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

Recruiting

• Conditions: AAT Deficiency; Alpha-1 Antitrypsin Deficiency; AATD; Cystic Fibrosis (CF)
• Interventions: Procedure: History and physical exam.; Procedure: Blood draw.; Procedure: Pulmonary function testing.; Drug: Albuterol inhaler.

• Registration Number: NCT01851642
• Lead Sponsor: University of Florida

Brief Summary

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.

Detailed Description

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.

Basic Information
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Recruitment & Eligibility
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Study & Design
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Trial Locations

Study Timeline

• Study Start: 2007-08-09
• Study First Submitted: 2013-04-25
• Study First Submitted QC: 2013-05-07
• Study First Posted: 2013-05-10
• Status Verified: 2024-10
• Last Update Submitted: 2024-10-21
• Last Update Posted: 2024-10-23
• Primary Completion: 2032-07-20
• Study Completion: 2033-07-20

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 220

Inclusion Criteria

• Signed informed consent
• Male or female 18 years of age or older
• Negative pregnancy test for women of childbearing potential
• Hemoglobin >12.5 g/dl measured on the day of participation
• Negative urine nicotine test

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Exclusion Criteria

• Pregnancy or breastfeeding
• Weight < 50 kg
• History of anemia requiring blood transfusions, erythropoietin supplementation, or iron supplementation within the past 36 months
• Known hemoglobin <12.5 g/dl within the past 90 days
• Systolic blood pressure > 180 mmHg and/or diastolic blood pressure >100 mmHg
• Poor venous access
• Large volume blood donation (>200 ml or 7 ounces) within the previous 56 days (e.g. blood donation for the purposes of blood banking)
• Clinically significant cardiac, hemostatic or neurological impairment or any other significant medical condition that, in the opinion of the investigator would affect subject safety (e.g., recent myocardial infarction, history of prolonged bleeding time, cerebral vascular accident, advanced cancer or uncontrolled medical condition)
• Psychiatric or cognitive disturbance or illness that would affect subject safety
• Current smoker

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Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
AAT Deficiency	History and physical exam.	Those diagnosed with Alpha-1 Antitrypsin (AAT) Deficiency. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
AAT Deficiency	Blood draw.	Those diagnosed with Alpha-1 Antitrypsin (AAT) Deficiency. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
AAT Deficiency	Albuterol inhaler.	Those diagnosed with Alpha-1 Antitrypsin (AAT) Deficiency. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
Cystic Fibrosis	Albuterol inhaler.	Those diagnosed with Cystic Fibrosis (CF) with mutation Delta F508. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
Without Lung Disease Diagnosis	Pulmonary function testing.	Those without the diagnosis of AAT Deficiency or CF. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
AAT Deficiency	Pulmonary function testing.	Those diagnosed with Alpha-1 Antitrypsin (AAT) Deficiency. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
Without Lung Disease Diagnosis	Albuterol inhaler.	Those without the diagnosis of AAT Deficiency or CF. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
Cystic Fibrosis	History and physical exam.	Those diagnosed with Cystic Fibrosis (CF) with mutation Delta F508. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
Cystic Fibrosis	Blood draw.	Those diagnosed with Cystic Fibrosis (CF) with mutation Delta F508. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
Cystic Fibrosis	Pulmonary function testing.	Those diagnosed with Cystic Fibrosis (CF) with mutation Delta F508. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
Without Lung Disease Diagnosis	History and physical exam.	Those without the diagnosis of AAT Deficiency or CF. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.
Without Lung Disease Diagnosis	Blood draw.	Those without the diagnosis of AAT Deficiency or CF. At every study visit, a history and physical exam (H\&P), blood draw, and pulmonary function testing (PFTs) with the use of an albuterol inhaler will be done.

Primary Outcome Measures

Name	Time	Method
Evaluation of macrophage function.	On average, within 30 days from the time the blood is collected.	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.

Secondary Outcome Measures

Name	Time	Method
Evaluation of lung function.	On average, within 30 days from the time the testing is completed.	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.
Comparison of the amount of an inflammatory marker in the blood, called C-reactive protein.	On average, within 30 days from the time the blood is collected.	From every study participant, we will collect blood from a vein through and IV.
Comparison of the amount of alpha-1 antitrypsin in the blood.	On average, within 30 days from the time the blood was collected.	From every study participant, we will collect blood from a vein through an IV.

Trial Locations

Locations (1)

Shands at the University of Florida; 🇺🇸 Gainesville, Florida, United States