Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2025 Jan 10;17(Suppl 1):72-79.
doi: 10.4274/jcrpe.galenos.2024.2024-7-2-S. Epub 2024 Dec 23.

Central Adrenal Insufficiency: Etiology and Diagnostic Approach

Melek Yıldız  1 , Ruken Yıldırım  2 , Firdevs Baş  1
Affiliations
Review

Central Adrenal Insufficiency: Etiology and Diagnostic Approach

Melek Yıldız et al. J Clin Res Pediatr Endocrinol. .

Abstract

Central adrenal insufficiency (CAI) occurs due to a pituitary gland disorder (secondary AI) or hypothalamic dysfunction (tertiary AI). It is a potentially life-threatening condition that has many congenital and acquired causes. Adrenocorticotropic hormone deficiency may be isolated or more commonly it can be accompanied by other pituitary hormone deficiencies or midline defects. The signs and symptoms of CAI are associated with glucocorticoid deficiency. A three-step diagnostic approach including dynamic stimulation tests is recommended in the evaluation of patients with suspected CAI. Here, members of the ‘Adrenal Working Group’ of ‘The Turkish Society for Pediatric Endocrinology and Diabetes’ present an evidence-based review with good practice points and recommendations for etiology and diagnostic approach in children and adolescents with CAI.

Keywords: ACTH; Central adrenal insufficiency; children; guideline; secondary.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest: None declared

Figures

Figure 1
Figure 1
Diagnostic approach in patients with suspected central adrenal insufficiency (modified from references 15 and 19) AI: adrenal insufficiency, ITT: insulin tolerance test, ACTH: adrenocorticotropic hormone
See this image and copyright information in PMC

References

    1. Taback SP, Dean HJ. Mortality in Canadian children with growth hormone (GH) deficiency receiving GH therapy 1967-1992. The Canadian Growth Hormone Advisory Committee. J Clin Endocrinol Metab. 1996;81(5):1693–1696. doi: 10.1210/jcem.81.5.8626817. - DOI - PubMed
    1. Mills JL, Schonberger LB, Wysowski DK, Brown P, Durako SJ, Cox C, Kong F, Fradkin JE. Long-term mortality in the United States cohort of pituitary-derived growth hormone recipients. J Pediatr. 2004;144(4):430–436. doi: 10.1016/j.jpeds.2003.12.036. - DOI - PubMed
    1. Vallette-Kasic S, Brue T, Pulichino AM, Gueydan M, Barlier A, David M, Nicolino M, Malpuech G, Déchelotte P, Deal C, Van Vliet G, De Vroede M, Riepe FG, Partsch CJ, Sippell WG, Berberoglu M, Atasay B, de Zegher F, Beckers D, Kyllo J, Donohoue P, Fassnacht M, Hahner S, Allolio B, Noordam C, Dunkel L, Hero M, Pigeon B, Weill J, Yigit S, Brauner R, Heinrich JJ, Cummings E, Riddell C, Enjalbert A, Drouin J. Congenital isolated adrenocorticotropin deficiency: an underestimated cause of neonatal death, explained by TPIT gene mutations. J Clin Endocrinol Metab. 2005;90(3):1323–1331. doi: 10.1210/jc.2004-1300. - DOI - PubMed
    1. Couture C, Saveanu A, Barlier A, Carel JC, Fassnacht M, Flück CE, Houang M, Maes M, Phan-Hug F, Enjalbert A, Drouin J, Brue T, Vallette S. Phenotypic homogeneity and genotypic variability in a large series of congenital isolated ACTH-deficiency patients with TPIT gene mutations. J Clin Endocrinol Metab. 2012;97:E486–E495. - PubMed
    1. Çetinkaya S, Güran T, Kurnaz E, Keskin M, Sağsak E, Savaş Erdeve S, Suntharalingham JP, Buonocore F, Achermann JC, Aycan Z. A Patient with proopiomelanocortin deficiency: an increasingly important diagnosis to make. J Clin Res Pediatr Endocrinol. 2018;10:68–73. - PMC - PubMed

Substances

LinkOut - more resources