Paediatric Adrenal Crisis

Introduction

• Consider in all acute severe cardiorespiratory collapse
• Majority of cases in children are primary adrenal collapse (e.g. congenital adrenal hyperplasia)
• Can also be known adrenal insufficiency in time of stress
• Glucocorticoid deficiency – Hypoglycaemia, hypotension, refractory shock
• Mineralocorticoid deficiency – Dehydration, hyperkalaemia, hyponatraemia, acidosis and pre-renal failure
• Always consider in collapsed neonate (especially male)
• Management – Fluid resuscitation, replace corticosteroid and treat hypoglycaemia
• Can be prevented by predetermined sick day plan

DDx

• Primary adrenal failure
  • Congenital adrenal hyperplasia (1/12500 live births)
    • 95% 21-hydroxylase deficiency
      • Catalyses conversion of progesterone and 17-hydroxyprogesterone to deoxycorticosterone and 11-deoxycortisol respectively
  • Addison’s disease (autoimmune)
  • Adrenal aplasia/hypoplasia
  • Adrenal infarction due to haemorrhage/sepsis (Waterhouse-Friedrichsen syndrome)
  • Trauma/tumour/post-surgical
  • Pyloric stenosis
  • Sepsis
• Secondary adrenal failure
  • CNS tumour or trauma
  • Idiopathic
  • Exogenous steroid therapy (including inhaled corticosteroids)

Examination

• Signs of glucocorticoid deficiency – Hypoglycaemia, hypotension, refractory shock
• Signs of mineralocorticoid deficiency – Dehydration, hyponatraemia, hyperkalaemia, pre-renal failure, acidosis (NOT Alkalosis as in pyloric stenosis)
• Signs of excessive ACTH secretion – Hyperpigmentation (esp. axillae, lips, nipples, genitalia, creases)
• Signs of glucocorticoid therapy – Cushingoid
• Signs of hypothalamic/pituitary disorder – Growth abnormality, midline defects, hypogonadism, diabetes insipidus or hypothermia
  • Classically virilization in absence of palpable gonads

Investigations

• BSL
• ECG
• Chem20
• Blood gas
• Save clotted blood for random cortisol level and 17-hydroxyprogesterone (elevated in 21-hydroxylase deficiency as builds up)

DDx

• Other causes of hyponatraemia
  • SIADH
  • Cerebral salt wasting
  • GI or urinary sodium losses
• Other causes of shock
  • Congenital cardiac disease
  • Septicaemia
  • Profound dehydration
  • Pyloric stenosis (not usually hyponatraemic or acidotic)

Treatment

• Fluid resuscitation
• Replace deficit + ongoing losses + maintenance over 24 hrs
  • Maintenance requirements are 1.5x normal in this situation
  • Use N/saline + 5% dextrose
• Replace corticosteroid
  • 2mg/kg IM hydrocortisone if known adrenal insufficiency and unwell but not dehydrated
  • If unwell, give hydrocortisone IV (or IM if delayed)
    • Neonate: 25mg stat then 10-25mg q6h
    • 1mo – 1 year: 25mg stat then 25mg q6h
    • 1-3yo: 50mg stat then 50mg q6h
    • 4-10yo: 75mg stat then 75mg q6h
    • >10yo: 100mg stat then 100mg q6h
• Maintenance doses of glucocorticoid +- mineralocorticoid initiated once stabilised

• Treat hypoglycaemia
  • Neonate/infant: 5mL/kg of 10% dextrose
  • Older child: 2mL/kg 25% dextrose
  • Maintenance fluid with 5-10% dextrose
• Treat hyperkalaemia (if K >7 with ECG changes)
  • Peaked T waves, prolonged PR, P wave wide/flat/disappears, wide QRS, conduction block, bradycardia, sine wave, asystole/VF
  • 0.5mL/kg 10% calcium gluconate over 3-5 min
  • 0.1U/kg/hr of insulin + 2mL/kg/hr of 50% dextrose
• Ongoing salt supplementation

Disposition

• Admit all with established adrenal crisis
• If mild case in susceptible patient that has responded to therapy, consider discharge after 6 hours of observation

Prevention

• Moderately unwell/38-39 degrees – 3x oral dose of oral hydrocortisone while sick
• If more unwell/ fever >39 – 4x oral dose of oral hydrocortisone while sick
• If vomiting – 2mg/kg IM hydrocortisone
• If gastroenteritis or diarrhoea – 4x oral dose of oral hydrocortisone while sick
• Do not increase fludrocortisone dosing

21-hydroxylase deficiency

• Presents in neonatal period in 1 of 4 ways:
  • Virilised female neonate
    • Clitoral enlargement through to complete labial fusion and male appearance without palpable gonads
    • Hyperpigmentation
    • FHx of parenteral consanguinity or other affected siblings
  • Male neonate with metabolic and haemodynamic collapse from a week post-birth
    • Scrotal hyperpigmentation may be only sign in first week
    • Mild FTT through to complete collapse
    • Poor feeding, lethargy, weight loss, FTT progressing to vomiting, salt-losing adrenal crisis and haemodynamic collapse
    • Often misdiagnosed as sepsis or pyloric stenosis
      • Major clinical differentiator is hyperpigmented scrotal skin and the nature of the electrolyte disturbance (??)
      • Most females are diagnosed in first days of life due to genital ambiguity
  • Significant metabolic derangement due to adrenal insufficiency
    • Usually weeks 2-8
    • Recurrent vomiting (misdiagnosed as pyloric stenosis), FTT and haemodynamic collapse
    • Hyperkalaemia, hyponatraemia, metabolic acidosis (!! NOT alkalosis as in pyloric stenosis) and hypoglycaemia (rarely)
  • Antenatal diagnosis

Last Updated on November 22, 2021 by Andrew Crofton