PERINATOLOGIYA I PEDIATRIYA.2015.1(61):54–59;doi10.15574/PP.2015.61.54

The state of the hormonal status of children with congenital adrenal hyperplasia dysfunction due to the 21-hydroxylase deficiency

Zhelinskaya N. B., Pogadaeva N. L., Globa E. V., Shevchenko I. Yu., Begutova T. N., Horoshaya O. A., Malashonok V. B.

Ukrainsky Scientific and Practical Center of Endocrine Surgery, Transplantation of Endocrine Organs and Tissues of the Ministry of Health of Ukraine, Kiev, Ukraine

Natsional Children's Specialized Hospital «Okhmatdet», Kiev, Ukraine

Objective — to analyze the hormonal status of Ukrainian children with congenital dysfunction of adrenal cortex, who were treated with different types of glucocorticoids; identify age-sensitive approach to substitution treatment of this pathology.

Patients and methods. A database of patients in the age of 0–17 years with congenital adrenal hyperplasia due to the 21-hydroxylase deficiency is found. Patients and their parents were interviewed about the performance of the doctor's recommendations regarding compliance with dosage and frequency of dosing. The obtained results were statistically processed with the use of standard methods of statistical analysis using software for the personal computer such as: Microsoft Excel and Statistica 6.0. Also were used the methods of descriptive statistics. The check of correspondence of examined variables to the normal distribution was performed with the use of the Kolmogorov-Smirnov test. The distribution of the investigated trait was considered normal at p>0.05. Data were analyzed using parametric and nonparametric statistical methods. Frequency of presence of cases in percentage points is examined and the results are presented as average value (M) ± standard deviation (SD). The Student t-test (parametric method) was used for comparison of two samples with normal and abnormal distribution and the Mann—Whitney test (non-parametric method), respectively, the difference was considered likely at p<0.05.

Results. It is found that nearly 50% of children with congenital adrenal hyperplasia receive inadequate hormone replacement therapy, 35% of patients have a level of 17 OHP, more than (+) 3σ value exceeding regulations. The ways of optimizing of hormonal therapy of different forms of congenital adrenal hyperplasia due to the 21-hydroxylase deficiency are shown.

Conclusions. Maintaining of normal compensation congenital adrenal hyperplasia in children is not an easy task, as there is always a risk between the overdose and insufficient dose of hormones due to the liability of metabolic processes in children in the period of active growth and development. It is necessary to conduct explanatory work for parents and adolescents with congenital adrenal hyperplasia to meet the recommended doses and regimens of hormonal preparations, timely monitor the hormonal status for the compensation improvement of congenital adrenal hyperplasia and prevent the development of complications of the disease.

Key words: congenital adrenal hyperplasia, children, hormonal compensation, 17-OHP, glucocorticoids, mineralocorticoids.

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