Asse GH/IGF-1 e Fibrosi Cistica

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.A new class of drugs in the treatment of Cystic Fibrosis (CF) includes two agents: Lumacaftor, which corrects CFTR channel protein, and Ivacaftor, which enhances the expression of this protein. In our study of 2014 we recruited a population of 50 adult patients with CF: 16 patients with growth hormone deficit (GHD): 7 patients with severe and 9 patients with partial GHD. Of the first nine patients whom we decided to re-evaluate with the GHRH + Arginine test, only 5 were in therapy with the association Lumacaftor / Ivacaftor. All five of these patients in therapy showed a marked improvement: two went from a severe GHD to a normal response to the GH / IGF-1 axis test, and three patients who had partial GHD went to normal response. Therefore, the pituitary gland may be damaged by CF and could benefit from the action of correcting drugs