Emergency management in Adrenogenital Syndrome (AGS)

Illnesses & emergencies, accidents, and surgical procedures

Special risk situations in which an adrenal crisis may occur include fever, respiratory or gastrointestinal infections, and other severe illnesses or accidents. To prevent an adrenal crisis, it is important to remember to take a hydrocortisone stress dose in case of fever or infections. In this situation, the hydrocortisone dose is at least doubled; in cases of high fever and poor condition, it is even tripled to quintupled! In case of doubt, always choose the higher hydrocortisone dose when ill! If vomiting recurs, a prednisone suppository can also be administered. Prednisone is a highly effective “cortisone preparation” that can also be given in suppository form.

The following table provides an overview of possible adjustments to the hydrocortisone stress dose. Since hydrocortisone has a relatively short duration of action, in severe illness it may also be useful to divide the increased hydrocortisone dose into several individual doses:

If nothing else helps and the condition continues to worsen, there is also the hydrocortisone emergency injection: hydrocortisone can either be injected into the muscle (“intramuscularly”), like a vaccination, or into the subcutaneous fatty tissue (“subcutaneous injection”). A subcutaneous injection is an off-label use of the medication. Nevertheless, many people with adrenal insufficiency prefer the subcutaneous injection, with the effect occurring only about 11 minutes later than with an intramuscular injection. In any case, after administering the hydrocortisone emergency ampoule, you should see your doctor to check your health status and discuss further steps.

Accidents

For minor injuries such as closed bone fractures, head contusions, or extensive abrasions, doubling the hydrocortisone dose is usually sufficient. In cases of open fractures, multiple fractures, polytrauma, or severe traumatic brain injury, a higher stress dose (3 to 5 times the normal dose) or an intravenous hydrocortisone stress dose of 100 mg/m²/day is indicated if fasting is required due to surgical procedures.

Surgical procedures

For minor outpatient procedures (e.g., at the dentist’s office), doubling the hydrocortisone dose is usually sufficient. For procedures under anesthesia or major surgeries, the anesthesiologist, in consultation with an endocrinologist, will manage the hydrocortisone stress dosing. It is important to point out the congenital adrenal insufficiency during the pre-anesthesia consultation and to bring all important documents (medical report with contact details, emergency card).

Adrenal crisis

Part of the definition of an adrenal crisis is that the clinical condition improves with glucocorticoid (hydrocortisone) administration. Adrenal crises occur at a frequency of about 5–10 per 100 patient-years. During an infectious disease, inflammatory messengers (cytokines) increase, causing an inflammatory reaction in the body. As part of this inflammatory reaction, glucocorticoid resistance develops. In healthy individuals, more cortisol is released to weaken the inflammatory response and overcome glucocorticoid resistance. In a person with adrenal insufficiency, without an increased hydrocortisone dose, the inflammatory reaction cannot be reduced and glucocorticoid resistance cannot be overcome. These two mechanisms drive patients into a potentially life-threatening adrenal crisis. Typical triggers for an adrenal crisis include infectious diseases (especially upper respiratory tract infections, other febrile infections, and gastrointestinal infections) as well as severe accidents or surgeries. The highest risk is seen in infants and school-age children, but reduced treatment adherence in adolescents and young adults also represents an increased risk for adrenal crisis. Because of this risk, people with adrenal insufficiency and their relatives are trained to administer a hydrocortisone stress dose in situations involving infections, fever, surgery, or anesthesia.

An adrenal crisis is defined as a significant impairment of health with at least two of the following symptoms or findings:

• Low blood pressure
• Nausea or vomiting
• Severe fatigue
• Fever
• Drowsiness / apathy
• Hyponatremia (= low sodium level in the blood, so-called salt loss; sodium ≤132 mmol/l) or hyperkalemia
• Hypoglycemia