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Adrenal crisis and acute adrenal insufficiency

  • See also       

    Intravenous fluid requirements 
    Dehydration
    Hyperkalaemia
    Adrenal insufficiency: steroid replacement before and after surgery or procedure requiring GA

    Key Points

    1. Adrenal crisis most commonly presents in children with known adrenal insufficiency who develop an intercurrent illness or injury
    2. Consider a new presentation of adrenal insufficiency in a child presenting with unexplained severe dehydration or shock 
    3. The classic triad for primary adrenal crisis is ↓ serum sodium, ↑ serum potassium, and ↓ 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 a susceptible patient. It should be considered in patients who have a history of:

    • known primary adrenal insufficiency
    • hypopituitarism (any known pituitary hormone deficit or clinical features indicating increased risk), or
    • previous or current prolonged course (2-4 weeks) of steroid therapy 

    Adrenal crisis may also be the first presentation of underlying adrenal insufficiency or there may be a history suggestive of chronic hypoadrenalism

    Assessment

    Red flag features in Red

    History

    • Weakness
    • Fatigue
    • Anorexia
    • Dizziness/ Syncope
    • Nausea / vomiting
    • Weight loss
    • Confusion
    • Seizure
    • Screen for infective symptoms or injury as a trigger for presentation

    Examination

    • Assess degree of dehydration
    • Hypotension, tachycardia
    • Pigmentation in skin creases, nail bed or scars (may be present in primary adrenal failure)
    • CNS signs (in Adrenoleukodystrophy)
    • Confusion → coma 

    Management

    Investigations

    All children:

    • Blood glucose (both bedside and formal): low in cortisol deficiency
    • UEC: hyperkalaemia and hyponatraemia indicates mineralocorticoid deficiency
    • Blood gas: Acidosis indicates mineralocorticoid deficiency

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

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

    Treatment

    Severely unwell

    Unstable / in crisis or unwell (vomiting/diarrhoea, drowsy)

    1. Steroid replacement

    1. Give IV bolus of 50-100 mg/m2 hydrocortisone (Solu-CortefTM) immediately (dose for age shown below).  If IV access is not immediately available, give IM while establishing intravenous access
    2. Consider repeating the IV/IM hydrocortisone dose if there is a poor response to initial steroid and fluid treatment
    3. Follow with hydrocortisone 6 hourly IV 
    4. 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 
    5. Mineralocorticoid replacement:  when the patent can tolerate oral fluids, start fludrocortisone (FlorinefTM) at maintenance doses (usually 0.05 - 0.1 mg daily).  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:

    Age Initial dose of IM/IV hydrocortisone THEN hydrocortisone every 6 hours*
    Neonate – 6 weeks 25 mg 5 – 10 mg
    6 weeks – 3 yrs 25 mg 10 mg
    3yrs – 12 yrs 50 mg 12.5 mg ages 3-6 yo 25 mg ages 6-12 yo
    ≥12 years 100 mg 25 mg

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

    2. Intravenous fluids

    See intravenous fluids guideline 
    Shock or moderate to severe dehydration:

    1. Give 0.9% sodium chloride (normal saline) 10-20 mL/kg during the first hour of treatment. Repeat until circulation is restored
    2. Replace remaining deficit + maintenance fluid requirements evenly over 24 hours with 0.9% sodium chloride and 5% glucose 
    3. Check pH, electrolytes and glucose frequently
      • Blood gas and blood glucose hourly for 2 hours; then 2-4 hourly once normoglycaemic and acidosis correcting
      • U&E: 2 hourly initially 
      • interval can then be extended once glucose stable and electrolytes normalising
      • Avoid rapid rise in serum sodium

    Mild or no dehydration:

    • No bolus
    • 1-1.5 x maintenance fluid volume as 0.9% sodium chloride and 5% glucose administered evenly over 24 hours
    • Check electrolytes and glucose frequently as above / clinically indicated

