N.YU. RAYGORODSKAYA, N.V. BOLOTOVA, V.K. POLYAKOV, A.S. MARCHENKO

Saratov State Medical University named after V.I. Razumovsky, Saratov

 Contact details:

Raygorodskaya N.Yu. — MD, Professor of the Department of Propedeutics of Children’s Diseases, Children’s Endocrinology and Diabetology

Address: 112 Bolshaya Kazachya St., 410012 Saratov, Russian Federation, tel.: +7-927-120-87-81, e-mail: nraygorodskaya@gmail.com

 The purpose — to analyze the age-specific features of compensation for congenital adrenal cortex dysfunction (CACD) caused by 21-hydroxylase deficiency and identify key clinical markers for timely diagnosis of decompensation in children.

Material and methods. We performed a retrospective analysis of medical records of 19 pediatric patients (5 boys and 14 girls) with CACD observed from 2014 to 2025. The analysis focused on treatment compliance, anthropometric (height, growth rate, height SDS, bone age) and laboratory (serum levels of 17-hydroxyprogesterone and renin) parameters across three age groups (under 4, 4–10, and 11–17 y.o.). Individual growth charts were a core tool for longitudinal visualization.

Results. A marked decline in therapy compliance was observed with advancing age, falling to 60% in adolescents. This trend paralleled an increased incidence of clinical decompensation, reaching 50% in the oldest group. Accelerated linear growth and bone age advancement emerged as critical and early signs of poor disease control, frequently preceding significant rises in 17-hydroxyprogesterone. Growth charts effectively illustrated these trends, guiding timely therapeutic interventions.

Conclusion. Longitudinal monitoring via growth charts is a crucial strategy, particularly during adrenarche and puberty, to circumvent challenges posed by fluctuating hormone levels and declining compliance. This method aids in preventing premature epiphyseal closure and optimizing final height in children with CACD.

Key words: 21-hydroxylase deficiency, final adult height, treatment monitoring, CACD decompensation, hyperandrogenism

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