Endocrine Abstracts

Congenital adrenal hyperplasia (CAH) represents one of the most prevalent forms of primary adrenal insufficiency, affecting an estimated 1 in 15,000 individuals. Suboptimal health status and care provision among adults living with CAH has been described in several countries. The CaHASE2 initiative was launched in 2023 to implement a robust, prospective, longitudinal cohort study aimed at analysing the health status of adults with CAH in the UK and Ireland. Following consensus on a minimal dataset for real-world data acquisition, participating centres commenced systematic data collection utilizing the international I-CAH registry. To date, 488 adults diagnosed with CAH have been successfully recruited, contributing data from 766 clinic visits for analysis. The current cohort predominantly comprises younger to middle-aged adults, although 130 participants are aged 51 years or older, providing valuable future opportunities to investigate health challenges that may emerge later in life. Preliminary analyses of this growing dataset show that a considerable number of recruited patients are classified as overweight or obese. Assessment of 17-hydroxyprogesterone (17OHP) concentrations suggests that a significant proportion of patients may be receiving overtreatment. Androstenedione levels are below 8 nmol/l in most patients. However, a significant fraction still exhibits suboptimal control based on hormonal biomarkers. Interestingly, preliminary findings reveal no discernible correlation between current glucocorticoid dosage and patient weight or body surface area. Presently, 23 centres are actively contributing data to the I-CAH registry, with additional sites pending approval, which is anticipated to further boost patient recruitment and data volume. Over the subsequent 12 months, the project will focus on collaborating with principal investigators to enhance the volume and completeness of longitudinal data entries. This improved data completeness will be crucial for comprehensively investigating variations in healthcare provision, health status, and outcomes, and will facilitate the provision of anonymised benchmarking data to all participating centres.