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

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