13.2 Acute Adrenal Crisis: Etiologies, Diagnosis & Emergency Stress Dosing Protocols

Acute Adrenal Crisis: Recognition, Diagnosis & Stress Dosing

Quick Answer: Treat suspected adrenal crisis immediately with parenteral hydrocortisone and resuscitation. Draw cortisol and ACTH before the first dose if sampling is immediately feasible. Volume depletion, hypoglycemia, or hyperkalemia may need simultaneous treatment. In an infant with vomiting and shock, severe hyponatremia plus hyperkalemia raises concern for salt-wasting congenital adrenal hyperplasia (CAH), even when external genitalia appear typical.

Recognize primary and central adrenal insufficiency

Cortisol supports glucose production, vascular responsiveness to catecholamines, and free water clearance. During infection, trauma, or surgery, a child with inadequate adrenal reserve may develop worsening weakness, vomiting, abdominal pain, hypoglycemia, or hypotension. Aldosterone deficiency adds renal sodium loss, volume depletion, and impaired potassium excretion. The findings vary. Treat suspected crisis when the history and presentation fit, including before hypotension becomes refractory.

Primary causes include CAH, autoimmune adrenalitis, and X-linked adrenoleukodystrophy (an $ABCD1$-related disorder). Aldosterone loss depends on the cause. Androgens can be elevated in 21-hydroxylase deficiency because steroid precursors are diverted toward androgen synthesis. Central causes include congenital or acquired pituitary disease, surgery or radiation affecting the hypothalamic–pituitary region, and suppression from exogenous glucocorticoids. Ask about current or recent oral, injected, inhaled, and topical steroids, dose and duration, recent taper or interruption, and whether the child retained the last dose. Do not use a single exposure cutoff to exclude crisis in an unstable child.

Identify the newborn with salt wasting

In salt-wasting CAH from $CYP21A2$-related 21-hydroxylase deficiency, impaired cortisol and aldosterone production can cause dehydration, weight loss, and shock in the first weeks of life. Increased androgen production can virilize the external genitalia of a 46,XX infant. A 46,XY infant can have typical-appearing genitalia, so examination alone cannot exclude CAH. Review the newborn screen and obtain diagnostic samples when feasible. An ill infant needs evaluation and treatment before screening results are available.

A 10-day-old infant who vomits after feeds and has lost substantial birth weight could initially suggest pyloric stenosis. Low sodium, high potassium, low bicarbonate, and low glucose together shift concern toward salt-wasting adrenal crisis. Prolonged vomiting from pyloric stenosis can produce hypochloremic metabolic alkalosis. Evaluate concurrent infection or other neonatal causes of shock as indicated.

Draw critical samples and plan definitive testing

Obtain point-of-care glucose and serum electrolytes promptly. When a pretreatment sample can be collected during access placement without delaying therapy, send serum cortisol and plasma ACTH. Add renin and aldosterone when primary mineralocorticoid deficiency is suspected, and 17-hydroxyprogesterone (17-OHP) when CAH is suspected. Record when each sample was drawn relative to hydrocortisone. If access or sampling takes time, give the emergency steroid and proceed with resuscitation.

Once the child is stable, endocrinology can plan cosyntropin testing when needed. A pretreatment cortisol and ACTH help localize the defect. Cosyntropin evaluates stimulated adrenal cortisol production using a protocol selected for the child's age and suspected diagnosis. Interpretation depends on the cortisol assay and collection time. The historical stimulated threshold of $18\text{ mcg/dL}$ is assay dependent, and some newer methods use lower cutoffs. Hydrocortisone is cortisol, so cortisol measured after a dose includes administered drug. Document dosing and coordinate any follow-up test with the lab and endocrinology. Never wait for a stimulation test during a crisis.

Replace cortisol and treat the immediate threats

Use parenteral hydrocortisone sodium succinate. Give IV hydrocortisone when access is available and use IM when obtaining access would delay the dose. Follow the child's written emergency plan or the pediatric protocol. The Endocrine Society primary adrenal insufficiency guideline gives a pediatric body-surface-area regimen of $50\text{ mg/m}^2$ initially, then $50–100\text{ mg/m}^2$ per 24 hours divided every six hours or as a continuous IV infusion. When the case explicitly uses this regimen for a child with BSA $0.8\text{ m}^2$, the initial dose is $50\times 0.8 = 40\text{ mg}$. The Endocrine Society CAH guideline separately gives a suggested initial age-based dose of $25\text{ mg}$ for infants and preschool children with CAH. These are separate protocols.

Septic shock and adrenal risk. A child with pneumonia, shock, hypoglycemia, and a history of months of systemic prednisone may have glucocorticoid-induced adrenal insufficiency alongside sepsis. Normal potassium is compatible with central suppression. Treat suspected adrenal crisis with stress-dose hydrocortisone while continuing antimicrobial and hemodynamic care. The 2026 pediatric Surviving Sepsis Campaign suggests against adding IV hydrocortisone when fluids and vasoactive medications restore stability. With ongoing instability and no specific adrenal indication, evidence is insufficient to recommend for or against hydrocortisone. Suspected or documented adrenal insufficiency remains an indication for stress dosing.

Transition to maintenance and prevent recurrence

After stabilization and return of enteral tolerance, coordinate the switch to oral stress dosing and then to maintenance with endocrinology. Base timing on recovery and the ongoing stressor. High stress doses of hydrocortisone provide mineralocorticoid activity. Resume or start fludrocortisone as the stress dose comes down when aldosterone deficiency requires replacement. For infants with classic CAH, the CAH guideline recommends fludrocortisone and sodium chloride supplementation in the newborn period and early infancy, adjusted with blood pressure, electrolytes, and renin. Central glucocorticoid deficiency alone generally requires no fludrocortisone.

Before discharge, teach caregivers which illnesses require additional oral glucocorticoid, what to do when vomiting prevents absorption, how to give IM hydrocortisone, and when to seek emergency help. Confirm they can demonstrate injection and have the medication, supplies, medical identification, and written plan. For a child on glucocorticoids for another condition, coordinate tapering and adrenal recovery assessment with the prescribing team.