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See also

Intravenous fluids��
Dehydration
Hyperkalaemia
Hypoglycaemia

Key points

1. Adrenal crisis most commonly presents in children with known adrenal insufficiency who develop an intercurrent illness or injury. Follow the child’s sick day management plan if available
2. Consider a new presentation of adrenal insufficiency in a child presenting with unexplained severe dehydration or shock��
3. The classic biochemical triad for primary adrenal crisis is��low serum sodium, high serum potassium, and low serum glucose
4. The key elements of treatment include fluid resuscitation, steroid replacement and management of glucose and potassium levels

Background

An adrenal crisis is a physiological event caused by an acute relative insufficiency of adrenal hormones. It may be precipitated by physiological stress in susceptible children. It should be considered in children who have a history of:

• Primary adrenal insufficiency (Addison’s disease)

or

• Secondary adrenal insufficiency:
• Prolonged steroid therapy (2-4 weeks) in the past 12 months
• Hypopituitarism (any known pituitary hormone deficit or clinical features indicating increased risk)

Adrenal crisis may also be the first presentation of underlying adrenal insufficiency or there may be a history suggestive of chronic hypoadrenalism. Consider first presentation of congenital adrenal hyperplasia (CAH) in the collapsed neonate

Assessment

History

• General: weakness, fatigue
• GI: anorexia, nausea/vomiting, abdominal pain, weight loss
• Neurological: dizziness/syncope, headache,��confusion, seizure��
• Fever, screen for infective symptoms or injury as a trigger for presentation
• Known adrenal insufficiency
• Pituitary hormone deficiency eg growth hormone deficiency, central hypothyroidism
• Primary adrenal disease eg CAH, Addison’s
• Chronic steroid use or steroid dependency
• History of cranial irradiation or midline damage eg post pituitary surgery, midline tumours
• Developmental regression (adrenoleukodystrophy)

Examination

• Assess level of consciousness��(reduced GCS is concerning)
• Assess degree of dehydration
• Vital signs:
• Hypotension
• Tachycardia
• Fever
• Lethargy
• Hyperpigmentation in skin creases, nail bed or scars (may be present in primary adrenal failure)
• Atypical genitalia or virilisation in neonates (CAH)
• Neurological changes (adrenoleukodystrophy)

Management

Investigations

All children

• Blood glucose (both bedside and formal): low in cortisol deficiency
• UEC: hyperkalaemia and hyponatraemia. Note that mineralocorticoid deficiency may not be present in secondary adrenal sufficiency. Mild hyponatraemia and normal potassium levels may be noted
• Blood gas: acidosis indicates mineralocorticoid deficiency
  

Additional investigations��if first presentation��(prior to steroid administration if possible)����

• Cortisol
• ACTH
• 17 hydroxyprogesterone
• Aldosterone
• Plasma renin activity
• Urine: urinary steroid profile and urinary sodium��

Treatment

Severely unwell/in crisis

Involve senior clinician and discuss with endocrinology

Children with known adrenal insufficiency will have an individualised sick day management plan. This should be followed in the first instance
��
1.��Steroid replacement
Glucocorticoid replacement

• Follow the child’s adrenal crisis management plan, if available
• If no adrenal crisis management plan available, give IV/IM hydrocortisone sodium succinate as per table below
• Consider repeating the IV/IM hydrocortisone dose if there is a poor response to initial steroid and fluid treatment in the first hour
• Follow with hydrocortisone every 6 hours IV��(see dosing table below)

Mineralocorticoid replacement (only in primary adrenal insufficiency)

• Initial correction is achieved with fluids and the mineralocorticoid activity of stress dose hydrocortisone

Recommended doses of 'stress' hydrocortisone (given IM or IV) by age:

*Some centres use continuous hydrocortisone infusions instead of intermittent dosing. This should be done under the guidance of the local endocrinology team��

��Note: The doses outlined equate to approximately 50-75 mg/m²��as a stat dose initially, followed by 50-75 mg/m²/day divided in 4 doses (6 hourly). These doses will also cover the child's mineralocorticoid replacement over this dosing period ()

Once child is stable:

• When the child is stable, reduce the IV dose, or if tolerating oral medications, switch to triple dose oral hydrocortisone replacement (~30-50 mg/m²/day). This can then be gradually reduced to maintenance levels following the advice of the local paediatric/endocrinology team
• For primary adrenal insufficiency, when the child can tolerate oral fluids, start fludrocortisone after discussion with endocrinologist (usually 50-100 micrograms/day)

2.��Intravenous fluids
See Intravenous fluids

Shock or moderate to severe dehydration:

• Give 0.9% sodium chloride (normal saline) 10 mL/kg IV bolus, assess response. Repeat until circulation is restored
• Replace remaining deficit + maintenance fluid requirements evenly over 24 hours with 0.9%��sodium chloride and 5% glucose��IV
• Check pH, electrolytes and glucose frequently
• Blood gas and blood glucose hourly for 2 hours (more frequent if hypoglycaemic, see below), then 2-4 hourly once normoglycaemic and acidosis correcting
• UEC: 2 hourly initially
• Interval can then be extended once glucose stable and electrolytes normalising, tailor to clinical situation
• Avoid rise in serum sodium >8 mmol/L in 24 hours (see Hyponatraemia)

Mild or no dehydration:

• No bolus
• 1-1.5 x maintenance fluid volume as 0.9% sodium chloride and 5% glucose IV administered evenly over 24 hours
• Check electrolytes and glucose 2-4 hourly as clinically indicated

