Adrenal disease

Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study

J Clin Endocrinol MetabPublished Population-based descriptive 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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