Study of Alpha-1 Antitrypsin Deficiency and Cystic Fibrosis Effects on Lung White Blood Cell Function

What this Trial Is About

Researchers are investigating how Alpha-1-antitrypsin AAT deficiency and Cystic Fibrosis CF, both genetic disorders, affect lung white blood cells called macrophages and their ability to respond to inflammation. AAT deficiency affects many people with chronic obstructive pulmonary disease COPD and may cause disease progression due to malfunctioning macrophages. CF causes lung tissue inflammation, and macrophages have a key role in both causing and resolving this inflammation. Participants are grouped into three categories those with AAT deficiency, those with CF carrying the Delta F508 mutation, and those without lung disease. At each study visit, all participants undergo a history and physical exam, blood draws, and lung function tests involving forceful breathing before and after using an Albuterol inhaler. The inhaler is used as part of the pulmonary function testing with at least a 30-minute wait between inhaler use and the second lung test. During the study, researchers evaluate lung macrophage function, blood levels of alpha-1 antitrypsin and an inflammatory marker called C-reactive protein, and lung function. These assessments are done on average within 30 days after sample collection or testing. Participants medical history, physical exams, and lung testing are repeated at each visit to monitor changes and understand how these diseases impact lung immune response and inflammation.

Lung Disease and Its Affect on the Work of White Blood Cells in the Lungs