The purpose of this study is to look at how Alpha-1-antitrypsin (AAT) deficiency and Cystic Fibrosis (CF) affect white blood cells in the lungs, called macrophages, and their ability to work.
AAT deficiency is a genetic disorder that affects around 100,000 people in the USA, including 1-3% of all people diagnosed with chronic obstructive pulmonary disease (COPD). In AAT deficient people diagnosed with COPD, it was originally believed the cause of the disease was due to a lack of supply of alpha-1 antitrypsin. However, early information gathered in our laboratory suggests another cause of the development of COPD and the progressing of the disease may be due to a malfunction in macrophages. CF is also a genetic disorder which affects 1/300 births among the Caucasian population. One of the main symptoms of CF is inflammation of the lung tissue. Lung macrophages play a major role in lung inflammation as well as in helping to resolve the inflammation. Inflammation is an important defense of the body. It is the body's response to infection causing germs and things that may cause irritation, as well as, a way for the body to repair damaged tissue. We suggest that the effects of AAT deficiency and CF decreases the inflammation response in the lungs and also restricts the ability of macrophages to correct that inflammation once it occurs.
Study Type
OBSERVATIONAL
Enrollment
220
At every study visit, participant's will be asked about their medical history and will have a physical exam.
At each study visit, participants will have an intravenous catheter (IV) placed in one of their veins and blood will be drawn from the IV for study testing.
At every study visit, participants will have their lung function assessed. This is done by blowing forcefully at least 3 times into a tube. Testing will be done two times; before and after the use of an Albuterol inhaler.
Shands at the University of Florida
Gainesville, Florida, United States
RECRUITINGEvaluation of macrophage function.
From every study participant, we will collect blood from a vein through the placement of an intravenous catheter (IV). We will complete various experiments that will allow us to see how well each participant's macrophage cells are working.
Time frame: On average, within 30 days from the time the blood is collected.
Comparison of the amount of alpha-1 antitrypsin in the blood.
From every study participant, we will collect blood from a vein through an IV.
Time frame: On average, within 30 days from the time the blood was collected.
Comparison of the amount of an inflammatory marker in the blood, called C-reactive protein.
From every study participant, we will collect blood from a vein through and IV.
Time frame: On average, within 30 days from the time the blood is collected.
Evaluation of lung function.
Lung function testing will be done on every study participant. This is is done by forcefully blowing into a tube on at least 3 separate occasions. Albuterol, an inhaled medication used to expand lung airways, will be given after lung function testing and then the testing will be repeated after 30 minutes.
Time frame: On average, within 30 days from the time the testing is completed.
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.
At every study visit, participating subjects will take 2 puffs of an Albuterol inhaler after the first set of PFTs, but before the second set of PFTs. There will be at least a 30 minute period after the use of the Albuterol inhaler and the second set of PFTs.