Adrenal insufficiency and adrenal crisis

The single idea that organises everything is where the axis breaks. Break it at the adrenal cortex and the child loses cortisol and aldosterone, so ACTH and renin climb and potassium rises. Break it at the pituitary or hypothalamus, or suppress it with exogenous steroid, and the child loses cortisol alone, so aldosterone, renin and potassium stay near-normal. That is why one biochemical disease presents as two different emergencies. ^[2]

This page covers the recognition and management of adrenal insufficiency and adrenal crisis in children: primary versus secondary disease, the autoimmune Addison picture, glucocorticoid-induced suppression, the salt-wasting crisis, the cortisol-ACTH-renin work-up, acute resuscitation with fluids and parenteral hydrocortisone, lifelong hydrocortisone and fludrocortisone replacement, and the stress-dose and emergency-injection plan. It links to the congenital adrenal hyperplasia and hypopituitarism leaves rather than repeating their full pathways. ^[1]

Overview & Definition

A child first seen pale, drowsy and vomiting with low blood pressure is the opening scene of adrenal insufficiency. The condition is a deficiency of adrenal glucocorticoid (cortisol), with or without mineralocorticoid (aldosterone), arising from disease of the adrenal cortex itself or from loss of the trophic drive that keeps it working. ^[2]

When the cortex fails directly the disease is primary, and both cortisol and aldosterone fall. The loss of aldosterone is what makes primary disease biochemically loud: the child wastes sodium, retains potassium, and dehydrates. ACTH rises unchecked and drives the hyperpigmentation that is the physical signature of chronic primary disease. This is Addison disease when autoimmune, and congenital adrenal hyperplasia when the cause is an enzyme block. ^[1]

When the trophic drive from the pituitary or hypothalamus fails, the disease is secondary or tertiary, and only cortisol falls. Aldosterone is controlled largely by the renin-angiotensin system rather than by ACTH, so sodium, potassium and blood pressure hold up far better. By far the commonest cause in children is suppression of the axis by exogenous glucocorticoid, given as oral, inhaled, nasal or topical steroid that is then withdrawn. ^[6]

References10ShowHide

1. [1]Bornstein SR; Allolio B; Arlt W; et al Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab, 2016.PMID 26760044
2. [2]Charmandari E; Nicolaides NC; Chrousos GP Adrenal insufficiency. Lancet, 2014.PMID 24503135
3. [3]Shulman DI; Palmert MR; Kemp SF; Lawson Wilkins Drug and Therapeutics Committee Adrenal insufficiency: still a cause of morbidity and death in childhood. Pediatrics, 2007.PMID 17242136
4. [4]Rushworth RL; Torpy DJ; Falhammar H Adrenal Crisis. N Engl J Med, 2019.PMID 31461595
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6. [6]Beuschlein F; Dekkers OM; Arlt W; et al European Society of Endocrinology and Endocrine Society Joint Clinical Guideline: Diagnosis and Therapy of Glucocorticoid-induced Adrenal Insufficiency. J Clin Endocrinol Metab, 2024.PMID 38724043
7. [7]Dong VH; Husebye ES; Tomlinson JW; et al Clinical features, investigation, and management of Addison's disease. Lancet Diabetes Endocrinol, 2026.PMID 41587556
8. [8]Chifu I; Quinkler M; Hahner S; et al Predisposing factors for adrenal crisis in chronic adrenal insufficiency: a case-control study. Eur J Endocrinol, 2023.PMID 38006230
9. [9]Hahner S; Loeffler M; Bleicken B; et al Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies. Eur J Endocrinol, 2010.PMID 19955259
10. [10]Pearce SHS; Napier CM; Mitchell AL; et al MANAGEMENT OF ENDOCRINE DISEASE: Residual adrenal function in Addison's disease. Eur J Endocrinol, 2021.PMID 33306039