Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study
Authors' 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.
Study context
- Population
- Finnish patients aged 0–20 years with primary adrenal insufficiency (PAI), born 1996–2016, identified from the Finnish National Care Register for Health Care and patient records
- Participants
- 97 patients
- Outcome
- Incidence and causes of PAI
Methodological detailsregistry-based, national
- Registry-based
- National
Main finding
As reported in the abstract
Incidence of PAI
- Incidence in first year of life, females
- 2.7/100 000 person-years
- Incidence in first year of life, males
- 4.0/100 000 person-years
- Incidence at 1-15 years, females
- 0.3/100 000 person-years
- Incidence at 1-15 years, males
- 0.6/100 000 person-years
- Cumulative incidence at age 15 years
- 10/100 000 persons
- Cumulative incidence at age 20 years
- 13/100 000 persons
Observational data: associations, not effects.
Further findings
Causes of PAI
- CAH, all patients
- 57%
- CAH, diagnosed before age 1 year
- 88%
- Autoimmune disease
- 29%
- Adrenoleukodystrophy
- 6%
- Other genetic causes
- 6%
- From age 5 years, most new cases due to autoimmune disease
Sex distribution
- Female
- 36%
Original publication
Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study. J Clin Endocrinol Metab. Published 22 May 2023. doi:10. 1210/ clinem/ dgad283
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