3.��Treat hypoglycaemia
Hypoglycaemia is common in infants and small children with adrenal insufficiency

• Give a bolus of 10% glucose 2-5 mL/kg IV��and recheck blood glucose level 15 minutes post treatment, then at 30 minutes, to ensure recovery to greater than 4.0 mmol/L
• Continue to monitor as described above��
• Maintenance fluids may require up to 10% glucose in 0.9% sodium chloride IV to maintain normoglycaemia��
• See Hypoglycaemia

4.��Hyperkalaemia
This usually normalises with fluid and hydrocortisone and fludrocortisone replacement

• Children with potassium >5.5 mmol/L should have an ECG and be on cardiac monitoring
• If potassium is >7.0 mmol/L and ECG changes of hyperkalaemia are present (eg peaked T waves ± wide QRS complex ± flattened P waves), treat with nebulised salbutamol and insulin and glucose infusion as per��Hyperkalaemia

5.��Precipitating illness/injury
Identify and treat the illness or injury that precipitated the adrenal crisis��

��

Management to prevent an adrenal crisis

Children with known adrenal insufficiency will have an individualised sick day management plan. This should be followed in the first instance
The following guidance is for when there is no sick day plan available �� ����

Moderately unwell
Moderate illness or injury and/or fever >38^oC and tolerating oral intake and medicines:

• Give hydrocortisone 30 mg/m²/day and increase fluid and carbohydrate intake��()

Vomiting and diarrhoea:

• If one or two vomits give hydrocortisone 30 mg/m²/day��(note that some children will have different corticosteroids as their maintenance steroids and may stress dose with this)
• Increase fluids and carbohydrates��
• If ongoing vomiting, consider child to have an imminent adrenal crisis, even if they are otherwise well.�� This is because oral medications are not reliably absorbed in this scenario
  
  • Where there is an obvious cause eg other family members with gastroenteritis, give an initial 'stress' dose of IV/IM hydrocortisone sodium succinate as above and observe for 4-6 hours ��
  • If there is any doubt as to the clinical status or ability to tolerate oral hydrocortisone, admit for ongoing parenteral hydrocortisone ��

Mildly unwell
For example, respiratory or ear infection with no more than low grade fever <38^oC, looks well, able to tolerate oral intake and no need for antibiotics

• Increase dose of hydrocortisone to 20 mg/m²/day

Surgery

Children with known or suspected adrenal insufficiency require IV stress dosing of hydrocortisone in the peri-operative period��

• This is required to replace the body's usual 'stress' cortisol response in such a scenario
• If adequate replacement is not given, an adrenal crisis may be precipitated

Recommended doses of 'stress' hydrocortisone (given IM or IV) by age:

Perioperative management

• Give stress doses based on age, as per table above
• For elective surgery, the 'initial' dose can be given at induction of anaesthesia (when an IV line can be more easily sited), with 6 hourly parenteral dosing continuing thereafter
• If the child requires emergency surgery, the initial dose should be administered without delay and ongoing doses continued 6 hourly thereafter. Doses can be given IM if IV access is not readily available
• Children having a short general anaesthetic for an elective non-invasive procedure (eg MRI) should have an initial 'stress' dose at induction.��If clinically well and tolerating oral intake after the procedure, they can then recommence their usual replacement therapy

Transition to oral steroid replacement therapy

Please discuss with the endocrinology team who will advise on individual dosing schedules

1. Child with known adrenal insufficiency

• Hydrocortisone replacement
  Once the child is stable and tolerating oral intake post-op, hydrocortisone cover can be changed to oral route. Follow their sick day management plan, or as per local paediatric or endocrinology team guidance
• eg 30-50 mg/m²/day for 3 days, 20 mg/m²/day for 2 days before resuming maintenance therapy��()
• Mineralocorticoid replacement
• Children with primary adrenal insufficiency require mineralocorticoid as well as glucocorticoid replacement��
• The mineralocorticoid activity of 'stress' parenteral doses of hydrocortisone will cover this requirement in the initial period
• Fludrocortisone replacement should be continued at usual maintenance doses
• In a child newly diagnosed with primary adrenal insufficiency, consult the endocrinology team for dosing advice (usually ~50-100 micrograms/day; higher doses required in neonates)

��2.��Child with suspected adrenal insufficiency who has not previously been on replacement therapy

• The initial 'stress' oral hydrocortisone replacement should be at a dose of between ~30-50 mg/m²/day��()
• This can then be gradually reduced to maintenance levels over ~4-5 days
• Usual replacement requirements are ~10-15 mg/m²/day for children with primary adrenal insufficiency, or ~5-8 mg/m²/day in secondary adrenal insufficiency
  

Consider consultation with local paediatric team when

In all cases of adrenal insufficiency

Consider transfer when

• Severe electrolyte or glucose abnormalities��
• Children not responding to stress dose steroids��
• Haemodynamic instability��
• Children requiring care above the level of comfort of the local hospital

For emergency advice and paediatric or neonatal ICU transfers,��see��Retrieval Services

Consider discharge when

Recovering from illness, tolerating oral intake
and

• back on usual dose of glucocorticoid

�� �� �� or

• have a clear plan for weaning steroids��

Parent information

Adrenal Crisis Prevention��

Additional notes

• Children on corticosteroids should wear an identification disc or bracelet carrying the words ‘Adrenal insufficiency:�� In emergency give hydrocortisone at 2 mg/kg IM/IV
• Children with known adrenal insufficiency should have an adrenal action plan for managing sick days��

Last updated October 2024