Treatment of CAH Due to 21-Hydroxylase Deficiency During Febrile Illness, Major Surgery, or Trauma
Clinical Scenario
This protocol addresses patients with congenital adrenal hyperplasia who require ongoing glucocorticoid treatment and present with an acute physiological stress. Standard maintenance dosing is insufficient in these situations, and a specific management approach applies.
- Febrile illness with body temperature above 38.5 °C
- Gastroenteritis with dehydration
- Major surgery accompanied by general anesthesia
- Major trauma
Treatment Approach (Partial Summary)
The protocol centres on adjusting the glucocorticoid regimen in response to acute stress. For adrenal crisis, parenteral hydrocortisone is administered, with the specific approach determined by patient age. Timing for returning to maintenance therapy is also specified.
This is a partial overview only — the full dosing algorithm, age-stratified guidance, and management sequence are available via the complete protocol below.
References
DOI: 10.1210/jc.2018-01865
In all patients with CAH who require GC treatment, for situations such as febrile illness (>38.5°C), gastroenteritis with dehydration, major surgery accompanied by general anesthesia, and major trauma we recommend increasing the GC dosage.
In contrast to maintenance treatment given three times daily, we suggest that stress dosing be given every 6 hours.
Successive IV HC may be administered as one-quarter of the initial parenteral HC dose (above) given every 6 h.
Patients should resume maintenance HC doses when stable and avoid fasting during acute illnesses.
View source ↗