ESA-SRB-AOTA 2019

The effect of patient-managed stress dosing on electrolytes and blood pressure in acute illness in children with congenital adrenal hyperplasia (#525)

Georgina L Chrisp 1 , David J Torpy 2 , Ann M Maguire 3 4 , Maria Quartararo 1 , Henrik Falhammar 5 6 7 , Bruce R King 8 9 , Craig F Munns 3 4 , Shihab Hameed 4 10 11 , Louise Rushworth 1
  1. School of Medicine, Sydney, University of Notre Dame Australia, Darlinghurst, NSW, Australia
  2. Endocrine and Metabolic Unit, Royal Adelaide Hospital and University of Adelaide, Adelaide, NSW, Australia
  3. The Children's Hospital, Westmead, NSW, Australia
  4. Sydney Medical School, The University of Sydney, Camperdown, NSW, Australia
  5. Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, , Sweden
  6. Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, , Sweden
  7. Menzies School of Health Research and Royal Darwin Hospital, Tiwi, NT, Australia
  8. John Hunter Children's Hospital, Newcastle, NSW, Australia
  9. University of Newcastle, Newcastle, NSW, Australia
  10. Sydney Children's Hospital, Randwick, NSW, Australia
  11. School of Women's and Children's Health, University of New South Wales, Kensington, NSW, Australia

Background: Adrenal crises (AC) are acute episodes of adrenal insufficiency (AI).  Manifestations include hypotension and electrolyte disturbances.  Glucocorticoid stress dosing (SD) can prevent AC progression but its effect on physiological parameters has not been assessed in a “real world setting”.

Aim: To assess the effect of prior self-managed glucocorticoid dose escalation on physiological markers in children with AI presenting to hospital for an acute illness.

Methods: An audit of records of all children with congenital adrenal hyperplasia (CAH) and an acute medical illness attending a paediatric referral hospital between 2000 and 2016.  Potassium, sodium and glucose levels, and hypotension (classified using age-related normal levels or delayed capillary return), were compared between children who had and had not used SD.

Results: There were 321 attendances by patients with CAH and an acute illness during the study period.  Any form of SD was used by 64.2% (n=206); IM was used by 22.1% (n=71) and oral only by 41.7% (n=134). Use of SD (oral and/or IM hydrocortisone) was associated with a significantly lower mean potassium level (SD= 4.02+/- 0.71 and No SD=4.27+/- 0.79 mmol/l, p<0.05).  More patients (71.6%, n=151) had used SD in the normokalaemic group than among those with hyperkalaemia (28.6%, n=4), p<0.01.  Linear regression analysis showed that age (in years): beta =-0.04 (-0.06, -0.02), diarrhoea: beta= 0.41 (0.21,0.61); and SD: beta= 0.21 (0.02,0.41) each exerted an independent significant lowering effect on potassium levels.  SD was not significantly associated with sodium or glucose concentrations or with estimates of hypotension. 

Conclusion: Glucocorticoid dose escalation, as it is implemented in the community, results in a significant reduction in hyperkalaemia and lowers mean potassium levels in patients with AI and an acute illness but does not alter significantly sodium and glucose concentrations.  The incidence of hypotension was not associated with SD in this population.