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

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...

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Autores principales: Borchers, Joonatan, Pukkala, Eero, Mäkitie, Outi, Laakso, Saila
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10583995/
https://www.ncbi.nlm.nih.gov/pubmed/37216903
http://dx.doi.org/10.1210/clinem/dgad283
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author Borchers, Joonatan
Pukkala, Eero
Mäkitie, Outi
Laakso, Saila
author_facet Borchers, Joonatan
Pukkala, Eero
Mäkitie, Outi
Laakso, Saila
author_sort Borchers, Joonatan
collection PubMed
description 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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spelling pubmed-105839952023-10-19 Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study Borchers, Joonatan Pukkala, Eero Mäkitie, Outi Laakso, Saila J Clin Endocrinol Metab Clinical Research Article 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. Oxford University Press 2023-05-22 /pmc/articles/PMC10583995/ /pubmed/37216903 http://dx.doi.org/10.1210/clinem/dgad283 Text en © The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Clinical Research Article
Borchers, Joonatan
Pukkala, Eero
Mäkitie, Outi
Laakso, Saila
Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study
title Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study
title_full Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study
title_fullStr Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study
title_full_unstemmed Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study
title_short Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study
title_sort epidemiology and causes of primary adrenal insufficiency in children: a population-based study
topic Clinical Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10583995/
https://www.ncbi.nlm.nih.gov/pubmed/37216903
http://dx.doi.org/10.1210/clinem/dgad283
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