    3. Treat hypoglycaemia

    Hypoglycaemia is common in infants and small children with adrenal insufficiency

    • Give an IV bolus of 10% dextrose 2-5 mL/kg and recheck blood glucose level after 30 minutes to ensure recovery to >4.0 mmol/L
    • Continue to monitor as described above 
    • Maintenance fluids may require up to 10% dextrose in 0.9% sodium chloride to maintain normoglycaemia 

    4. Hyperkalaemia

    This usually normalises with fluid, electrolyte and steroid replacement

    • Children with potassium >6.0 mmol/L should have 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 either calcium gluconate or insulin 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 >380C and tolerating oral intake and medicines

    • Give glucocorticoids 30 mg/m2/day and increase fluids and carbohydrates 

    Vomiting and diarrhoea

    • If one or two vomits give glucocorticoids 30 mg/m2/day 
    • 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 gastro, etc, give an initial 'stress' dose of IV / IM hydrocortisone 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 <380C, looks well, able to tolerate oral intake and no need for antibiotics

    • Increase dose of glucocorticoids to 20 mg/m2/day. 

    Surgery

    See guideline for stress dosing pre and perioperatively 

    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, call the Paediatric Infant Perinatal Emergency Retrieval (PIPER) Service: 1300 137 650.

    Consider discharge when:

    Recovering from illness

    and

    • back on usual dose of glucocorticoid

    OR

    • have a clear plan for weaning steroids 

    Parent information

    Adrenal Crisis Prevention 

    Additional notes

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

    Last Updated August, 2019

  • Reference List

    1. Auron, M. & Raissouni N. (2015). Adrenal Insufficiency. Paediatrics in Review, 36(3), pp. 92-103
    2. Bornstein, S. R., Allolio, B., Arlt, W., Barthel, A., Don-Wauchope, A., Hammer, G. D., Husebye, E. S., Merke, D. P., Murad, M. H., Stratakis, C. A., & Torpy, D. J. (2016). Diagnosis and treatment of Primary Adrenal Insufficiency: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab, 101(2), pp. 364-389.
    3. Bowden, S. A. & Henry, R. (2018). Pediatric Adrenal Insufficiency: diagnosis, management and new therapies. International Journal of Pediatrics, DOI: 10.115/2018/1739831.
    4. El-Maouche, D., Hargreaves, C. J., Sinaii, N., Mallappa, A., Veeraraghavan, P., & Merke, D. P. (2018). Longitudinal assessment of illnesses, stress dosing and illness sequelae in patients with Congenital Adrenal Hyperplasia. J Clin Endocrinol Metab, 103(6), pp.2336-2345.
    5. Güran, T. (2017). Latest insights on the etiology and management of Primary Adrenal Insufficiency in children. J Clin Res Pediatr Endocrinol, 9(suppl 2), pp. 9 – 22.
    6. Kirkgoz, T. & Guran, T. (2018). Primary adrenal insufficiency in children: diagnosis and management. Best Practice & Research Clinical Endocrinology & Metabolism, 32, pp. 397-424.
    7. Miller, B. S., Spencer, S. P., Geffner, M. E., Gourgari, E., Lahoti, A., Kamboj, M. K., Stanley, T. L., Uli, N. K., Wickow, B. A., & Sarafoglou K. (2019). Emergency management of adrenal insufficiency in children: advocating for treatment options in outpatient and field settings. J Investig Med. Epub doi:10.1136/jim-2019-000999.
    8. Starship “Stress Steroid Management” guideline: https://www.starship.org.nz/guidelines/stress-steroid-management/
    9. Perth Children’s Hospital “Adrenal insufficiency” guideline: https://pch.health.wa.gov.au/For-health-professionals/Emergency-Department-Guidelines/Adrenal-insufficiency
    10. Sydney Children’s Hospital “Adrenal insufficiency: emergency management” guideline: http://www.schn.health.nsw.gov.au/_policies/pdf/2014-9017.pdf