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Adrenal Insufficiency in Children

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Dr David Torpy, Australia    21 November 2018

  1. Adrenal insufficiency refers to low plasma cortisol, often confirmed by adrenocorticotropic hormone (ACTH) stimulation testing.
  2. Majority of adrenal insufficiency cases in children (primary/secondary) have a genetic etiology. Testing is based on AI category and associated features. Precise diagnosis may assist in prognosis/management and may assist with family counseling/prenatal diagnosis.
  3. Adrenal crisis is an acute deterioration in health associated with hypotension. Resolution occurs following parenteral glucocorticoid administration.
  4. Adrenal insufficiency treatment in children (chronic) – Treatment with hydrocortisone in 2 or 3 divided doses (total daily dose 8-12 mg/m2 body surface area [BSA], 0.2-0.3 mg/kg) over other types of glucocorticoids replacement therapies; In children with primary adrenal insufficiency, synthetic, long-acting glucocorticoids should be avoided; In confirmed aldosterone deficiency, fludrocortisone can be given; In infants, sodium chloride supplements should be given.
  5. Adrenal crisis treatment in children – Hydrocortisone IV doses (<3 years – 25 mg; 3-12 years – 50 mg; >12 years – 100 mg; OR Hydrocortisone 100 mg/m2 BSA); Bolus D5 normal saline 20 mL/kg over 1 hour, further infusion based on standard resuscitation guidelines.
  6. Adrenal insufficiency in children is rare. Suspect if there is fatigue, weight loss, upper GI distress, hypotension, especially if chronic or subacute.
  7. Clinical assessment of BP, especially for postural hypotension is useful.
  8. Hypoglycemia, and hyponatremia are common; hyperkalemia and pigmentation are not reliably present.
  9. Around 50% have congenital adrenal hyperplasia (CAH), 30% autoimmune, adrenal insults, ACTH resistance and other genetic causes follow.
  10. Associated features are key to establishing genetic cause.
  11. Adrenal insufficiency challenges – Glucocorticoid dosing: There is no reliable biochemical marker of tissue glucocorticoid sufficiency; Adrenal crises are frequent (6-8%/year), and the current preventive strategies not fully effective; Impaired well-being in 40% treated adults (?children).

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