Fleming Louise, Van Riper Marcia, Knafl Kathleen
J Pediatr Health Care. 2017 Sep-Oct;31(5):560-577. doi: 10.1016/j.pedhc.2017.02.004. Epub 2017 Apr 13.
Congenital adrenal hyperplasia (CAH) is an endocrine condition that requires parents to administer steroids up to three times daily, supplementing maintenance doses with oral or injectable doses during times of illness. The purpose of this integrative review was to explore the management, care, and associated health issues for children with CAH and the family response to the condition. Four literature indexes were searched, with 39 articles included. Four themes emerged: (a) Health- and Development-Related Issues, (a) Effects of Excess Androgens, (c) Life Experience of CAH, and (d) Managing and Averting Adrenal Crisis. Families having a child with CAH face complex concerns related their child's growth, the fear of adrenal crisis events, and the consequences of atypical genitalia for affected girls. Future studies should focus on interventions that provide guidelines to increase parental preparedness in managing adrenal crises and creating support systems for affected girls.
先天性肾上腺皮质增生症（CAH）是一种内分泌疾病，需要父母每天给孩子服用多达三次类固醇药物，在孩子生病期间还要通过口服或注射补充维持剂量。本综合综述的目的是探讨CAH患儿的管理、护理及相关健康问题，以及家庭对该疾病的反应。检索了四个文献索引，共纳入39篇文章。出现了四个主题：（a）与健康和发育相关的问题，（b）雄激素过多的影响，（c）CAH患儿的生活经历，以及（d）管理和避免肾上腺危象。有CAH患儿的家庭面临着与孩子成长、肾上腺危象事件的恐惧以及受影响女孩生殖器异常的后果相关的复杂问题。未来的研究应侧重于提供指导方针的干预措施，以提高父母应对肾上腺危象的准备程度，并为受影响的女孩建立支持系统。
