SAT303 High Frequency Of Stress Dose Adjustment And Adrenal Crisis In Adult Patients With Congenital Adrenal Hyperplasia
Authors: Tschaidse Lea , Wimmer Sophie , Auer Matthias , Lottspeich Christian , Nowotny Hanna Franziska , Dubinski Ilja , Schmidt Heinrich , Quinkler Marcus , Reisch Nicole 
Published: October 2023

Disclosure: L. Tschaidse: None. S. Wimmer: None. M. Auer: None. C. Lottspeich: None. H.F. Nowotny: None. I. Dubinski: None. H. Schmidt: None. M. Quinkler: None. N. Reisch: None.

Background: Due to impaired cortisol synthesis, patients with congenital adrenal hyperplasia (CAH) require lifelong glucocorticoid replacement therapy, including stress dose adjustment, to prevent life-threatening adrenal crises (AC). Previous studies indicate a high incidence of AC in patients with CAH but also inadequate stress dose adjustment. The aim of this study was to prospectively investigate the incidence of AC, the frequency and details of stress dose adjustment as well as knowledge of the disease in adult patients with CAH. Methods: A total of 162 adult CAH patients with CAH were enrolled in this prospective, multi-centre study. Data was collected using a patient diary with the following aspects being assessed: frequency, cause, duration and dosage of dose adjustments and the occurrence of AC. In case of AC, additional medical records were reviewed and patient interviews were conducted. Additionally, it was assessed if current sick day rules of the German Society of Endocrinology (DGE) were followed adequately. Knowledge of disease was assessed using the CAH Knowledge Assessment Questionnaire (CAHKAQ) in the German version. Results: We found an AC incidence of 8.4 per 100 patient years in 145 patients. 29 patients were subsequently excluded from further analysis due to habitual dose adjustments. Of 116 patients, 77.6% reported at least one dose adjustment with a total of195.4 dose adjustments per 100 patient years. There were no significant differences of median frequency in dose adjustments between male and female patients (2.0 (5.0) vs 3.0 (6.0), p = .829) or salt-wasting and simple-virilizing patients (2.0 (5.0) vs 3.5 (8.3), p =.111). The causes for dose adjustment were mental distress (23.8%), strenuous physical activity (14.6%), flu-like symptoms (12.7%), gastrointestinal symptoms (10.1%), medical procedures (9.1%), fever (8.6%), discomfort (6.8%), pain or trauma (6.3%), unspecific physical symptoms (5.5%) and other forms of infection (2.6%). According to the DGE recommendations, in 24.1% of cases, dose adjustment was unnecessary and in 33.9% of cases, the dosage of adjustment was incorrect. A total of 34.8% of dose adjustments were performed according to sick day rule recommendations of the DGE. There was a significant positive correlation of the frequency of dose adjustments and the incidence of AC (r =.24, p = .011) and CAHKAQ Score (r =.23, p = .014). Conclusion: Adult patients with CAH show a high incidence of AC and stress dose adjustments. Compared to the retrospective AC incidence in the same cohort, we found an even higher AC incidence in this prospective study. The majority of stress dose adjustments were not in accordance to the recommendations of the DGE. These findings suggest the need for structured and repeated education of patients and their caregivers to avoid unnecessary dose adjustments while preventing AC, with particular focus on the timing of transition.

Presentation: Saturday, June 17, 2023