The incidence of primary adrenal insufficiency is highest soon after birth, but remains consistent from 1 to 15 years of age, according to research published in The Journal of Clinical Endocrinology & Metabolism.
The study authors included participants aged 0 to 20 years in a population-based, descriptive study to examine the epidemiology and determine the causes of primary adrenal insufficiency. The team obtained diagnostic data from children born between 1996 and 2016 and calculated incidence rates in relation to person-years among individuals of the same age.
Of the 97 patients included in the cohort, the highest age-specific incidence of primary adrenal insufficiency was observed in participants aged younger than 1 year (3.4/100,000 person-years), and this value was higher among men and boys compared with girls and women (4.0/100,000 vs 2.7/100,000 person-years). At ages 1 to 15 years this incidence was significantly reduced (0.4/100,000 person-years).
Among the study sample, congenital adrenal hyperplasia (CAH) caused primary adrenal insufficiency in 57% of patients. Stratified according to age, CAH caused the disease in 88% of participants aged younger than 1 year. Autoimmune disease was the second highest cause, which occurred in 29% of patients.
The most frequent cause of primary adrenal insufficiency was CAH until age 4 years, but at age 5 years, the prevalence of autoimmune disease began to rise. After age 8 years, 95% of new cases were attributed to autoimmune disease.
The study authors highlight the significance of these etiological findings.
“It is important to recognize these differences in [primary adrenal insufficiency] etiology in different ages since the cause of [primary adrenal insufficiency] affects the diagnostic measures, treatment, and follow-up of the patient,” the researchers explain.
Study limitations include a small sample size and an ethnically homogenous cohort, which may affect the generalizability of the findings.
Borchers J, Pukkala E, Mäkitie O, Laakso S. Epidemiology and causes of primary adrenal insufficiency in children: a population-based study. J Clin Endocrinol Metab. Published online May 22, 2023. doi:10.1210/clinem/dgad283