OBJECTIVES: Adrenal crisis (AC) is a life-threatening complication in children with congenital adrenal hyperplasia (CAH). While physiological stressors are well-recognized precipitants, the contribution of psycho-emotional stressors remains poorly understood. This study evaluated the prevalence of parent-reported adrenal crises associated with psycho-emotional stressors in children with CAH and examined associations with child age, sex, and CAH phenotype. METHODS: This cross-sectional observational study enrolled parents of children under 18 years of age with CAH living in the United States (IRB H25-0397; n=131). Parent-perceived psycho-emotional adrenal crisis (PEAC) prevalence was assessed, and associations with child age, sex, and phenotype were evaluated. Parents also reported perceived triggers, symptoms, and management strategies for suspected PEAC events. RESULTS: A total of 115 parents representing 118 children with clinically diagnosed CAH participated. Children were predominantly female (58.5 %), had a median age of 6 years, and most had salt-wasting CAH (76.1 %). Lifetime parent-reported AC prevalence was 85.5 %, with 47.0 % reporting at least one AC in the prior year. Suspected PEAC events were reported in 24.3 % of children, and 11.3 % experienced a suspected event within the previous 12 months. Overall AC rates did not differ by sex; however, recent suspected PEAC events were more frequently reported in female children. Half of parents reported proactively increasing glucocorticoid doses during psycho-emotional stress, a practice strongly associated with prior PEAC history. Most suspected PEAC episodes were managed at home, though approximately one-third required emergency medical care. CONCLUSIONS: Psycho-emotional stressors may contribute to suspected adrenal crises in children with CAH, particularly among female or older children. Further research involving both parents and clinicians is needed to validate these findings and to optimize guidance for PEAC recognition and management.
Cubberley et al. (Thu,) studied this question.