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Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study
Joonatan Borchers1,2,3, Eero Pukkala4,5, Outi Mäkitie1,2,3,6
1Children's Hospital, Pediatric Research Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Insights
Primary adrenal insufficiency (PAI) incidence peaks in infancy, then stabilizes. Congenital adrenal hyperplasia is the most common cause in young children, while autoimmune disease dominates in older children.
Area of Science:
- Pediatric Endocrinology
- Rare Diseases
- Public Health Epidemiology
Background:
- Primary adrenal insufficiency (PAI) is a rare but serious condition affecting children.
- Comprehensive epidemiological data on PAI in pediatric populations, particularly in Nordic countries, is limited.
Purpose of the Study:
- To determine the incidence and identify the causes of primary adrenal insufficiency (PAI) in Finnish children.
- To provide a detailed epidemiological profile of PAI in a pediatric population.
Main Methods:
- A population-based descriptive study was conducted using data from the Finnish National Care Register for Health Care.
- Patient records of children (aged 0-20 years) diagnosed with adrenal insufficiency between 1996 and 2016 were analyzed.
- Incidence rates were calculated based on person-years within the Finnish population.
Main Results:
- A total of 97 children were diagnosed with PAI. The incidence was highest in the first year of life (4.0/100,000 person-years in males, 2.7/100,000 in females).
- Congenital adrenal hyperplasia accounted for 57% of cases overall and 88% of those diagnosed before age 1. Autoimmune disease was the cause in 29% of cases, becoming the leading cause after age 5.
- Cumulative incidence reached 10/100,000 by age 15 and 13/100,000 by age 20.
Conclusions:
- Following an initial peak in infancy, the incidence of PAI remains relatively consistent through childhood and adolescence.
- Approximately 1 in 10,000 children are diagnosed with PAI by age 15, with varying etiologies throughout childhood.
Context:
Incidence and causes of primary adrenal insufficiency (PAI) have not been comprehensively studied in children.
Objective:
Our objective was to describe the epidemiology and to assess causes of PAI in Finnish children.
Methods:
A population-based descriptive study of PAI in Finnish patients aged 0-20 years.Diagnoses referring to adrenal insufficiency in children born in 1996-2016 were collected from the Finnish National Care Register for Health Care. Patients with PAI were identified by studying patient records. Incidence rates were calculated in relation to person-years in the Finnish population of same age.
Results:
Of the 97 patients with PAI, 36% were female. The incidence of PAI was highest during the first year of life (in females 2.7 and in males 4.0/100 000 person-years). At 1-15 years of age, the incidence of PAI in females was 0.3/100 000 and in males 0.6/100 000 person-years. Cumulative incidence was 10/100 000 persons at age of 15 years and 13/100 000 at 20 years. Congenital adrenal hyperplasia was the cause in 57% of all patients and in 88% of patients diagnosed before age of 1 year. Other causes among the 97 patients included autoimmune disease (29%), adrenoleukodystrophy (6%), and other genetic causes (6%). From the age of 5 years, most of the new cases of PAI were due to autoimmune disease.
Conclusion:
After the first-year peak, the incidence of PAI is relatively constant through ages 1-15 years, and 1 out of 10 000 children are diagnosed with PAI before the age of 15 years.
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