Introduction: There is no unified approach in clinical practice regarding the medical management of congenital adrenal hyperplasia (CAH), despite existent international guidance. We aimed to explore geographical and temporal variations in the treatment with glucocorticoids and mineralocorticoids of patients with CAH.
Methods: We collected data recorded by 33 centres from 16 countries in the I-CAH Registry. We analysed patient visits between 1982 and 2018, exploring the type, dose and timing of glucocorticoid and mineralocorticoid replacement. We used the conversion rate: 20 mg hydrocortisone = 4 mg prednisolone = 0.25 mg dexamethasone = 25 mg cortisone acetate.
Results: 4934 patient visits from 601 patients with CAH (56% females) were analysed. Glucocorticoid replacement consisted primarily of hydrocortisone in children (88.8%) most frequently given in three daily doses (75%) and of prednisolone in adults (50.1%) usually given as one daily dose (67%). Glucocorticoid doses expressed as hydrocortisone-equivalent in mg/m2 per day (median with interquartile range) were 13.5 (10.317.8) in the 01 years, 11.9 (9.914.4) in 18 years, 13.0 (10.715.5) in 812 years, 14.0 (11.617.5) in 1218 years, 13.5 (11.119.2) in 1830 years and 12.9 (8.916.8) in the over 30 year-old patient subgroup. Glucocorticoid doses were significantly reduced after 2010 in patients 01 years (P<0.001) and 18 years (P<0.001), increased in patients 1830 years (P=0.014) and statistically similar in the other age subgroups. Mineralocorticoid replacement was used for 81.9% patients, relative doses varying across age groups, with a fludrocortisone dose (μg/m2/day, median with interquartile range) of 312 (208476) in the 01 years, 139 (94205) in 18 years, 54 (4291) in 812 years, 51 (3476) in 1218 years, 41 (3174) in 1830 years and 85 (51107) in the over 30 year-old patients. There was wide variation among different countries and centres regarding type, dose and timing of glucocorticoid and mineralocorticoid treatment.
Conclusion: Our findings suggest international variations in hormone replacement therapy, with a tendency for higher doses in younger patients. Further evidence regarding the impact of different treatment regimens on health outcomes is needed to explore the benefits of a more uniform approach in the management of CAH.
27 - 29 Nov 2019
British Society for Paediatric Endocrinology and Diabetes