Abstract: Objective To investigate the quality of life and its influencing factors in children and adolescents with congenital adrenal hyperplasia(CAH),so as to provide evidence for improving their quality of life. Methods From January 2013 to December 2018,a total of 48 children and adolescents with CAH aged 5 to 18 years in Jiangxi Provincial Children's Hospital and their parents were investigated by using the Pediatric Quality of Life Inventory Measurement Models 4.0(PedsQL4.0).Meanwhile 119 healthy children and adolescents aged 5 to 18 years and their parents were selected as control group.SPSS 22.0 software was used for statistical analysis. Results Significant differences existed between CAH children and healthy controls in social function(self-report:t=2.711,caregivers proxy-report:t=3.048),school performance(self-report: t=2.718,caregivers proxy-report: t=4.671),social psychological function(self-report: t=3.674,caregivers proxy-report: t=5.587)and total scores(self-report: t=4.026,caregivers proxy-report: t=5.342)(P<0.05).There was no significant difference in health-related quality of life between male and female children and adolescents with CAH(P>0.05).The scores of each domain of PedsQL in CAH children and adolescents with poor hormone control,complications and no regular follow-up were significantly lower than those in the normal control group(P<0.05).CAH children who were older and with longer course had lower scores in each domains. Conclusions The quality of life of children and adolescents with CAH is significantly impaired,especially in children and adolescents with poor hormone control,complications,no regular follow-up,long course of disease and old age,which needs more attention from society and family.

Key words: children and adolescents, congenital adrenal hyperplasia, PedsQL, quality of life

摘要： 目的 调查先天性肾上腺皮质增生症(CAH)儿童及青少年的生存质量及其影响因素,为提高CAH患儿的生存质量提供依据。方法 采用PedsQL4.0调查江西省儿童医院2013年1月—2018年12月间48例诊断为CAH的5～18岁儿童及青少年和家长,119名健康儿童及青少年和家长作为对照组,应用SPSS 22.0软件进行统计分析。结果 CAH患儿自评量表及家长代评量表各项得分(各单一领域得分、社交心理功能和总分)均低于健康对照组,其中社交功能(自评t=2.711,代评:t=3.048)、学校表现(自评:t=2.718,代评:t=4.671)、社交心理功能(自评:t=3.674,代评:t=5.587)、总分(自评:t=4.026,代评:t=5.342)与对照组比较,差异有统计学意义(P<0.05)。CAH患儿男女之间健康相关生活质量比较,差异无统计学意义(P>0.05);激素控制不良、有并发症、无正规随访的CAH患儿各项得分均低于对照组,且差异有统计学意义(P<0.05);年龄大和病程长的CAH患儿,各项得分更低。结论 CAH儿童及青少年的生存质量存在明显损害,特别是激素控制不良、有并发症、无正规随访、病程长、年龄大的CAH患儿损害更严重,需要社会和家庭高度关注。

关键词: 儿童青少年, 先天性肾上腺皮质增生症, PedsQL量表, 生存质量