Epidemiology and Causes of Primary Adrenal Insufficiency in Children: A Population-Based Study.
PAI
autoimmune
congenital adrenal hyperplasia
incidence
prevalence
Journal
The Journal of clinical endocrinology and metabolism
ISSN: 1945-7197
Titre abrégé: J Clin Endocrinol Metab
Pays: United States
ID NLM: 0375362
Informations de publication
Date de publication:
18 10 2023
18 10 2023
Historique:
received:
18
02
2023
medline:
23
10
2023
pubmed:
23
5
2023
entrez:
22
5
2023
Statut:
ppublish
Résumé
Incidence and causes of primary adrenal insufficiency (PAI) have not been comprehensively studied in children. Our objective was to describe the epidemiology and to assess causes of PAI in Finnish children. 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. 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. 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.
Identifiants
pubmed: 37216903
pii: 7175449
doi: 10.1210/clinem/dgad283
pmc: PMC10583995
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
2879-2885Informations de copyright
© The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society.
Références
Endocr Rev. 2002 Jun;23(3):327-64
pubmed: 12050123
J Clin Endocrinol Metab. 2010 Sep;95(9):4133-60
pubmed: 20823466
J Clin Invest. 1992 Aug;90(2):584-95
pubmed: 1644925
J Clin Endocrinol Metab. 2002 Apr;87(4):1829-33
pubmed: 11932325
Nature. 1994 Dec 15;372(6507):635-41
pubmed: 7990953
Pediatrics. 1998 Apr;101(4 Pt 1):583-90
pubmed: 9521938
J Clin Endocrinol Metab. 2006 Aug;91(8):2843-50
pubmed: 16684821
J Clin Endocrinol Metab. 2017 Sep 1;102(9):3546-3556
pubmed: 28911151
J Clin Res Pediatr Endocrinol. 2017 Dec 30;9(Suppl 2):9-22
pubmed: 29280740
Endocrine. 2017 Dec;58(3):521-527
pubmed: 29039147
J Clin Endocrinol Metab. 2011 Jun;96(6):E925-8
pubmed: 21470994
N Engl J Med. 1996 Oct 17;335(16):1206-12
pubmed: 8815944
JCI Insight. 2016 Aug 18;1(13):
pubmed: 27588307
Lancet. 2014 Jun 21;383(9935):2152-67
pubmed: 24503135
N Engl J Med. 2020 Sep 24;383(13):1248-1261
pubmed: 32966723
Clin Endocrinol (Oxf). 2020 Jan;92(1):11-20
pubmed: 31610036
J Clin Endocrinol Metab. 2016 Jan;101(1):284-92
pubmed: 26523528
J Pediatr Endocrinol Metab. 2019 Jun 26;32(6):615-622
pubmed: 31141483
Nat Genet. 2000 Jul;25(3):298-301
pubmed: 10888877
Genet Med. 2017 Nov;19(11):1276-1279
pubmed: 28541281
J Clin Endocrinol Metab. 2005 Jun;90(6):3243-50
pubmed: 15811934
Endocr Connect. 2017 Jul;6(5):289-296
pubmed: 28446514
J Paediatr Child Health. 2004 Nov;40(11):596-9
pubmed: 15469526
J Clin Endocrinol Metab. 2016 Aug;101(8):2975-83
pubmed: 27253668
J Clin Endocrinol Metab. 2013 May;98(5):2009-18
pubmed: 23543658
Eur J Endocrinol. 2019 Mar;180(3):223-234
pubmed: 30608902
J Clin Endocrinol Metab. 2019 Jan 1;104(1):118-126
pubmed: 30252065
Eur J Hum Genet. 1999 Feb-Mar;7(2):188-96
pubmed: 10196702
J Clin Endocrinol Metab. 2012 Apr;97(4):1114-24
pubmed: 22344197
Clin Endocrinol (Oxf). 1994 Dec;41(6):757-61
pubmed: 7889611
J Clin Endocrinol Metab. 2000 Mar;85(3):1059-65
pubmed: 10720040
J Clin Endocrinol Metab. 2009 Dec;94(12):4882-90
pubmed: 19858318
Medicine (Baltimore). 1981 Sep;60(5):355-62
pubmed: 7024719
Eur J Endocrinol. 2017 Aug;177(2):187-194
pubmed: 28546232
Clin Endocrinol (Oxf). 2002 Jun;56(6):787-91
pubmed: 12072049
Postgrad Med J. 1997 May;73(859):286-8
pubmed: 9196701
Ann Neurol. 2001 Apr;49(4):512-7
pubmed: 11310629
J Clin Endocrinol Metab. 2021 Mar 8;106(3):762-773
pubmed: 33247909
Endocrinol Metab (Seoul). 2017 Dec;32(4):466-474
pubmed: 29271619
Front Endocrinol (Lausanne). 2015 Aug 05;6:113
pubmed: 26300845
J Clin Endocrinol Metab. 1999 May;84(5):1762
pubmed: 10323417
J Clin Endocrinol Metab. 1997 Oct;82(10):3293-7
pubmed: 9329356
Pediatrics. 1988 Jun;81(6):866-74
pubmed: 3259306