Central adrenal insufficiency in children and adolescents
Abstract
Central adrenal insufficiency (CAI) is a life-threatening condition caused by either pituitary disease (secondary adrenal insufficiency) or impaired hypothalamic function with inadequate CRH production (tertiary adrenal insufficiency). ACTH deficiency may be isolated or, more frequently, occur in conjunction with other pituitary hormone deficiencies and midline defects. Genetic mutations of the TBX19 causing isolated CAI are rare but a number of genes encoding transcription factors involved in hypothalamic-pituitary gland development, as well as other genes including POMC and PC1, are associated with ACTH deficiency. CAI is frequently identified in congenital, malformative, genetic, and epigenetic syndromes as well as in several acquired conditions of different etiologies. The signs and symptoms vary considerably and depend on the age of onset and the number and severity of associated pituitary defects. They may include hypoglycemia, lethargy, apnea, poor feeding, prolonged cholestatic jaundice, jitteriness, seizures, and sepsis in the neonate, or nonspecific signs such as fatigue, hypotension, vomiting and hyponatremia without hyperkalemia in children. The diagnosis of CAI relies on the measurement of morning cortisol concentrations along with dynamic test for cortisol release with different stimulating agents. Early recognition of CAI and its correct management are mandatory in order to avoid both morbidity and mortality in affected neonates, children and adolescents.
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Newborns with AI typically present with severe hypoglycaemia, seizures, failure to thrive, prolonged cholestatic jaundice, and in some cases coma.
“Newborns with AI typically present with severe hypoglycaemia, seizures, failure to thrive, prolonged cholestatic jaundice, and in some cases coma.”
From Hypoglycaemia in adrenal insufficiency (2023) · cites this paper as [1]
Lack of cortisol results in slow transport and maturation of bile acid synthesis, causing conjugated hyperbilirubinemia with raised liver enzymes, usually presenting at a median age of 13 days of life.
“The lack of cortisol results in slow transport and maturation of bile acid synthesis, causing conjugated hyperbilirubinemia with raised liver enzymes, which usually presents at a median age of 13 days of life (1).”
From Hypoglycaemia in adrenal insufficiency (2023) · cites this paper as [1]
In children and young adults with AI, they may have hypoglycaemia, weakness, fatigue, gastrointestinal symptoms, headaches, muscle and joint pains.
“In children and young adults with AI, they may have hypoglycaemia, weakness, fatigue, gastrointestinal symptoms, headaches, muscle and joint pains (1, 2).”
From Hypoglycaemia in adrenal insufficiency (2023) · cites this paper as [1]
Due to gastric acids and hepatic first-pass effect, the recommended glucocorticoid treatment in children with primary adrenal insufficiency is slightly higher, ranging between 6-12mg/m2/day.
“Due to gastric acids and hepatic first-pass effect, the recommended glucocorticoid treatment in children with primary AI is slightly higher, ranging between 6-12mg/m2/day (1, 49).”
From Hypoglycaemia in adrenal insufficiency (2023) · cites this paper as [1]
Basal secretion of cortisol in children and young adults is known to be between 5-6mg/m2/day.
“The basal secretion of cortisol in children and young adults is known to be between 5-6mg/m2/day (1).”
From Hypoglycaemia in adrenal insufficiency (2023) · cites this paper as [1]
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References 128 · Cited by 75
- Authors
- Patti, G., Guzzeti, C., Di Iorgi, N., Maria Allegri, A. E., Napoli, F., Loche, S., & Maghnie, M.
- Affiliations
- Istituto Giannina Gaslini (IT)
- Ospedale Microcitemico (IT)
- Corresponding author
- Maghnie, Mohamad
- Venue
- Best Practice & Research Clinical Endocrinology & Metabolism
- Publisher
- Elsevier BV
- Published
- 2018-08
- Volume
- 32
- Issue
- 4
- Pages
- 425-444
- ISSN
- 1521-690X (ISSN-L)
- OpenAlex
- W2797156057
- Cited by
- 75
- References
- 128
- Citations by year
- 2026: 3 · 2025: 8 · 2024: 16 · 2023: 11 · 2022: 11 · 2021: 11 · 2020: 8 · 2019: 7
- FWCI
- 1.00
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Best Practice & Research Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- CINECA IRIS Institutial Research Information System (University of Genoa) (submittedVersion)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
- Life Sciences › Neuroscience › Behavioral Neuroscience › Stress Responses and Cortisol
- Keywords
- secondary adrenal insufficiency, ACTH deficiency, tertiary adrenal insufficiency, pituitary disorders, hypothalamic dysfunction, TBX19, proopiomelanocortin, PC1, congenital syndromes, hypoglycemia, cortisol measurement, ACTH stimulation test, children and adolescents
- MeSH terms
- Adolescent, Adrenal Insufficiency, Adrenocorticotropic Hormone, Child, Female, Humans, Hydrocortisone, Hypothalamo-Hypophyseal System, Infant, Newborn
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