Adrenal insufficiency and adrenal crisis — structured clinical encounter

Recognition and immediate action

The candidate recognises the full primary picture: the hyperpigmentation of the palmar creases, gum margins and old scars, the postural hypotension, the weight loss, and the hyponatraemia with hyperkalaemia and hypoglycaemia. The first action is empiric: 10 to 20 mL per kg of 0.9% saline to perfusion, intravenous dextrose for the hypoglycaemia, and parenteral hydrocortisone 50 to 100 mg stat then 50 to 100 mg per square metre per 24 hours, with cultures and antibiotics because sepsis coexists. [4]

Confirming the diagnosis

Bloods drawn before or alongside the first hydrocortisone dose settle the diagnosis: cortisol low, ACTH high, renin high, aldosterone low. The candidate orders a 250-microgram cosyntropin stimulation test for confirmation, 21-hydroxylase antibodies to prove autoimmunity, and adrenal imaging to exclude an infiltrative or infective cause. The link to her type 1 diabetes and thyroiditis is named as autoimmune polyglandular syndrome type 2, with screening for the further associated endocrinopathies. [1]

Family communication

The conversation names the disease in plain language: her immune system has attacked her adrenal glands, she now needs lifelong replacement of the two hormones they made, and with treatment she can expect a normal life. The candidate builds the stress-dose plan — double or triple the hydrocortisone for any illness, the parent-held emergency intramuscular hydrocortisone injection, a MedicAlert and a school care plan — and teaches the family to inject first and call for help second. [7]

Long-term follow-up and the preventable death

The candidate closes on monitoring and prognosis: titrate hydrocortisone to growth, wellbeing and renin rather than to a single number, screen periodically for the associated autoimmune diseases, and arrange structured transition to adult endocrinology. The preventable failure is named — the missed crisis treated as gastroenteritis or sepsis while the adrenal fails — and the countermeasure is the emergency plan and a low threshold for empiric hydrocortisone in any sick child with an unexplained electrolyte pattern. [3]