Adrenal Insufficiency, Prader Willi Syndrome
Conditions
Keywords
Metyrapone, ACTH stimulation test
Brief summary
The purpose of this study is to determine presence of central adrenal insufficiency in children with Prader Willi Syndrome using low dose (1 mcg) ACTH stimulation test compared to results of overnight metyrapone test.
Interventions
OTHERLow dose (1 mcg) ACTH stimulation test
Subjects will have baseline cortisol and ACTH drawn followed by administration of 1 mcg/m2 (max 1 mcg) of cortrosyn. Blood will be drawn at 20 and 40 minutes post cortrosyn for peak cortisol assessment of central adrenal insufficiency.
OTHEROvernight metyrapone test
Subjects will receive metyrapone 30 mg/kg (max 3 grams) by mouth at midnight. Blood will be drawn at 0800 AM the following morning for ACTH, cortisol and 11 deoxycortisol for assessment of central adrenal insufficiency.
Sponsors
Nationwide Children's Hospital
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
2 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
* Individuals with Prader Willi Syndrome ages 2 years and older
Exclusion criteria
* Individuals who are ill at the time of study * Individuals who are pregnant at the time of study * Individuals who it is deemed unsafe to stop taking medications known to affect the results of they study (hydrocortisone, phenytoin, estrogen, acetaminophen, oral anti-diabetic agents).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Prescence of central adrenal insufficiency using overnight metyrapone test | 8 hours |
| Presence of central adrenal insufficiency using low dose (1 mcg) ACTH stimulation test | 40 minutes |
Secondary
| Measure | Time frame |
|---|---|
| Determine peak cortisol value on low dose (1 mcg) ACTH stimulation test that can accurately predict central adrenal insufficiency based on overnight metyrapone test with good sensitivity and specificity. | 0 |
Countries
United States
Outcome results
None listed