoarticular disorders followed by gastrointestinal disorders. Musculoskeletal and urinary tract disorders occurred in 33% and 19.5%, respectively. Renocardiac syndrome is of particular interest from the viewpoint of comorbidity. This case report addresses renocardiac syndrome in a 6-year-old boy. Eight phenotypic and six visceral signs of CTD were identified in this child. Cardiac and renal abnormalities prevailed among internal signs. Therefore, CTD plays an important role in the development of renocardiac syndrome. KEYWORDS: connective tissue disease, renocardiac syndrome, children, heart, kidney, comorbidity. FOR CITATION: Cherkasov N.S., Davydova O.V., Derbeneva L.I., Lutsenko Yu.A. Renocardiac syndrome in a child with non-inherited connective tissue disease. Russian Journal of Woman and Child Health. 2021;4(2):184–188. DOI: 10.32364/2618-8430-2021-4-2-184-188.

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Aim: to determine clinical prognostic relevance of elastin and laminin in children with undifferentiated connective tissue disease (UCTD). Patients and Methods: 47 children with UCTD aged 3–6 were enrolled. The diagnosis was verified by ruling out genetic disorders via analyzing family tree, genetic consultation, etc. Phenotypic and visceral signs were interpreted based on "Congenital and multifactorial connective tissue diseases in children" guidelines (2016). The results of cardiovascular and urinary tract ultrasound were analyzed. Serum levels of elastin and laminin were measured by the sandwich ELISA using high-sensitive kits. All tests were performed at admission and after two years. Results: prognostic relevance (prognostic coefficient) of individual phenotypic and visceral signs and lab tests and prognostic threshold value (the sum of prognostic coefficients indicating high, moderate, or low risk of UCTD progression) were determined. It was demonstrated that measuring elastin and laminin levels improves the efficacy of UCTD progression risk assessment. This phenomenon was supported by the values of their prognostic coefficient, which were comparable to the values of significant phenotypic and visceral signs. Furthermore, the accuracy of prognosis based on the use of a set of prognostic indicators (phenotypic, visceral, and biochemical) was supported by the escalation of dysplasia signs. After two years, disease progression was reported in 36 out of 38 children with a moderate and high risk of UCTD progression as identified by the initial evaluation. Conclusion: given the relevance of elastin and laminin in dysplasia pathogenesis, these parameters are considered informative to assess the risk of dysplasia progression in children aged 3–6. It is reasonable to use elastin and laminin levels as additional criteria to evaluate the risk of UCTD progression. KEYWORDS: undifferentiated connective tissue disease, prediction, children, phenotypic signs, laminin, elastin, risk of progression, cardiovascular system, urinary system. FOR CITATION: Cherkasov N.S., Doronina T.N., Prakhov A.V., Lutsenko Yu.A. Clinical prognostic relevance of elastin and laminin in children with undifferentiated connective tissue disease. Russian Journal of Woman and Child Health. 2021;4(4):351–354 (in Russ.). DOI: 10.32364/2618-8430-2021-4-4-351-354.