MANAGEMENT

General Initial Management1. assessment and control of the airways and of ventilation, 2. ABG, ECG and blood pressure monitoring.3. Other measures include:– i.v. glucose and thiamine as required,– emergency measurement of antiepileptic drug levels,

electrolytes and magnesium, – a full haematological screen, – measures of hepatic and renal function.

The cause of the status should be identified urgently and may require treatment in its own right.

Management:

• First line medication: BZPs.• Second line medication:

Phenobarb > phenytoin (in patients under the age of 1-2 yrs.)

Management:

• Other investigations and management strategies include:

• Stat ABG/VBG (with lytes, Hb, & lactate).

• Full set of labwork including LFT’s, ammonia, urine and blood cultures.

• Empiric antibiotics (if needed).• Head U/S or CT

Acute Hyponatremic hypovolemia with neurologic symptoms

• Treatment of choice is 3% hypertonic saline at 100 ml/h.– For each 100 ml of 3% hypertonic saline, serum sodium concentration

increase s by approximately 2 mmol/l. – Duration of hypertonic saline treatment is based on the improvement

in the patient’s symptoms and signs.

• Patients with acute hyponatremia– may be lethargic, disoriented, agitated, and have anorexia and nausea . – Physical findings: abnormal sensorium, pathological reflexes, Cheyne-

Stokes respiration, hypothermia, and seizures. • Severe symptoms: seizures, obtundation, and coma,

– 3% sodium may be infused at 4–6 ml/kg/h.

• The immediate treatment of hyponatremic seizures in neonates:– provide enough sodium in a 10-minute period to elevate serum

sodium level to 125 mEq/L by using 3% normal saline solution.

• The amount of sodium required : (125 -?) x (0.6) x (wt kg) = X mEq

?= patient’s serum sodium, 0.6 is the dilution constant,

X = number of mEq to correct sodium level to 125 mEq.

• Neonates with SIADH– should also be given furosemide 1 mg/kg intravenously – followed by replacing urinary sodium milliequivalent for milliequivalent

with 3% normal saline solution.

• Neonates with congenital adrenal hyperplasia and neonates receiving diluted formulas – do not require furosemide.

• Antiepileptic drugs should be used if seizures persist after the infusion of 3% normal saline solution or if it is not available.

Treatment of CAH• Patients suspected of 21-hydroxylase deficiency

should have the following bloodwork sent:1. Electrolytes2. Glucose3. 17-hydroxyprogesterone levels4. Cortisol levels5. Aldosterone and renin levels.

Treatment of CAH• After drawing appropriate bloodwork:

1. Pt’s with dehydration, hyponatremia, or hyperkalemia should receive a bolus of isotonic crystalloid to restore volume.

2. Hypoglycemic patients should receive a dextrose bolus +/- infusion.

3. Pt’s suspected of adrenal insufficiency should be treated with steroids empirically (i.e. rather than waiting for the results of confirmatory studies).

Treatment of CAH• When administering steroids:– Use an initial dose of HC 1-2 mg/kg IV (followed by q6h

dosing)• The disadvantage of hydrocortisone is that it will confound

any ACTH-Stim testing.• The advantage of hydrocortisone is that it is a complete

steroid—with both glucocorticoid and mineralocorticoid activity.

• Hydrocortisone is recommended in the pediatric population because of its lower potency, which permits easier titration of appropriate doses.

Classic CAH

• Glucocorticoids: hydrocortisone is the glucocorticoid of choice during childhood.– Longer-acting glucocorticoids, such as prednisolone and

dexamethasone, can be used in adults, but they are generally avoided in children because of concerns about growth suppression.

• Mineralocorticoids: to control electrolytes and plasma renin activity. – Mineralocorticoid replacement is achieved with

fludrocortisone.

• Infants with salt-losing CAH often need sodium chloride supplementation. – Routine salt supplementation is not usually needed after the first 6-12

months of life. – Additional salt intake may be needed with exposure to hot weather or with

intense exercise.

• Treatment during physical stress, e.g. febrile illness, surgery, trauma:– Patients with classic CAH need increased, e.g. doubling or tripling, doses

of hydrocortisone.– Intravenous hydration may be required.– Hypoglycaemia may occur with exercise, illness or fasting. Intake of

carbohydrates and glucose should be increased.

All patients should wear or carry medical alert identification specifying adrenal insufficiency.