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Endocrine Abstracts (2017) 49 EP641 | DOI: 10.1530/endoabs.49.EP641

Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I3PT, Universitat Autònoma de Barcelona, Sabadell, Barcelona, Spain.


Background: Prader-Willi syndrome (PWS) is a rare genetic disorder evolving morbid obesity and growth hormone deficiency (GHD). Testing GH-IGF1 axis is required before treatment with rhGH in adults with PWS (1). The most common used tests in these patients are GHRH+arginine (GHRH+a) and insulin tolerance test. There are no prospective data for glucagon test (GT) in comparison to GHRH+a in adults with PWS. Mechanism by which GT causes GH release remains unclear, glucagon-induced GH release via noradrenaline secretion has been postulated.

Objectives: To compare two diagnostic tests to evaluate GH secretion status in a cohort of adults with PWS.

Methods: Twenty-one SPW (8M and 13F, aged 29(17–51), BMI 36.2+/−10.2 kg/m2) participated in the study. After an 8–10 h fast, all patients underwent the two different provocative tests in separate days: GHRH (1 μg/kg, max 100 μg) + arginine (5 g/kg, max. 30 g) and GT (1 mg). We considered GHD according to BMI-specific cut-offs in GHRH+a (2) and when GH peak was <3 ng/ml in GT.

Results: With the GHRH+a test, 14 of 21(66′7%) met diagnostic criteria for GHD and 19 of 21 (90%) with GT. Only two patients did not meet criteria for GHD with both tests. Most patients achieved GH peak at minute 45: 3.31 ng/ml (1.6–9.6), median (interquartile range) with GHRH+a test, whereas GH response was blunted with GT. After the glucagon injection, an increment in plasma glucose levels was observed at 30 min with a peak at 60 min. One patient suffered an asymptomatic hypoglycemia during the test.

Conclusions: Glucagon seems to fail to stimulate GH in adults with PWS. Further studies are necessary to elucidate if the underlying autonomic dysfunction described in PWS could be involved.

References

1. J Clin Endocrinol Metab, 2013,98:E1072–E1087.

2. Ther Clin Risk Manag 2016, 12;873–81.

Volume 49

19th European Congress of Endocrinology

Lisbon, Portugal
20 May 2017 - 23 May 2017

European Society of Endocrinology 

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