Adrenal Crisis in Pediatric Emergency

An adrenal crisis is a physiological event caused by an acute relative insufficiency of adrenal hormones. It may be precipitated by physiological stress, such as infection or surgery in a susceptible patient. It should be considered in patients with:

• congenital adrenal hyperplasia

• hypopituitarism with replacement therapy

• previous or current prolonged steroid therapy

• a history of central nervous system pathology or neurosurgical procedures.

Assessment

Signs of both glucocorticoid deficiency and mineralocorticoid deficiency should be looked for if adrenal insufficiency is suspected. In the history and examination, you should look for the following:

• glucocorticoid deficiency presenting with weakness, anorexia, nausea and/or vomiting, hypoglycemia, hypotension (particularly postural) and shock

• mineralocorticoid deficiency presenting with dehydration, hyperkalemia, hyponatremia, acidosis and prerenal renal failure.

Investigations

Prompt treatment is essential and should be based on the following investigations:

• immediate blood glucose using a Dextrostix

• serum glucose, urea, sodium and potassium

• arterial or capillary acid base

Where the underlying diagnosis of adrenal insufficiency is suspected, you should collect at least 2 ml of clotted blood for later analysis (cortisol and 17-hydroxyprogesterone). The results are unlikely to be available to help in the emergency management of the patient.

Management

Susceptible patients who present with vomiting

Susceptible patients who present with vomiting but who are not other-wise unwell should be considered to have incipient adrenal crisis. To prevent this from possibly developing further:

• Administer i.v. or i.m. hydrocortisone 2 mg /kg weight

• Give oral fluids and observe for 4–6 hours before considering discharge.

• Discuss with appropriate consultant.

For all other children

For all other children where adrenal insufficiency is suspected, the various abnormalities should be considered, namely, fluid requirements, hydrocortisone requirements, hypoglycemia, hyperkalemia and precipitating causes such as sepsis.

1. Give intravenous fluids

The level of dehydration should be assessed and intravenous fluids given accordingly. For management of various levels of dehydration,

Management of dehydration in adrenal insufficiency

a. For shock and severe dehydration

• Normal saline 20 ml/kg weight i.v. bolus. Repeat until circulation is restored.
• Administer remaining deficit plus maintenance fluid volume as normal saline in 5% dextrose evenly over 24 hours.
• Check electrolytes and glucose frequently.
• After the first few hours, if serum sodium is >130 mmol/liter, change to half normal saline.
• 10% dextrose may be needed to maintain normoglycemia.

b. For moderate dehydration in adrenal insufficiency

• Normal saline 10 ml/kg i.v. bolus. Repeat until circulation is restored.
• Administer remaining deficit plus maintenance fluid volume as normal saline in 5% dextrose evenly over 24 hours.

c. For mild dehydration in adrenal insufficiency

• No bolus.
• 1.5 times maintenance fluid volume administered evenly over 24 hours.

2. Give hydrocortisone

Hydrocortisone must be given intravenously. If intravenous access is difficult, hydrocortisone should be given intramuscularly while you are establishing the intravenous line, in the following doses:

Neonate: hydrocortisone 25 mg stat and then 50 mg/m² per 24 hours by continuous infusion

• 1 month–1 year: hydrocortisone 25 mg stat, then 50 mg/m² body surface per 24 hours by continuous infusion
• Toddlers (1–3 years): hydrocortisone 25–50 mg stat then 50 mg/m² per 24 hours by continuous infusion
• Children (4–12years): hydrocortisone 50–75 mg stat, then 50 mg/m² per 24 hours by continuous infusion
• Adolescents and adults: hydrocortisone 100–150 mg stat, then 50 mg/m² per 24 hours by continuous infusion

When the patient is stable, you should reduce the intravenous hydrocortisone dose, and then switch to triple dose oral hydrocortisone therapy, gradually reducing to maintenance levels (10–15 mg/m² per day).

In patients with mineralocorticoid deficiency, start fludrocortisone at maintenance doses (usually 0.1mg daily) as soon as the patient is able to tolerate oral fluids.

3. Treat hypoglycemia

Hypoglycemia is common in infants and small children. Treat with an intravenous bolus of 5 ml/kg weight 10% dextrose in a neonate or infant and 2 ml/kg weight of 25% dextrose in an older child or adolescent. Maintenance fluids should contain 5–10% dextrose.

4. Treat hyperkalemia

Hyperkalemia usually normalises with fluid and electrolyte replacement.

• If potassium is above 6 mmol/liter, perform an ECG and apply a cardiac monitor as arrhythmias and cardiac arrest can occur.
• If potassium is above 7 mmol/liter and hyperkalemic ECG changes are present (e.g. peaked T waves, wide QRS complex), give 10% calcium gluconate 0.5 ml/kg weight i.v. over 3–5 minutes. Commence an infusion of insulin 0.1 units/kg weight per hour i.v. together with an infusion of 50% dextrose 2 ml/kg weight per hour.
• If the potassium is above 7 mmol/liter with a normal ECG, give sodium bicarbonate 1–2 mmol/kg weight i.v. over 20 minutes, with an infusion of 10% dextrose at 5 ml/kg weight per hour.

5. Identify and treat potential precipitating causes such as sepsis.

6. Admit to appropriate inpatient facility.

Prevention

The prevention of a crisis is usually possible in susceptible individuals. Situations likely to precipitate a crisis should be anticipated and the patient given:

• triple normal oral maintenance steroid dose for 2–3 days during stress (e.g. fever, fracture, laceration requiring suture)

• intramuscular hydrocortisone when absorption of oral medication is doubtful (e.g. in vomiting or severe diarrhea)

• increased parenteral hydrocortisone (1–2 mg/kg weight) before anesthesia, with or without an increased dose postoperatively.