OR06-05 Incidence and Risk Factors for Adrenal Crisis in Pediatric-onset Adrenal Insufficiency: A Multicenter, Prospective, Observational Study

Abstract T. Ishii: None. M. Hosokawa: None. Y. Ichihashi: None. Y. Sato: None. N. Shibata: None. K. Nagasaki: None. K. Ikegawa: None. Y. Hasegawa: None. T. Hamajima: None. F. Nagamatsu: None. S. Suzuki: None. C. Numakura: None. N. Amano: None. G. Sasaki: None. K. Nagahara: None. S. Soneda: None. D. Ariyasu: None. M. Maeda: None. H. Kamasaki: None. K. Aso: None. T. Hasegawa: None. Introduction: Adrenal crisis is a well-known life-threatening complication that occurs during follow-up of patients with adrenal insufficiency. No prospective study has thoroughly investigated adrenal crisis in children and adults with pediatric-onset primary or secondary adrenal insufficiency. Purpose: To determine the incidence and risk factors for adrenal crisis in patients with pediatric-onset adrenal insufficiency. Materials and methods: This multicenter, prospective cohort study enrolled patients diagnosed with adrenal insufficiency at ≤ 15 years of age. The incidence of adrenal crisis was calculated as events per person-year (PY), and risk factors for adrenal crisis were assessed using Poisson regression multivariable analysis. Results: The study population comprised 349 patients (164 male, 185 female) with a total follow-up of 961 PY. The median age at enrollment was 14.3 years (interquartile range [IQR] 8.5-21.2 years), and the median follow-up was 2.8 years (IQR 2.2-3.3 years). Of these patients, 213 (61%) had primary adrenal insufficiency and 136 (39%) had secondary adrenal insufficiency. Forty-one adrenal crisis events occurred in 31 patients during the study period. The calculated incidence of adrenal crisis was 4.27 per 100 PY (95% confidence interval [CI] of 3.15-5.75). Poisson regression analysis identified younger age at enrollment (relative risk [RR] 0.93 [95% CI 0.89-0.97]) and increased number of infections (RR 1.17 [95% CI 1.07-1.27]) as significant risk factors. Female sex (RR 0.99 [95% CI 0.53-1.86]), primary adrenal insufficiency (RR 0.65 [95 % CI 0.30-1.41]), or equivalent dosage of hydrocortisone per square meter of body area (RR 1.02 [95% CI 0.96-1.08]) was not a significant risk factor. Discussion and Conclusion: A substantial proportion of patients with pediatric-onset adrenal insufficiency experience adrenal crisis. Younger age and an increased number of infections are independent risk factors for developing adrenal crisis in these patients. Thus, particular attention should be devoted to younger patients and those who experience recurrent infections. Saturday, June 1, 2024