2015-2017 йиллар давомида РИЭИАТМда ТБУБД ташхиси билан текширилган 38та болалар ва усмирларда клиник-гормонал хусусият- лар урганилди. 38 (100%) текширилган ТБУБД ташхисли беморнинг 16 тасини (42%) угил, 22 тасини (58%) циз болалар ташкил цилади. Ка- салланиш чуцциси 1-3 ёш. Болаларнинг буйи 52-162 см оралицда. Уртача буй баландлиги кур- саткичи цизларда 118,6±4,72 см, угил болалар- да 123,32±10,6 см ни ташкил цилади. Гормонал текширувлар натижасида ТБУБД билан огриган цизларда цонда 170КСнинг 2,9-15,6 нмоль/л гача, уртача 7,97±0,66 нмоль/л ошганлиги аницланди. Угил болаларда эса 170КСнинг цондаги мицдори 5,6-21,0 нмоль/лгача, уртача 11,2±1,54 нмоль/л оралигида булди.
The aim of study was clinical and hormonal feature of patients with congenital adrenal hyperplasia (CAH), surveyed in RSSPMCE. 38 children and adolescents were with CAH, that were treated in RSSPMCE clinic from 2015 to 17 years 38 surveyed children had 16 (42%) boys and 22 (58%) girls. The ages peak of diagnosis this disease were from 1 to 3 years. The patient’s height was from 52 to 162 cm at girls were averagely 118,6±4,72 cm; boys were 123,32±10,6 cm. The results of hormonal analysis showed: serum 17-OP in girls with CAH - from 2,9 до 15,6 nmol/1 averagely 7,97±0,66 nmol/1 that reliably were higher than normal values in age groups (p<0,01). In boys 17-OP were from 5,6 to 21,0/mol/l and 11,2±1,54 nmol/1, it was higher than norm. The criteria of therapy’s adequacy were height curve and dynamics of bone age progression. Height rate decrease indicated glucocorticoid’s overdose. The target of 17-OP was upper limit of norm or slightly higher.
| № | Имя автора | Должность | Наименование организации |
|---|---|---|---|
| 1 | Ahmedova S.U. | TasPMI | |
| 2 | Shomuratova M.S. | РСНПМЦ эндокринологии им акад. Ё.Х. Туракулова |
| № | Название ссылки |
|---|---|
| 1 | 1. Андреева Е.Н. Врожденная дисфункция коры надпочечников (адроногенетальный синдром, скрининг, диагностика, лечение) Методические рекомендации. - Москва, 2010. - 35 с. 2. Дедов И.И. Эндокринология. - Москва, 2016. - 304 с. 3. Исмаилов С.И. Клиническое руководство по эндокринологии. - Ташкент 2018. - 984 с. 4. Ужегова Ж.А. Федеральные клинические рекомендации-протоколы по ведению пациентов с врожденной дисфункцией коры надпочечников в детском возрасте. - Проблемы эндокринологии. -2014,-№2.-С. 42-50. 5. Azziz R., Hincapie L.A., Knochenhauer E.S. et al. Screening for 21-hydroxylase-deficient nonclassic adrenal hyperplasia among hyperandrogenic women: a prospective study. Fertil Steril. -872:915-925. 6. Bidet M., Bellanne-Chantelot C., Galand-Portier M.B. et al. Clinical and molecular characterization of a cohort of 161 unrelated women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and 330 family members. J Clin Endocrinol Metab. - 2009. -894:1570-1578; 7. Hannah-Shmouni F, Morissette R, Sinaii N, Elman M, Prezant TR, Chen Щ Pulver A, Merke DP. Revisiting the prevalence of nonclassic congenital adrenal hyperplasia in US Ashkenazi Jews and Caucasians. Genet Med. 2017 Nov;19(ll):1276-1279. 8. Li-PingTsai, Rosenfield R.L. Serum cortisol and 17-hydroxyprogesterone concentrations in children with classic congenital adrenal hyperplasia. J Clin Endocrinol Metab.- 2015. - P.87:2993. 9. New M.I. Extensive clinical experience: nonclassical 21-hydroxylase deficiency. J Clin Endocrinol Metab. - 2008,- P.91:4205-4214; 10. Wedell, Serum cortisol and 17-hydroxyprogesterone interrelation in classic 21-hydroxylase deficiency: is currenreplacement therapy satisfactory? J Clin Endocrinol Metab. - 2011. - P. 86:4679-4685; |