Hospitalizations and outcomes in Congenital Adrenal Hyperplasia across the lifespan: A population-based cohort study

BACKGROUND: Congenital adrenal hyperplasia (CAH) is associated with lifelong risk of adrenal crises and increased cardiometabolic morbidity. However, population-level data on hospitalization patterns across the lifespan are limited. METHODS: In this population-based retrospective cohort study, we used nationwide Swiss hospital data (2011-2022). Hospitalizations with CAH (ICD-10-GM E25) were compared with all other hospitalizations without CAH. Outcomes included hospitalization incidence, main reasons for admission, number of diagnoses per hospitalization, and in-hospital outcomes. Analyses were stratified into neonates and infants, young children, children and adolescents, and adults. RESULTS: Among 12,569,835 hospitalizations, 2,020 involved individuals with CAH. Hospitalization incidence in CAH was highest in neonates and infants and young children, whereas in the control group it increased with age. Endocrine and metabolic disorders were the leading causes of admission across all age groups (neonates and infants: OR 66.5 [95% CI, 8.88-498.07]; young children: OR 21.42 [95% CI, 4.67-98.20]; children and adolescents: OR 53.32 [95% CI, 7.08-401.72]; adults: OR 2.61 [95% CI, 1.53-4.46]). Individuals with CAH had a substantially higher comorbidity burden across the lifespan (mean 5.5 diagnoses per hospitalization at birth versus similar burden only around age 60 in the control group). CAH was associated with an increased risk of severe in-hospital outcomes, including intensive care unit admission (OR 1.55 [95% CI, 1.20-2.01]), with the largest differences in neonates/infants. CONCLUSIONS: Across all age groups, CAH is associated with earlier and more complex hospitalizations and higher risk of severe in-hospital outcomes than in control groups, particularly in early life. These findings support the need for heightened awareness and age-specific management strategies.