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. Cystic fibrosis (CF) is a multi-system hereditary disease characterized by pancreatic injury, poor growth, under-nutrition, chronic pulmonary disease, and the potential for hepatic disease. It is known that each of these features can be present in infants. The progression of lung disease determines survival; however how the other abnormalities relate to this progression is incompletely understood. In addition, given the difficulty with performing pulmonary function tests at a young age improved markers for lung disease are needed. The early recognition of liver involvement may allow improved treatment approaches for those patients who have hepatic disease. Within this study we aim to develop a panel of biomarkers of disease progression in CF that can predict outcome in later childhood. We also test the usefulness of this panel of biomarkers in predicting outcome most importantly pulmonary outcome. The biomarkers that we are testing are: Serum immunoreactive trypsinogen and lipase, fecal elastase, anthropometrics, DEXA to assess bone density, fat soluble vitamin and carotene levels, putative markers of inflammation: (c-reactive protein, elastase alpha one antiprotease Complexes, IL-6, ECP Myeloperoxidase), CT scan of the chest, Systemic markers of hepatic disease, and Nasal Potential Difference Measurements. Our long term objective is to use biomarkers of disease progression as a basis for early intervention. Newborn screening for CF is likely to become more widespread and hence the development of biomarkers for disease progression in a population identified through newborn screening is an important goal.
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