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Central Adrenal Insufficiency in Young Adults with Prader-Willi Syndrome.
MedLine Citation:
PMID:  23311724     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
OBJECTIVE: A high prevalence (60%) of central adrenal insufficiency (CAI) has been reported in Prader-Willi syndrome (PWS) using the metyrapone test. We have assessed CAI in adults with PWS using the low-dose short synacthen test (LDSST). DESIGN: Basal cortisol and ACTH, and 30 minute cortisol after the administration of 1 μg synacthen were determined in 53 PWS adults (33 females). A peak cortisol value of ≥ 500 nmol/l was taken as normal. Hormonal profiles were analyzed in relation to gender, genotype and phenotype. Deficient patients were retested by high-dose short synachten test (HDSST) or a repeat LDSST. RESULTS: Mean±SD basal cortisol and ACTH were 336.6±140.7 nmol/l and 4.4±3.7 pmol/l, respectively. Cortisol rose to 615.4±135.0 nmol/l after LDSST. Eight (15.1%) patients had a peak cortisol response <500 nmol/l, with a lower mean±SD (range) basal cortisol of 184.9±32.0 (138.0-231.7) compared with 364.1±136.6 (149.0-744.5) in normal responders (p < 0.001). Seven of the 8 patients underwent retesting, with 4 (7.5%) showing persistent suboptimal responses. Basal and peak cortisol correlated in females (r = 0.781, p < 0.001). Logistic regression revealed that only female gender and baseline cortisol were predictors of cortisol peaks (adjusted R square 0.505). CONCLUSIONS: While CAI can be part of the adult PWS phenotype, it has a lower prevalence (7.5%) than previously reported. Clinicians are advised to test PWS patient for CAI. Our study also shows that basal cortisol is closely correlated to adrenal response to stimulation, indicating that its measurement may be helpful in selecting patients for LDSST. © 2013 Blackwell Publishing Ltd.
Authors:
Graziano Grugni; Luciano Beccaria; Andrea Corrias; Antonino Crinò; Marco Cappa; Clotilde De Medici; Stefania Di Candia; Luigi Gargantini; Letizia Ragusa; Alessandro Salvatoni; Alessandro Sartorio; Sabrina Spera; Simeone Andrulli; Giuseppe Chiumello; Alessandro Mussa;
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-1-13
Journal Detail:
Title:  Clinical endocrinology     Volume:  -     ISSN:  1365-2265     ISO Abbreviation:  Clin. Endocrinol. (Oxf)     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-1-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0346653     Medline TA:  Clin Endocrinol (Oxf)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2013 Blackwell Publishing Ltd.
Affiliation:
Auxology Division, S. Giuseppe Hospital, Research Institute, Istituto Auxologico Italiano (Italian Auxological Institute), Verbania, Italy.
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