Dr Kola Tytler MBBS CertHE MBA MSt MRCGPClinical Lead • iatroX
Recommended management strategies for a patient with classic congenital adrenal hyperplasia (CAH) presenting with an adrenal crisis include:
- Immediate administration of intravenous or intramuscular hydrocortisone without delay for diagnostic tests; the adult emergency dose is 100 mg, while paediatric doses vary by age and body surface area (e.g., 100 mg for children over 6 years, 50 mg for children 1–5 years, and 25 mg for infants under 1 year) NICE CKS.
- Initiate rapid intravenous infusion of 0.9% sodium chloride (1 litre over 30 minutes) to correct hypovolaemia and electrolyte imbalances NICE NG243.
- Frequent monitoring of vital signs including blood pressure and heart rate, as well as electrolyte and glucose levels, to guide ongoing management NICE NG243.
- Emergency admission to hospital for specialist care and further management; do not delay transfer by performing diagnostic tests prior to treatment NICE CKS.
- High-dose hydrocortisone provides sufficient mineralocorticoid effect during crisis; therefore, emergency fludrocortisone administration is not required NICE CKS.
- Provide patients and carers with an emergency management plan including instructions for self-administration of hydrocortisone injections and sick-day rules to prevent crisis NICE NG243.
These steps are critical to stabilise the patient and prevent life-threatening complications associated with adrenal crisis in CAH NICE CKS,NICE NG243.