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Deregulation of the growth hormone/insulin-like growth factor-1 axis in adults with cystic fibrosis

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Abstract

Purpose

Patients with cystic fibrosis (CF) present with signs and symptoms that overlap with those of adult growth hormone deficiency (GHD) syndrome: loss of muscle mass, bone fragility and lower stress tolerance. In literature, the prevalence of GHD in pediatric CF patients is higher than general population, but these studies have been performed on children with growth delay. To our knowledge, there are no studies on adult patients. The aim of this paper is to evaluate GH–IGF1 axis in an adult CF population.

Methods

Fifty clinically stable adult patients, 30 males; age 36 ± 2 years; BMI 21.39 ± 0.22 kg/m2 and FEV1 67 ± 4% were studied. Data regarding glycometabolic status and results of pituitary, thyroid, parathyroid, gonadal and adrenal function tests were recorded. All patients underwent a GH releasing hormone (GHRH) + Arginine stimulation test to confirm a GHD.

Results

GHRH + Arginine test revealed the presence of GHD in 16 patients (32%); specifically 7 patients had a severe deficiency and 9 a partial deficiency.

Conclusions

Adult patients with CF may show GHD. These patients should be followed over time to assess if the GHD could impact the clinical progression of CF.

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Authors and Affiliations

  1. Section of Medical Pathophysiology, Endocrinology, Department Experimental Medicine, Sapienza University, 00161, Rome, Italy

    C. Pascucci, A. M. Isidori, C. Lubrano, L. Gnessi & A. Lenzi

  2. Pediatrics Department, Cystic Fibrosis Regional Center, Sapienza University of Rome, Rome, Italy

    R. V. De Biase, D. Savi & S. Quattrucci

  3. Cystic Fibrosis Unit, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy

    D. Savi

Authors
  1. C. Pascucci
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  2. R. V. De Biase
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  3. D. Savi
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  4. S. Quattrucci
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  5. A. M. Isidori
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  6. C. Lubrano
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  7. L. Gnessi
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  8. A. Lenzi
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Corresponding author

Correspondence to C. Pascucci.

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Conflict of interest

The authors declare that they have no conflicts of interest in this work.

Ethical approval

The present study was approved by the ethics committee of the University and adhered to the tenets of the Declaration of Helsinki.

Informed consent

Additionally, the written informed consents were signed by all participants.

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Cite this article

Pascucci, C., De Biase, R.V., Savi, D. et al. Deregulation of the growth hormone/insulin-like growth factor-1 axis in adults with cystic fibrosis. J Endocrinol Invest 41, 591–596 (2018). https://doi.org/10.1007/s40618-017-0784-x

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  • DOI: https://doi.org/10.1007/s40618-017-0784-x

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