Health-related quality of life in children with congenital adrenal hyperplasia

Health and Quality of Life Outcomes

Research

Nancy Vanderburg¹  Thang N. Tran²  Kyriakie Sarafoglou³  Jane Torkelson³  Alyssa Halper³  Maria Teresa Gonzalez-Bolanos³  Mary C. Hooke⁴ 

[1] Minnesota Department of Health, St. Paul, MN, USA;University of Minnesota College of Pharmacy, Minneapolis, MN, USA;University of Minnesota Masonic Children’s Hospital, 2450 Riverside Ave. East Bldg, Rm MB671, 55454, Minneapolis, MN, USA;University of Minnesota School of Nursing, Minneapolis, MN, USA;

关键词: Congenital adrenal hyperplasia;    Quality of life;    Pediatrics;    Glucocorticoids;    Hydrocortisone;    Fatigue;    Cortisol;    Androgen;   

DOI  :  10.1186/s12955-017-0769-7

 received in 2017-05-10, accepted in 2017-09-27,  发布年份 2017

来源: Springer

【 摘 要 】

BackgroundChildren with congenital adrenal hyperplasia (CAH) require life-long glucocorticoid replacement and have daily intermittent hyper/hypocortisolemia and hyperandrogenemia. Health-related quality of life (HRQL) is important for understanding the impact the disease and therapy have on physical, mental, emotional, and social functioning. Little is known about HRQL in CAH. We compared HRQL in children with CAH to healthy norms and examined how these scores related to physiologic variables.MethodsA cross-sectional study examined 45 patients (mean age 8.2(4.5) years). Thirty-two self-reported their quality of life (QoL) on the PedsQL™ Generic Core Scale and PedsQL™ Fatigue Scale, and 44 parents completed a parent report. Bone age Z-scores were calculated from the most recent bone age.ResultsChildren with CAH did not report lower QoL than healthy norms. However, their parents reported lower overall QoL and fatigue scores than parents of healthy norms. Children with CAH rated sleep poorer than their parents. QoL scores did not differ by sex or CAH subtype and were not associated with total daily hydrocortisone dose. Bone age Z-scores were negatively associated with child-reported emotional health and cognitive fatigue.ConclusionsParents of children with CAH reported a negative impact of disease on their children’s QoL, but their children did not. The negative associations between bone age Z-scores and emotional health and cognitive fatigue suggest an impact from chronic hypocortisolemia and hyperandrogenemia.

【 授权许可】

CC BY   
© The Author(s). 2017