改善先天性肾上腺皮质增生症患者终身高的药物治疗进展  

The advances in medical treatment of improving the final height in patients with congenital adrenal hyperplasia

作　　者：陆飞宇 卢琳 潘慧 朱惠娟 陆召麟 LU Fei-yu;LU Lin;PAN Hui;ZHU Hui-juan;LU Zhao-lin(Department of Endocrinology,Key Laboratory of Endocrinology of National Health Commission,Translational Medicine Center of Peking Union Medical College Hospital,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Pediatric Endocrinology,The First Affiliated Hospital of Jilin University,Changchun 130021,China)

机构地区：[1]中国医学科学院北京协和医院内分泌科,卫健委内分泌重点实验室,协和转化医学中心,北京100730 [2]吉林大学第一医院小儿内分泌科,长春130021

出　　处：《临床药物治疗杂志》2019年第11期24-28,共5页Clinical Medication Journal

基　　金：国家重点研发计划罕见病临床队列研究(2016YFC0901500);中国医学科学院医学与健康科技创新工程项目(CAMS-2017-I2M-1-011)

摘　　要：先天性肾上腺皮质增生症(congenital adrenal hyperplasia,CAH)在治疗过程中会面临终身高矮的问题,原因可能为未充分抑制的雄激素促进骨骺融合,在治疗过程中出现中枢性性早熟(central precocious puberty,CPP)加速骨骺融合,糖皮质激素的过量应用,以及盐皮质激素补充不足等。研究提示,某些药物包括促性腺激素释放激素类似物(gonadotrophin releasing hormone analogue,GnRHa)、重组人生长激素(recombinant human growth hormone,rhGH)、芳香化酶抑制剂及雄激素受体拮抗剂等可以通过不同作用机制改善CAH患者的终身高。本文对上述药物改善CAH患者终身高的情况进行综述,以期为临床工作提供依据。Congenital adrenal hyperplasia(CAH)will face impaired final height during the treatment.Final height may be impaired due to insufficient inhibition of androgens which promoted epiphyseal fusion,occurrence of central precocious puberty(CPP)during the treatment which accelerated epiphyseal fusion,excessive of glucocorticoid and deficit of mineralocorticoid,etc.There are some medications that could improve final height to some extent.Gonadotrophin releasing hormone analogue(GnRHa),recombinant human growth hormone(rhGH),aromatase inhibitor,and androgen receptor antagonists could improve the final height through different mechanisms.The improvement of final height of CAH patients with the above drugs were reviewed in order to provide evidence for clinical management in such conditions.

关 键 词：先天性肾上腺皮质增生症 21-羟化酶缺陷症 终身高 药物治疗 

分 类 号：R596.1[医药卫生—内科学]