<p>Congenital adrenal hyperplasia (CAH) is a chronic endocrine disorder requiring lifelong management that may significantly impact health-related quality of life (HRQoL) in pediatric patients. Despite growing recognition of psychosocial outcomes in CAH, data from diverse populations remain limited. This cross-sectional study evaluated HRQoL in 40 children and adolescents with CAH (aged 2–18&#xa0;years) followed at our pediatric endocrinology clinic, compared with 82 healthy controls. The Pediatric Quality of Life Inventory (PedsQL) was administered to assess physical, emotional, social, and school functioning domains. Clinical data including 17-hydroxyprogesterone (17-OHP), adrenocorticotropic hormone (ACTH), androstenedione levels, bone age, treatment regimens, and surgical history were collected. Statistical analyses included Mann–Whitney U test, chi-square test, and Spearman correlation. The mean age of patients with CAH was 11.85 ± 4.3&#xa0;years. Based on child self-reports, total HRQoL scores were significantly lower in the CAH group compared with controls (81.69 ± 11.6 vs. 87.95 ± 6.0, p = 0.007). Emotional functioning (p = 0.001), social functioning (p = 0.039), school functioning (p = 0.048), and psychosocial health scores (p = 0.002) were also significantly lower in patients, whereas physical functioning scores were comparable between groups (p = 0.117). Parent proxy-reports similarly demonstrated significantly lower total HRQoL (p = 0.001), social functioning (p &lt; 0.001), school functioning (p = 0.010), and psychosocial health scores (p = 0.001) in the CAH group. No significant differences were observed between child and parent assessments. Disease duration showed positive correlations with child-reported physical functioning (r = 0.402, p = 0.012), emotional functioning (r = 0.595, p &lt; 0.001), psychosocial health (r = 0.394, p = 0.014), and total HRQoL scores (r = 0.393, p = 0.015). No consistent associations were identified between HRQoL scores and glucocorticoid dose, BMI SDS, height SDS, biochemical control, or history of surgery. </p><p><i>Conclusions</i>: Children and adolescents with CAH experience substantial impairments in HRQoL across all functional domains, particularly in psychosocial functioning.These findings should be interpreted with caution given the relatively small sample size of this study. Nevertheless, they suggest.Nevertheless, they suggest the need for comprehensive, multidisciplinary care approaches that address not only biochemical control but also psychological and social well-being in pediatric CAH management.<Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> <p><b>What is Known:</b></p> </entry> </row> <row> <entry align="left" nameend="c2" namest="c1"> <p>• <i>Congenital adrenal hyperplasia requires lifelong glucocorticoid therapy. Its potential to impair health-related quality of life in affected children is increasingly recognized.</i></p> </entry> </row> <row> <entry align="left" nameend="c2" namest="c1"> <p><b>What is New:</b>&#xa0;</p> </entry> </row> <row> <entry align="left" nameend="c2" namest="c1"> <p>•<i> Children and adolescents with CAH had significantly lower HRQoL than healthy controls on both self- and parent proxy-reports, with impairment concentrated in the psychosocial (emotional, social, and school) domains rather than physical functioning.</i></p> </entry> </row> <row> <entry align="left" nameend="c2" namest="c1"> <p>• <i>HRQoL scores were unrelated to glucocorticoid dose, biochemical control, anthropometry, or surgical history.</i></p> </entry> </row> </tbody> </tgroup> </Table></p>

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Health-related quality of life in children and adolescents with congenital adrenal hyperplasia: a cross-sectional study using the pediatric quality of life inventory

  • Pınar Kocaay,
  • Emre Gürbüz,
  • Sirmen Kızılcan Çetin,
  • Berrak Naz Kırgıl,
  • Derya Tepe,
  • Can Alp Genç,
  • Orkun Tolunay,
  • Fatih Gürbüz

摘要

Congenital adrenal hyperplasia (CAH) is a chronic endocrine disorder requiring lifelong management that may significantly impact health-related quality of life (HRQoL) in pediatric patients. Despite growing recognition of psychosocial outcomes in CAH, data from diverse populations remain limited. This cross-sectional study evaluated HRQoL in 40 children and adolescents with CAH (aged 2–18 years) followed at our pediatric endocrinology clinic, compared with 82 healthy controls. The Pediatric Quality of Life Inventory (PedsQL) was administered to assess physical, emotional, social, and school functioning domains. Clinical data including 17-hydroxyprogesterone (17-OHP), adrenocorticotropic hormone (ACTH), androstenedione levels, bone age, treatment regimens, and surgical history were collected. Statistical analyses included Mann–Whitney U test, chi-square test, and Spearman correlation. The mean age of patients with CAH was 11.85 ± 4.3 years. Based on child self-reports, total HRQoL scores were significantly lower in the CAH group compared with controls (81.69 ± 11.6 vs. 87.95 ± 6.0, p = 0.007). Emotional functioning (p = 0.001), social functioning (p = 0.039), school functioning (p = 0.048), and psychosocial health scores (p = 0.002) were also significantly lower in patients, whereas physical functioning scores were comparable between groups (p = 0.117). Parent proxy-reports similarly demonstrated significantly lower total HRQoL (p = 0.001), social functioning (p < 0.001), school functioning (p = 0.010), and psychosocial health scores (p = 0.001) in the CAH group. No significant differences were observed between child and parent assessments. Disease duration showed positive correlations with child-reported physical functioning (r = 0.402, p = 0.012), emotional functioning (r = 0.595, p < 0.001), psychosocial health (r = 0.394, p = 0.014), and total HRQoL scores (r = 0.393, p = 0.015). No consistent associations were identified between HRQoL scores and glucocorticoid dose, BMI SDS, height SDS, biochemical control, or history of surgery.

Conclusions: Children and adolescents with CAH experience substantial impairments in HRQoL across all functional domains, particularly in psychosocial functioning.These findings should be interpreted with caution given the relatively small sample size of this study. Nevertheless, they suggest.Nevertheless, they suggest the need for comprehensive, multidisciplinary care approaches that address not only biochemical control but also psychological and social well-being in pediatric CAH management.

What is Known:

Congenital adrenal hyperplasia requires lifelong glucocorticoid therapy. Its potential to impair health-related quality of life in affected children is increasingly recognized.

What is New: 

Children and adolescents with CAH had significantly lower HRQoL than healthy controls on both self- and parent proxy-reports, with impairment concentrated in the psychosocial (emotional, social, and school) domains rather than physical functioning.

HRQoL scores were unrelated to glucocorticoid dose, biochemical control, anthropometry, or surgical history.