| Normal cortisol response to high-dose synacthen and insulin tolerance test in children and adults with Prader-Willi syndrome. | |
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MedLine Citation:
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PMID: 20980432 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
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CONTEXT: Prader-Willi syndrome (PWS) is a genetic disease associated with hypogonadism and partial GH insufficiency, possibly explained in part by a hypothalamic dysfunction. Partial insufficiency of the hypothalamic-pituitary-adrenal (HPA) axis has recently been suggested. OBJECTIVE: The objective of the study was to further explore the HPA axis in PWS by use of routine tests. DESIGN: Nonselected PWS patients were examined with a standard high-dose synacthen test or the insulin tolerance test (ITT). A random serum (s) cortisol was measured in case of acute illness. SETTING: The study was conducted at university hospitals in Denmark and Sweden. PATIENTS: Sixty-five PWS patients with a confirmed genetic diagnosis participated in the study. MAIN OUTCOME MEASURES: A s-cortisol value above 500 nmol/liter as well as an increase of 250 nmol/liter or greater was considered a normal response. RESULTS: Fifty-seven PWS patients (median age 22 yr, total range 0.5-48 yr) were examined with the high-dose synacthen test. The median s-cortisol at the time of 30 min was 699 (474-1578) nmol/liter. Only one patient had a s-cortisol level below 500 nmol/liter but an increase of 359 nmol/liter. This patient subsequently showed a normal ITT response. Two patients had increases less than 250 nmol/liter but a time of 30-min s-cortisol values of 600 nmol/liter or greater. These three patients were interpreted as normal responders. Eight patients [aged 26 (16-36) yr] examined with the ITT had a median peak s-cortisol of 668 (502-822) nmol/liter. Four children admitted for acute illnesses had s-cortisol values ranging from 680 to 1372 nmol/liter. CONCLUSION: In this PWS cohort, the function of the HPA axis was normal, suggesting that clinically significant adrenal insufficiency in PWS is rare. |
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Authors:
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Stense Farholt; Rasmus Sode-Carlsen; Jens Sandahl Christiansen; John R Østergaard; Charlotte Høybye |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2010-10-27 |
Journal Detail:
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Title: The Journal of clinical endocrinology and metabolism Volume: 96 ISSN: 1945-7197 ISO Abbreviation: J. Clin. Endocrinol. Metab. Publication Date: 2011 Jan |
Date Detail:
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Created Date: 2011-01-06 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0375362 Medline TA: J Clin Endocrinol Metab Country: United States |
Other Details:
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Languages: eng Pagination: E173-80 Citation Subset: AIM; IM |
Affiliation:
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Centre for Rare Diseases, Department of Pediatrics, Aarhus University Hospital Skejby, 8200 Aarhus N, Denmark. stenfarh@rm.dk. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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