Paediatric Acute care Guidelines PMH Emergency Department

Background

  • Contact the Paediatric Endocrinologist 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; think of this possibility with any severely ill child with severe dehydration or shock
  • Neonatal “collapse” in male at 1-3 weeks of age (CAH)

Causes

Primary adrenal diseases (↑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
  • UEC and glucose (formal) 

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

Management

1. Management of children with minor intercurrent illness who are able to 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 presenting to hospital:
    • If moderately unwell and/or temperature is 38º – 39ºC – give 3 times their usual dose of hydrocortisone
    • If more unwell and/or temperature > 39º C  – give 4 times their usual dose of hydrocortisone
    • If vomiting or diarrhoea treat as below 
2. Management of children with minor intercurrent illness who are not able to tolerate oral medication (vomiting)
  • Susceptible patients who present with vomiting but are not otherwise unwell should be considered to have incipient adrenal crisis
  • To attempt to prevent this from developing further:
    • Administer IM or IV hydrocortisone 2 mg/kg
    • Give trial of oral fluids, if tolerating observe for 4-6 hours before considering discharge
      • If tolerating oral fluids discuss with Emergency or Endocrine consultant before discharge
      • If not tolerating oral fluids IV fluids are required (see below)
3. For all other children
  • Give intravenous fluids
Shock or severe dehydration Moderate dehydration

Mild or no 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
  • 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
  • No bolus
  • Give maintenance + % dehydrated fluid volume administered evenly over 24 hours
*Additional dextrose may be required to ensure euglycaemia
  • Give hydrocortisone:
    • Administer hydrocortisone intravenously
    • Cease Fludrocortisone whilst on IV Hydocortisone
    • If IV access is difficult, give IM while establishing intravenous line
    • Initial bolus dose given is according to age:
Age  Weight  Hydrocortisone Bolus Dose
< 6 months  < 7 kg 25mg
6 months – 2 years 8-12 kg  50mg
3-10 years 13-30kg 75mg
> 10 years  > 30kg  100mg
Approximately 2mg/kg   
 
This must be followed by regular hydrocortisone
  • 1mg/kg IV 4 hourly until stable
  • Note: calculations are not accurate for infants < 10kg
  • If stable, discuss further management 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 – See ED Guideline: 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, Sp02, 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

References

External Review: Aris Siafarikas (Endocrinology and Diabetes Consultant): August 2015

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