MANAGEMENT

General Initial Management 1.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.Electrolytes 2.Glucose 3.17-hydroxyprogesterone levels 4.Cortisol levels 5.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.