Central adrenal insufficiency in young adults with Prader-Willi Syndrome

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

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

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-min 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 analysed 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

Original languageEnglish
Pages (from-to)371-378
Number of pages8
JournalClinical Endocrinology
Volume79
Issue number3
DOIs
Publication statusPublished - Sep 2013

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Prader-Willi Syndrome
Adrenal Insufficiency
Hydrocortisone
Young Adult
Adrenocorticotropic Hormone
Metyrapone
Interpersonal Relations
Genotype
Phenotype

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism

Cite this

Central adrenal insufficiency in young adults with Prader-Willi Syndrome. / Grugni, Graziano; Beccaria, Luciano; Corrias, Andrea; Crinò, Antonino; Cappa, Marco; De Medici, Clotilde; Di Candia, Stefania; Gargantini, Luigi; Ragusa, Letizia; Salvatoni, Alessandro; Sartorio, Alessandro; Spera, Sabrina; Andrulli, Simeone; Chiumello, Giuseppe; Mussa, Alessandro.

In: Clinical Endocrinology, Vol. 79, No. 3, 09.2013, p. 371-378.

Research output: Contribution to journalArticle

Grugni, G, Beccaria, L, Corrias, A, Crinò, A, Cappa, M, De Medici, C, Di Candia, S, Gargantini, L, Ragusa, L, Salvatoni, A, Sartorio, A, Spera, S, Andrulli, S, Chiumello, G & Mussa, A 2013, 'Central adrenal insufficiency in young adults with Prader-Willi Syndrome', Clinical Endocrinology, vol. 79, no. 3, pp. 371-378. https://doi.org/10.1111/cen.12150
Grugni, Graziano ; Beccaria, Luciano ; Corrias, Andrea ; Crinò, Antonino ; Cappa, Marco ; De Medici, Clotilde ; Di Candia, Stefania ; Gargantini, Luigi ; Ragusa, Letizia ; Salvatoni, Alessandro ; Sartorio, Alessandro ; Spera, Sabrina ; Andrulli, Simeone ; Chiumello, Giuseppe ; Mussa, Alessandro. / Central adrenal insufficiency in young adults with Prader-Willi Syndrome. In: Clinical Endocrinology. 2013 ; Vol. 79, No. 3. pp. 371-378.
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AU - Corrias, Andrea

AU - Crinò, Antonino

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AU - Di Candia, Stefania

AU - Gargantini, Luigi

AU - Ragusa, Letizia

AU - Salvatoni, Alessandro

AU - Sartorio, Alessandro

AU - Spera, Sabrina

AU - Andrulli, Simeone

AU - Chiumello, Giuseppe

AU - Mussa, Alessandro

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N2 - 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-min 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 analysed 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

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