Pediatric emergency medicine trisk 1883 1883
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hydrocortisone intravenously. In the absence of a body surface area calculation, hydrocortisone
can be given as 1 to 2 mg/kg in critical illness. Subsequent management is hydrocortisone 50
mg/m2/24 hrs given intravenously continuously or divided every 6 hours. Volume expansion is
accomplished with normal saline (20 to 60 mL/kg) in the first hour, followed by fluids
appropriate for maintenance and replacement. Additional Na+ may be needed in primary
adrenal insufficiency because of ongoing urinary Na+ losses. These fluids should contain 10%
dextrose and should not contain potassium until the serum potassium is within the normal
range.
Mineralocorticoid therapy is rarely important in the acute phase, provided fluid therapy is
adequate; however, patients with primary adrenal insufficiency may need replacement with a
mineralocorticoid for long-term management. Hydrocortisone acts at the mineralocorticoid
receptor when dosed at stress levels of 50 mg/m2/day. Subsequent long-term therapy can be
accomplished with fludrocortisone. Specific therapy directed toward correction of the
hyperkalemia is rarely required unless cardiac ECG changes (peaked T wave, prolonged QRS
duration) or arrhythmias are present. Hypoglycemia is remedied by the use of dextrose and by
the hyperglycemic effects of glucocorticoids. The precipitating factor, such as infection, also
requires appropriate therapy.
Improvement in peripheral circulation and blood pressure should occur quickly with
therapy. Dramatic improvement often occurs in all parameters within hours after the first dose
of glucocorticoid. Because adrenal crisis is commonly brought on by another stress such as
infection, the symptoms of malaise, anorexia, and lethargy may take longer to resolve.
Clinical Indications for Discharge or Admission
Once instituted, high-dose glucocorticoid therapy should be continued for 48 hours, and
adequate hydration should be maintained either orally or intravenously. The patient known to
be at risk for adrenal insufficiency should wear an identifying bracelet to alert ED personnel to
this possibility.
CONGENITAL ADRENAL HYPERPLASIA
Goal of Treatment
To rapidly initiate treatment for acute salt-wasting crisis and adrenal insufficiency.
CLINICAL PEARLS AND PITFALLS
ED presentations include ambiguous genitalia, acute salt-wasting crisis, and
precocious puberty.
Patients with acute salt-wasting crisis must be recognized and treated immediately
with fluid resuscitation, glucocorticoids, and careful monitoring of electrolytes.
Administer emergency glucocorticoid therapy (50 mg/m2) to patients with known
AI/congenital adrenal hyperplasia (CAH) with fever (T >101.3°F [38.5°C]),
emesis/diarrhea, fracture, altered mental status, or shock.
Current Evidence
