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. 

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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 is the child with known adrenal insufficiency who has an intercurrent illness.
  • New presentation of adrenal insufficiency: consider this possibility with any critically ill child with unexplained severe dehydration or shock.
  • Neonatal ‘collapse’ in male at 1-3 weeks of age (Congenital Adrenal Hyperplasia).

Causes

Primary Adrenal Disease (↑ACTH Levels)

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

Secondary Adrenal Insufficiency (↓ACTH Levels)

  • Pituitary disorders
  • Hypothalamic disorders

Withdrawal from pharmacological doses of corticosteroids

Assessment

Clinical features

  • Shock
  • 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 Na+, high K+)
  • Elevated serum urea and creatinine
  • Low cortisol.

Investigations

  • Blood glucose level (bedside)
  • Blood gas
  • EUC 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

Management of 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 these guidelines 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.
    • If more unwell and / or temperature >39ºC give four times their usual dose of hydrocortisone.
    • 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 IM or IV hydrocortisone 2mg/kg.
    • Give oral fluids, if tolerating, and observe for 4-6 hours before considering discharge.
    • If tolerating oral fluids discuss with Emergency of Endocrine consultant before discharge.
    • If not tolerating oral fluids IV fluids are required (see below).

For all other children

Give intravenous fluids:

Shock or severe dehydration

  • 0.9% saline 20 mL/kg IV bolus
  • Repeat until circulation is restored
  • Give remaining deficit plus maintenance as:
    • 0.9% saline with 5% dextrose* over 24 hr
  • Check electrolytes and glucose hourly

Moderate dehydration

  • 0.9% saline 20 mL/kg IV bolus even if NOT clinically shocked
  • Give remaining deficit plus maintenance as:
    • 0.9% saline with 5% dextrose* over 24 hr

Mild or no dehydration

  • No bolus
  • Give maintenance + % dehydration fluid volume administered evenly over 24 hours
* Additional dextrose may be required to ensure euglycaemia

Give hydrocortisone:

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

  • 1mg/kg IV 4 hourly until stable
  • Note: calculations are not accurate for infants <10 kg
  • If stable, discuss further with Paediatric Endocrinologist.
Treat Hypoglycaemia:
  • Hypoglycaemia is common in infants and small children
  • Treat with 2mL/kg of 10% dextrose IV over 20 minutes
  • Maintenance fluids should contain between 5% and 10% dextrose.
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

  • Baseline observations heart rate, respiratory rate, temperature, SpO2, capillary refill, BP and neurological observations
  • At least hourly observations
  • Hourly BGL (increase frequency if initial BGL was not within normal limits)
  • ECG if clinically indicated and cardiac monitoring.

Bibliography

  1. Kirkland L- Adrenal crisis e medicine 2003
  2. Nelson Textbook of Pediatrics: 20th Edition Robert M. Kliegman, Bonita M.D. Stanton, Joseph St. Geme, Nina F Schor Publisher: Elsevier 

Endorsed by:  Director, Emergency Department  Date:  Jan 2017


 Review date:   Jan 2021


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