- Title
- Normal cortisol response on low-dose synacthen (1 μg) test in children with Prader Willi Syndrome
- Creator
- Nyunt, Ohn; Cotterill, Andrew M.; Archbold, Sinead M.; Wu, Joyce Y.; Leong, Gary M.; Verge, Charles F.; Crock, Patricia A.; Ambler, Geoffrey R.; Hofman, Paul; Harris, Mark
- Relation
- Journal of Clinical Endocrinology & Metabolism Vol. 95, Issue 12, p. E464-E467
- Publisher Link
- http://dx.doi.org/10.1210/jc.2010-0647
- Publisher
- The Endocrine Society
- Resource Type
- journal article
- Date
- 2010
- Description
- Introduction: It has been postulated that central adrenal insufficiency (CAI), resulting from hypothalamic dysfunction, may contribute to the increased unexplained death rates in Prader Willi syndrome (PWS). A study using the overnight metyrapone test reported a 60% prevalence of CAI in children with PWS. We used a low-dose Synacthen test to screen for CAI in children with PWS. Methods: We studied 41 children with genetic diagnosis of PWS [20 males; mean age, 7.68 (±5.23) yr] in five pediatric endocrinology centers in Australasia. All participants were randomly selected, and none had a history of Addisonian crisis. Ten of the cohort were receiving sex hormone therapy, 19 were receiving GH, and four were receiving T4. Their mean body mass index z-score was +1.48 (±1.68). Baseline morning ACTH and cortisol levels were measured, followed by iv administration of 1 μg Synacthen. Post-Synacthen cortisol levels were measured at 30 min, and a cortisol level above 500 nmol/liter was considered normal. Results: The mean baseline ACTH and cortisol were 15 (±14) ng/liter and 223 (±116) nmol/liter, respectively. The mean 30-min plasma cortisol was 690 (±114) nmol/liter, and the average increase from baseline was 201%. Conclusions: Our result suggests that CAI is rare in children with PWS.
- Subject
- central adrenal insufficiency; hypothalamic dysfunction; Prader Willi syndrome; cortisol responses
- Identifier
- http://hdl.handle.net/1959.13/928914
- Identifier
- uon:10483
- Identifier
- ISSN:0021-972X
- Language
- eng
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