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Pediatric Adrenal Insufficiency: Challenges and Solutions

Authors :
Nisticò D
Bossini B
Benvenuto S
Pellegrin MC
Tornese G
Source :
Therapeutics and Clinical Risk Management, Vol Volume 18, Pp 47-60 (2022)
Publication Year :
2022
Publisher :
Dove Medical Press, 2022.

Abstract

Daniela Nisticò,1 Benedetta Bossini,1 Simone Benvenuto,1 Maria Chiara Pellegrin,1 Gianluca Tornese2 1University of Trieste, Trieste, Italy; 2Department of Pediatrics, Institute for Maternal and Child Health IRCCS Burlo Garofolo, Trieste, ItalyCorrespondence: Gianluca TorneseDepartment of Pediatrics, Institute for Maternal and Child Health IRCCS Burlo Garofolo, Via dell’Istria 65/1, Trieste, 34137, ItalyTel +39 040 3785470Email gianluca.tornese@burlo.trieste.itAbstract: Adrenal insufficiency is an insidious diagnosis that can be initially misdiagnosed as other life-threatening endocrine conditions, as well as sepsis, metabolic disorders, or cardiovascular disease. In newborns, cortisol deficiency causes delayed bile acid synthesis and transport maturation, determining prolonged cholestatic jaundice. Subclinical adrenal insufficiency is a particular challenge for a pediatric endocrinologist, representing the preclinical stage of acute adrenal insufficiency. Although often included in the extensive work-up of an unwell child, a single cortisol value is usually difficult to interpret; therefore, in most cases, a dynamic test is required for diagnosis to assess the hypothalamic-pituitary-adrenal axis. Stimulation tests using corticotropin analogs are recommended as first-line for diagnosis. All patients with adrenal insufficiency need long-term glucocorticoid replacement therapy, and oral hydrocortisone is the first-choice replacement treatment in pediatric. However, children that experience low cortisol concentrations and symptoms of cortisol insufficiency can take advantage using a modified release hydrocortisone formulation. The acute adrenal crisis is a life-threatening condition in all ages, treatment is effective if administered promptly, and it must not be delayed for any reason.Keywords: adrenal gland, primary adrenal insufficiency, central adrenal insufficiency, Addison disease, children, adrenal crisis, hydrocortisone

Details

Language :
English
ISSN :
1178203X
Volume :
ume 18
Database :
Directory of Open Access Journals
Journal :
Therapeutics and Clinical Risk Management
Publication Type :
Academic Journal
Accession number :
edsdoj.bb7b7cb7041c982fe16d479896b38
Document Type :
article