ECE2014 Poster Presentations Pituitary Clinical (<emphasis role="italic">Generously supported by IPSEN</emphasis>) (108 abstracts)
Assessment of the hypothalamus–pituitary–adrenal axis with different corticotropin tests in adult patients with Prader–Willi syndrome
Graziano Grugni 1 , Andrea Corrias 2 , Alessandro Sartorio 1 , Luciano Beccaria 3 , Sarah Bocchini 4 , Stefania Di Candia 5 , Danilo Fintini 4 , Lorenzo Iughetti 6 , Alessandro Mussa 2 , Letizia Ragusa 7 , Luigi Gargantini 8 , Alessandro Salvatoni 9 , Maurizio Delvecchio 10 , Giuseppe Chiumello 5 & Antonino Crinò 4
1Italian Auxological Institute, Verbania, Italy; 2Regina Margherita Children’s Hospital, Turin, Italy; 3A. Manzoni Hospital, Lecco, Italy; 4Bambino Gesù Children’s Hospital, Rome, Italy; 5S. Raffaele Hospital, Milan, Italy; 6University of Modena and Reggio Emilia, Modena, Italy; 7Oasi Maria SS, Troina (EN), Italy; 8Civic Hospital, Treviglio (BG), Italy; 9University of Insubria, Varese, Italy; 10Casa Sollievo della Sofferenza,
S. Giovanni Rotondo (FG), Italy.
Introduction: Hypothalamic–pituitary anomalies are well proven in Prader–Willi syndrome (PWS). In this context, it has been previously reported that central adrenal insufficiency (CAI) may be part of the PWS phenotype. However, the diagnostics of CAI is critical and debated, due to the lack of fully reliable tests. Several studies have looked at the clinical usefulness of the low dose (1 μg) short Synacthen test (LDSST) compared to the conventional dose (250 μg) test (SDSST) in patients with pituitary disease. Actually, the dose used in the SDSST is considered supraphysiological and might produce a deceivingly adequate cortisol (F) response. Nevertheless, other reports suggested no difference between the LDSST and SDSST in patients with multiple pituitary hormone deficiency.
Objective: To compare the F response to both LDSST and SDSST in a large group of PWS adults.
Methods and patients: Forty-six PWS subjects, 23 males, aged 18–41 years, underwent LDSST and SDSST. In each test blood samples for F determination were taken at 0 and 30 min. A F peak >18.1 μg/dl was considered to be a normal response.
Results: The mean peaks of F after LDSST and SDSST were 22.2±0.7 μg/dl (mean±S.E.) and 22.7±0.7 μg/dl respectively (P=0.8). The average increase of F from baseline was 10.4±0.7 μg/dl (LDSST) and 12.1±0.7 μg/dl (SDSST) (P=0.02). The LDSST and SDSST produced 32 normal and two abnormal concordant results. Seven patients who passed the SDSST failed the LDSSD, while five subjects failed the SDSST but passed the LDSSD.
Conclusions: F responses to different SSTs were discordant in 26.1% of our cases. In this light, further research is needed to identify the more reliable test for diagnosing CAI in these patients – e.g. an ITT or overnight metyrapone test on those who had abnormal response to LDSST or SDSST in order to confirm the presence of CAI.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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