What is the management of suspected adrenal gland issues in children?

Management of Suspected Adrenal Gland Issues in Children

Children with suspected adrenal insufficiency require immediate treatment with intravenous or intramuscular hydrocortisone 100 mg (or 50 mg/m² body surface area) without waiting for diagnostic confirmation, as treatment delay can be fatal. 1

Immediate Recognition and Emergency Management

Clinical Presentation Requiring Urgent Action

• Suspect adrenal crisis in any child presenting with unexplained collapse, hypotension, vomiting, diarrhea, or altered mental status 1
• Key clinical features include hyperpigmentation, hyponatremia, hyperkalemia, acidosis, and hypoglycemia 1, 2
• Nonspecific symptoms such as severe fatigue, anorexia, nausea, abdominal pain, and drowsiness are common preceding features 3, 4

Acute Crisis Treatment Protocol

• Administer hydrocortisone 100 mg IV/IM immediately (or 50 mg/m² body surface area in children), followed by 100 mg every 6-8 hours until recovery 1
• Simultaneously infuse isotonic (0.9%) sodium chloride solution at 1 L/hour initially until hemodynamic improvement 1
• Search for and treat the underlying precipitant (infection, trauma, stress) once treatment has been initiated 1
• Never delay treatment for diagnostic procedures—treatment of suspected acute adrenal insufficiency takes absolute priority 1

Diagnostic Approach After Stabilization

Initial Diagnostic Testing

• Obtain paired serum cortisol and plasma ACTH levels as the primary diagnostic test 1
• In acute illness: serum cortisol <250 nmol/L with elevated ACTH is diagnostic of primary adrenal insufficiency 1
• Serum cortisol <400 nmol/L with elevated ACTH raises strong suspicion requiring further evaluation 1

Confirmatory Testing in Equivocal Cases

• Perform the 250-μg ACTH (synacthen/tetracosactide) stimulation test when diagnosis is uncertain 1, 5
• Peak serum cortisol <500 nmol/L (or <18 μg/dL) at 30 or 60 minutes confirms adrenal insufficiency 1, 5
• The high-dose test is preferred over low-dose due to easier administration and established reference ranges 5

Etiological Investigation

• Start with measurement of 21-hydroxylase (anti-adrenal) autoantibodies 1
• If antibodies are negative, obtain CT imaging of the adrenal glands 1
• In male patients, assay very long-chain fatty acids to exclude adrenoleukodystrophy 1
• Consider autoimmune polyglandular syndrome type 1 (APS-1) in children presenting with adrenal insufficiency plus hypoparathyroidism and/or candidiasis—confirm with anti-interferon omega antibodies or AIRE gene mutation analysis 1

Chronic Replacement Therapy

Glucocorticoid Dosing

• Children require hydrocortisone 6-10 mg/m² body surface area daily in split doses 1
• First dose immediately after waking, last dose at least 6 hours before bedtime 1
• Use the lowest dose compatible with health and sense of well-being 1

Mineralocorticoid Replacement

• Most children with primary adrenal insufficiency require fludrocortisone 50-200 μg daily as a single dose 1
• Children and younger adults typically require higher doses than older patients 1
• Advise liberal salt intake and avoidance of liquorice and grapefruit juice 1

Stress Dosing Guidelines

• During minor to moderate illness, double or triple oral hydrocortisone doses 1
• Surgery and invasive procedures require IV/IM hydrocortisone with increased oral doses 1
• Patients taking physiological replacement doses (≥10-15 mg/m² hydrocortisone equivalent for ≥1 month) are at significant risk of adrenal crisis during stress 6

Special Pediatric Considerations

Age-Specific Issues

• Adrenal lesions in young adults, children, and pregnant patients are more likely to be malignant and require expedited evaluation 1
• Corticotroph adenomas account for 55% of pituitary adenomas in children aged 0-11 years 6
• Glucocorticoid-remediable hypertension typically appears early in life, usually in childhood 1

Growth and Development Monitoring

• Monitor linear growth velocity in all children receiving glucocorticoids, as growth suppression can occur even without laboratory evidence of HPA axis suppression 7
• Growth velocity may be more sensitive than cosyntropin stimulation testing for detecting systemic glucocorticoid exposure 7
• Titrate to the lowest effective dose to minimize growth effects 7

Congenital Adrenal Hyperplasia Specific Management

• Measure serum 17-hydroxyprogesterone to exclude congenital adrenal hyperplasia in bilateral adrenal lesions 1
• Hydrocortisone is the drug of choice; mineralocorticoid supplementation is particularly important in infancy 7

Prevention and Patient Education

Emergency Preparedness

• All patients must wear Medic Alert identification jewelry and carry a steroid alert card 1
• Provide supplies for self-injection of parenteral hydrocortisone at home 1, 8
• Provide an Adrenal Insufficiency Action Plan with specific stress hydrocortisone doses (oral and IM/IV) to families and emergency personnel 8

Common Pitfalls

• Parents frequently follow sick-day guidance for oral hydrocortisone but rarely administer intramuscular hydrocortisone despite training—this represents a critical gap requiring enhanced education strategies 4
• Failure to increase glucocorticoid doses during acute stress is the primary cause of preventable adrenal crises 3
• Adrenal crisis results in death in 1 in 200 episodes, with mortality rate of 0.5 per 100 patient years 8, 3

Precipitating Factors to Anticipate

• Gastrointestinal illness with vomiting/diarrhea is the most common precipitant 3, 4
• Other infectious diseases, major pain, surgery, strenuous physical activity, and heat exposure require stress dosing 3

Long-Term Follow-Up

Monitoring Schedule

• Review patients at least annually with assessment of health, well-being, weight, blood pressure, and serum electrolytes 1
• Monitor for development of new autoimmune disorders, particularly hypothyroidism 1
• Assess for complications of glucocorticoid therapy including bone mineral density every 3-5 years 1

Prognosis and Mortality

• Children with unspecified adrenal insufficiency are overrepresented in mortality statistics 4
• Estimated incidence of adrenal death is approximately one per 300 patient years 4
• Younger children (<2 years) with longer PICU stays and higher cumulative glucocorticoid doses are at increased risk for developing adrenal insufficiency 9