Adrenal insufficiency

Disclaimer

These guidelines have been produced to guide clinical decision making for the medical, nursing and allied health staff of Perth Children’s Hospital. They are not strict protocols, and they do not replace the judgement of a senior clinician. Clinical common-sense should be applied at all times. These clinical guidelines should never be relied on as a substitute for proper assessment with respect to the particular circumstances of each case and the needs of each patient. Clinicians should also consider the local skill level available and their local area policies before following any guideline.

Read the full PCH Emergency Department disclaimer.

Aim

To guide staff with the assessment and management of adrenal insufficiency.

Background

Children with adrenal insufficiency may present critically unwell. Contact the on call Paediatric Endocrinologist early if adrenal insufficiency is suspected.

Possible presentations

The most common presentation is the child with known adrenal insufficiency who presents with an intercurrent illness.
Neonatal ‘collapse’ in males at 1-3 weeks of age (Congenital Adrenal Hyperplasia).
Consider a new presentation of adrenal insufficiency in any critically ill child with unexplained severe dehydration or shock.

Causes

Primary adrenal disease (increased adrenocorticotropic hormone (ACTH) levels)

Addison's disease
Congenital adrenal hyperplasia (CAH)
Adrenal aplasia / hypoplasia
Adrenoleukodystrophy

Adrenal destruction secondary adrenal insufficiency (decreased ACTH levels)

Pituitary disorders
Hypothalamic disorders

Withdrawal from pharmacological doses of corticosteroids.

Assessment

Clinical features

Shock
Hypotension
Hypoglycaemia (confusion, coma)
Muscle weakness
Lethargy
Vomiting
Syncope
Dizziness
Weight loss
Depression and anorexia
Increased pigmentation in creases
Dehydration, hypotension and shock.

Biochemical features

Hypoglycaemia
Electrolyte disturbances (low sodium, high potassium)
Elevated serum urea and creatinine
Low cortisol.

Investigations

Blood glucose level (bedside)
Blood gas
Electrolytes, urea, creatinine and glucose (formal).

Where the underlying diagnosis is not known, collect at least 2mL of clotted blood for later analysis (cortisol and 17 hydroxyprogesterone) and keep a specimen on ice for ACTH analysis.

Management to prevent an adrenal crisis

Management of children children with minor inter-current illness who can tolerate oral medication (not vomiting)

Children with adrenal insufficiency or at risk (i.e. on steroids) must be given increased doses of replacement hydrocortisone during illness or stress.
Parents will often have sick day management plans and may have tried these strategies prior to presentation at hospital:
- If moderately unwell and / or temperature is 38ºC–39ºC give three times their usual dose of hydrocortisone.¹
- If more unwell and / or temperature >39ºC give four times their usual dose of hydrocortisone.¹
- If vomiting or diarrhoea treat as below.

Management of children with minor inter-current illness who are not able to tolerate oral medication (vomiting)

Susceptible patients who present with vomiting but are otherwise unwell should be considered to have incipient adrenal crisis.
To attempt to prevent the above from developing further:
- Administer intramuscular (IM) or intravenous (IV) hydrocortisone 2-4mg/kg (maximum 100mg).¹
- Give oral fluids, if tolerating, and observe for 4-6 hours before considering discharge.
- If tolerating oral fluids discuss with Emergency or Endocrinology consultant before discharge.
- If not tolerating oral fluids IV fluids are required (see below).

Management of patients in adrenal crisis

Give intravenous fluids.

IV fluids in adrenal insufficiency

Shock or severe dehydration

Sodium Chloride 0.9% 20 mL/kg IV bolus
Repeat until circulation is restored
Give remaining deficit plus maintenance as:
- Sodium chloride 0.9% with glucose 5% over 24 hr
Check electrolytes and glucose hourly.

Moderate dehydration

Sodium Chloride 0.9% 20 mL/kg IV bolus even if not clinically shocked
Give remaining deficit plus maintenance as:
- Sodium chloride 0.9% with glucose 5% over 24 hr

Mild or no dehydration

No bolus
Give maintenance + % dehydration (Sodium chloride 0.9% with glucose 5%*) fluid volume administered evenly over 24 hours

* Additional glucose may be required to ensure euglycaemia in patients in adrenal crisis

Give hydrocortisone

Administer hydrocortisone intravenously
Withhold fludrocortisone whist on IV hydrocortisone
If IV access is difficult, give IM while establishing IV line
Initial bolus dose given is according to age:

Dosing

Recommended doses of 'stress' hydrocortisone (given IM or IV) by age ^4,5,6

Age	Weight	Hydrocortisone bolus dosage
<6 Months	<7kg	25mg
6 months – 2 years	8-12kg	50mg
3-10 years	13-30kg	75mg
>10 years	>30kg	100mg
Approximately 2-4mg/kg

This must be followed by regular hydrocortisone:

1 month - 12 years: 0.5-1mg/kg IV/IM 4-6 hourly until stable¹
12 - 18 years: 50-100mg every 6-8 hours until stable¹
If stable, discuss further with Paediatric Endocrinologist.

Treat Hypoglycaemia:

Hypoglycaemia is common in infants and small children
Treat with 2mL/kg of glucose 10% IV over 20 minutes
Maintenance fluids should contain between 5% and 10% glucose.

Treat Hyperkalaemia:

Hyperkalaemia usually normalises with fluid and electrolyte replacement

Identify and treat potential precipitating causes such as sepsis
Admit to appropriate inpatient facility.

Nursing

Record baseline observations: heart rate, respiratory rate, temperature, oxygen saturations, capillary refill, blood pressure and neurological observations on the observation and response tool
At least hourly observations
Hourly blood glucose level (BGL) (increase frequency if initial BGL was not within normal limits)
Electrocardiogram (ECG) if clinically indicated and cardiac monitoring
Fluid balance monitoring.

References

AMH Children’s Dosing Companion July 2021; Cited 14 October 2021; Hydrocortisone - AMH Children's Dosing Companion (health.wa.gov.au)
Kirkland L- Adrenal crisis e medicine 2003
Nelson Textbook of Pediatrics: 20th Edition Robert M. Kliegman, Bonita M.D. Stanton, Joseph St. Geme, Nina F Schor Publisher: Elsevier
The Royal Children's Hospital, Melbourne, Australia, Clinical Practice Guideline on Adrenal crisis and acute adrenal insufficiency, Aug 2019, cited 20 October 2021. Available from: Clinical Practice Guidelines : Adrenal crisis and acute adrenal insufficiency (rch.org.au)
Great Ormond Street Hospital for Children, NHS Foundation Trust, Emergency steroid management plan for patients on long term, high dose glucocorticoid treatment: information for families, Dec 2019, Cited 20 October 2021. Available from: Emergency_steroid_treatment_plan_F2208_FINAL_Dec19.pdf (gosh.nhs.uk)
Society for Endocrinology, Adrenal Crisis Information, Cited 20 October 2021. Available from: Adrenal crisis | Society for Endocrinology

Endorsed by:	Drugs and Therapeutics Committee	Date:	Nov 2021
		Review date:	Nov 2024

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