Health-Related Quality of Life in Children with Congenital Adrenal Hyperplasia

Abstract

Background/Aims: Factors in congenital adrenal hyperplasia (CAH) that may affect quality of life (QOL) include the need for lifelong medication, the risk of adrenal crisis, and hyperandrogenic symptoms. The objectives were to evaluate health-related QOL (HRQOL) in children with CAH, and whether CAH poses an additional burden compared to other endocrine disorders. Methods: The validated PedsQL 4.0 generic core scales were administered to subjects (8-18 years) with CAH and hypothyroidism and their parents. The minimal clinically important difference (MCID) was determined for each scale score, allowing a comparison with the healthy population. A score of >1 standard deviation below the population mean was considered at risk for impaired HRQOL. Results: In CAH, the mean total HRQOL scores were >1 MCID below the population mean, and a higher percentage than expected had scores considered at risk. Conclusion: Compared to subjects with hypothyroidism, subjects with CAH self-reported lower school domain scores. CAH subjects more frequently reported peers not wanting to be friends.

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

“…In contrast to the two previous reports [ 3 , 4 ], our pediatric participants with CAH did not report lower overall PedsQL™ Generic scores than healthy norms. In fact, they scored significantly higher on the physical health subscale.…”

“…HRQL questionnaires are short, validated screening tools that are particularly useful when treating children with chronic conditions, such as CAH, to understand how the disease and therapy impact physical, emotional, and social functioning [ 13 ]. There are only two previous studies that examined QoL in children with CAH using validated HRQL screening tools [ 3 , 4 ]. However, these studies did not examine the relationship between QoL scores and hydrocortisone dosing or chronic androgen exposure, as measured by bone age Z-scores.…”

“…Fluctuating cortisol and adrenal androgen concentrations may impact quality of life (QoL) and may affect energy levels in children with CAH. There have only been two studies that have examined overall QoL in children with CAH [ 3 , 4 ], and none have examined fatigue. In addition, no studies have correlated fatigue and overall QoL with hydrocortisone dosing or measures of androgen exposure and disease control in children, such as bone age Z-scores.…”

Psychological adjustment, quality of life, and self-perceptions of reproductive health in males with congenital adrenal hyperplasia: a systematic review

“…In contrast, a Finnish study [ 69 ], including men and women with CAH, showed a better health-related QoL than the general Finnish population. Yau et al [ 66 ] found no differences in overall or specific health-related QoL between males and females, but again results were not presented separately. Halper et al [ 65 ] did not find any differences in QoL scores between sex and CAH subtypes.…”

“…The authors assume it might be explained by the children being on hydrocortisone, which has been found to have a less negative impact on QoL in adults than other glucocorticoids [ 67 ]. Yau et al [ 66 ] recruited 33 parents and their children with CAH, who also completed measures of QoL (PedsQL 4.0 generic core scales). Scores were compared to children with hypothyroidism.…”