This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This is an open-label study that will be performed with each adult CF subject acting as his/her own control. The objectives are as follows: 1) characterize the physiology of GH secretion in adult Cystic Fibrosis patients in response to growth hormone releasing hormone (GHRH), the combination of low dose GHRP-W + GHRH, and low dose GHRP-2 and high dose GHRP-2 subcutaneously; 2) Determine the effects of GHRP-2 given over 90 days on IGF-1, GH, prolacitn, leptin, ANP and cortisol and on lean body mass, fat mass, and bone mineral content. There will also be evaluations of the effects of pulmonary function as measured by the maximal inspiratory and expiratory forces, spirometry and exercise tolerance, as well as disease-specific quality of life and dyspnea indices.
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