Research progress of metabolic syndrome in children with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency

VernacularTitle:先天性肾上腺皮质增生症21羟化酶缺乏儿童代谢综合征研究进展
Author: Juan LIN ; Huamei MA
Publication Type:Journal Article
Keywords: Congenital adrenal hyperplasia; 21-hydroxylase deficiency; Metabolic syndrome; Cardiovascular diseases
From: Chinese Journal of Applied Clinical Pediatrics 2016;31(8):631-634
CountryChina
Language:Chinese
Abstract: Congenital adrenal hyperplasia (CAH) owing to steroid 21-hydroxylase deficiency (21-OHD) was a relatively frequent of autosomal recessive disorders characterized by the inactivation of the steroid-synthesizing enzyme in the adrenocortex.Corticosteroids (glucocorticoids and mineralocorticoid) replacement therapy was the primary treatment of 21-OHD.The main objective of 21-OHD treatment in children was to maintain normal growth.Inadequate or excessive treatment was commonly observed.A number of studies reported that 21-OHD adult were at increasing risk of developing metabolic syndrome and cardiovascular events.However,there was few researches on 21-OHD children with metabolic disorders,and no domestic reports.The article summarized recent clinical research progresses in research on the alterations of lipid and carbohydrate metabolism in children with classic 21-OHD.