ESPE Abstracts

Objective: Despite published guidelines, there is no single approach to management in congenital adrenal hyperplasia (CAH). The objective of the study was to explore the variations in treatment for CAH in Spain.

Material and methods: A retrospective study in a single center was done analyzing the data provided by patients and relatives through the Spanish Association of CAH from a online survey of 25 questions. It was diffused from December 2021 to February 2022 using the google-docs format. A descriptive statistical analysis was performed. Data from patients aged 0-18 years with classical-CAH were collected. The type of treatment, formulation, dose, administration schedule, as well as the action in case of stress/decompensation were analyzed. Patients were subdivided into 4 age groups (<2 years, 2-8 years, 8-12 years, 12-18 years). The study was approved by the hospital ethics committee.

Results: 51 valid responses were received. The mean age of the patients was 6.2 years (2 months-18 years), 53,5% women. 11 belonged to Group 1, 23 to Group 2, 7 to Group 3 and 10 to Group 4. All used Hydrocortisone (HC) (except two, with a night dose with dexamethasone) and 21% administered it in solution form. The mean dose was 12.1mg/m2/day (7-24mg/m2/day), with Group 3 having the lowest dose (13.6; 12.4; 11.4 and 13.1mg/m2/day, respectively). 67% of patients administer 3doses/day, the rest 4doses/day. The timing of HC administration was highly variable, 29 patients (55%) administered a dose past 11pm (13.4% even at dawn); 42% administered a higher dose first thing in the morning. In case of stress/decompensation, most doubled or tripled the dose, 7 of them had administered HC IM at home, most due to lack of oral tolerance. Regarding the fludrocortisone replacement, the average dose prescribed was 0.08mg/day, administered in 2 doses in 71% of the patients without changes in age but with a wide variety of schedule.

Conclusions: -This study shows a global vision of the management of CAH in our country. The doses follow the international recommendations, being lower in periods of greatest growth.

-The diversity in HC dosage draws attention, specially the distribution and schedule, a fact that would indicate the need for a more physiological dosage or to carry out personalized cortisol secretion studies.

-Regarding situations of decompensation, it would be necessary to consider whether to have home access to parenteral HC.