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青春期21-羟化酶缺乏症用药策略

高亢 宋福英 陈晓波

中国实用儿科杂志2024,Vol.39Issue(12):916-920,5.
中国实用儿科杂志2024,Vol.39Issue(12):916-920,5.DOI:10.19538/j.ek2024120607

青春期21-羟化酶缺乏症用药策略

Drug strategy for adolescent 21 hydroxylase deficiency

高亢 1宋福英 1陈晓波1

作者信息

  • 1. 首都儿科研究所附属儿童医院内分泌科,北京 100020
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摘要

Abstract

Management of adolescents with 21-hydroxylase deficiency(21-OHD)presents unique challenges because of poor adherence to medical therapy and the increase in drug metabolismand clearance rate.Rational use of adrenocortical hormone for adolescents with 21-OHD is emphasized to avoid excessive androgen and premature closure of epiphysis caused by insufficient glucocorticoids,and to avoid drug overdose resulting in secondary Cushing syndrome.It is very important to monitor the growth and development of adolescents and biomarkers,adjust the medication in time and find the complicating diseases correctly,such as testicular adrenal rest tumours(TARTs).There is no guideline or consensus on the application of growth hormones and aromatase inhibitors,and the indications for withdrawal of gonadotropin releasing hormone analogues in adolescent children,and individualized diagnosis and treatment is required.

关键词

21-羟化酶缺乏症/基因重组人生长激素/芳香化酶抑制剂/促性腺激素释放激素类似物

Key words

21-hydroxylase deficiency/recombinant human growth hormone/aromatase inhibitors/gonadotropin releasing hormone analogues

分类

医药卫生

引用本文复制引用

高亢,宋福英,陈晓波..青春期21-羟化酶缺乏症用药策略[J].中国实用儿科杂志,2024,39(12):916-920,5.

基金项目

首都卫生发展专项(2018-2-2101) (2018-2-2101)

中国实用儿科杂志

OA北大核心CSTPCD

1005-2224

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