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先天性肾上腺皮质增生症患儿的管理。

Management of Infants with Congenital Adrenal Hyperplasia.

机构信息

Department of Pediatrics, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India.

Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Indian Pediatr. 2020 Feb 15;57(2):159-164.

Abstract

Treatment of congenital adrenal hyperplasia (CAH) requires lifelong replacement of glucocorticoids with regular follow up to manage associated morbidities. The current review focuses on follow-up and management of infants diagnosed with classical CAH pertinent to Indian context. Early initiation of oral hydrocortisone in divided doses is recommended after diagnosis in newborn period, infancy and childhood. Fludrocortisone is recommended for all infants with classical CAH. All infants should be monitored as per protocol for disease and treatment related complications. The role of prenatal steroids to pregnant women with previous history of CAH affected infant for prevention of virilization of female fetus is controversial.

摘要

先天性肾上腺皮质增生症(CAH)的治疗需要终身用糖皮质激素替代,并定期随访以管理相关的并发症。本综述主要关注在印度背景下诊断为经典 CAH 的婴儿的随访和管理。建议在新生儿期、婴儿期和儿童期确诊后立即开始口服氢化可的松分剂量治疗。氟氢可的松推荐用于所有经典 CAH 婴儿。所有婴儿都应按照方案监测疾病和治疗相关并发症。对于曾生育过患有 CAH 的女性胎儿的孕妇,产前使用类固醇预防女性胎儿的男性化的作用存在争议。

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