| name | es-acthd-treatment-regimen |
| description | This skill identifies the appropriate glucocorticoid replacement regimen for childhood cancer survivors with adrenocorticotropic hormone deficiency (ACTHD), recommending hydrocortisone dosing and stress coverage strategies identical to those used in the noncancer population. Triggers include clinician questions such as “What steroid dose should I use for ACTHD replacement?” or “How do I determine glucocorticoid therapy for adrenal insufficiency?” |
Select glucocorticoid regimen for ACTHD
STEP 1 — Gather Information
Confirm ACTHD diagnosis via appropriate dynamic testing (e.g., insulin tolerance test or low-/high-dose ACTH stimulation test) per guideline 6.3; obtain baseline weight, height, and body surface area; assess for concurrent illnesses or planned procedures requiring stress dosing; review current medications that may affect cortisol metabolism (e.g., enzyme-inducing antiepileptics).
STEP 2 — Rule In / Rule Out
Is ACTHD biochemically confirmed? If yes, proceed to glucocorticoid replacement planning; if no, reconsider diagnosis and evaluate for other causes of fatigue, hypotension, or hyponatremia (e.g., primary adrenal insufficiency, sepsis, medication effect) before initiating steroids.
STEP 3 — Classify or Stratify
Calculate physiologic hydrocortisone dose based on body surface area: typical pediatric replacement is 8–10 mg/m²/day divided into two or three doses (e.g., higher dose in the morning). Adjust for age/weight if BSA unavailable: approximate 0.15–0.2 mg/kg/dose TID.
STEP 4 — Decide
Prescribe calculated hydrocortisone regimen with standard stress coverage: double or triple the daily dose during mild illness, fever, or minor stress; administer parenteral hydrocortisone (50–100 mg/m² bolus then continuous infusion or q6h dosing) for major stress, surgery, or adrenal crisis; provide emergency injectable kit and medical alert identification.
Clinical Guardrails / Mimics / Pitfalls
Do not confuse ACTHD with primary adrenal insufficiency (which requires mineralocorticoid replacement); avoid over-replacement to prevent iatrogenic Cushingoid features, weight gain, or bone loss; remember that enzyme-inducing antiepileptics may increase hydrocortisone clearance, necessitating higher doses; never abruptly discontinue glucocorticoids without taper; stress dosing is essential during illness, surgery, or trauma to prevent adrenal crisis.
Concrete Clinical Example
A 12-year-old female survivor of craniopharyngioma treated with 36 Gy cranial radiation presents with fatigue and hypotension; ACTHD confirmed by low-dose ACTH stimulation test. Weight 38 kg, BSA ~1.2 m². Prescribe hydrocortisone 10 mg/m²/day = 12 mg total daily, divided as 6 mg AM, 3 mg early afternoon, 3 mg late afternoon. For fever >38°C, double dose to 24 mg/day divided TID. Provide emergency hydrocortisone injection card.
Source: Hypothalamic Pituitary and Growth Disorders in Survivors of Childhood Cancer: An Endocrine Society Clinical Practice Guideline, Endocrine Society, 2018, https://doi.org/10.1210/jc.2018-01175
