Tissue Collection From People With Cystic Fibrosis

NCT00015756 | Completed

• 2 other identifiers: 01016301-H-0163
• Study Type: observational
• Target: 100
• Locations: 1 country
• Sites: 2

Brief Summary

This study will examine the relationship between bacterial products in lung infections in cystic fibrosis and disease severity. It will examine plasma and lung tissue from cystic fibrosis patients. Patients with cystic fibrosis and having certain genetic characteristics, who are between 9 and 65 years of age and any cystic fibrosis patient undergoing lung transplantation at INOVA Fairfax Hospital in Fairfax, Virginia, may be eligible for this study. Patients who cannot undergo apheresis may be asked to provide up to an additional 100 cc (7 tablespoons) of blood for research to look at bacterial products. Lung specimens of participating transplant patients will be collected at INOVA Fairfax Hospital. Patients who participate in the apheresis portion of the study will be admitted to the NIH Clinical Center for 2 to 3 days. Apheresis is a procedure for collecting large quantities of specific blood components. For this study, plasma-the liquid part of the blood-will be collected. For the procedure, whole blood is collected through a needle in an arm vein, similar to donating blood. The blood is separated into its components by centrifugation (spinning), the plasma and white cells are extracted and collected in a bag, and the red cells are returned to the body, either through the same needle or through another needle in the other arm. During the hospital stay, patients may also be asked to participate in other cystic fibrosis studies involving blood tests, an echocardiogram (ultrasound test of the heart), urine pregnancy test, and pulmonary function (breathing) tests.

Conditions

Cystic Fibrosis

Keywords

Apheresis; Transplantation; Type III Secretion Pathway; Exotoxins; Pseudomonas Aeruginosa; Cystic Fibrosis; CF

Eligibility Criteria

• Age: 9 Years - 65 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• Individuals with CF with an identified mutation in the cystic fibrosis transmembrance regulator (CFTR) (i.e., any of the known variants of the CFTR gene, such as the change in F508 allele) who are between the ages of 18 years to 65 years will be included in the apheresis portion of the study. Patients will have been or will be genotyped under another protocol. Patients must be colonized or have a history of colonization with P. aeruginosa. Patients may have cultured other organisms (e.g., Burkholderia cepacia).
• All patients undergoing lung transplantation will be included in the study. Based on data from various CF registries, the mean age for patients receiving transplant is 26 years with a range from 5 to 59 years. Any patient age 9 and over with CF undergoing transplantation will be eligible for the explantation portion of the study.

You may not qualify if:

• Patients with CF will be excluded from the apheresis portion of the study if they are less than 18 years, weigh less than 40kg, or demonstrate:
• Cardiovascular instability;
• Severe anemia (hematocrit less than 28 percent, hemoglobin less than10gm/ml);
• Thrombocytopenia (Platelets less than 50,000);
• Inadequate venous access in the upper extremities (No central venous catheters will be used);
• Severe coagulation disorder;
• Positive serology for hepatitis B, C, or HIV;
• Pregnancy;
• Pulmonary hypertension;
• Respiratory exacerbation requiring intravenous antibiotics;
• Any other condition which the physician or Apheresis Unit staff considers a contraindication to the procedure.
• Individuals who are unable to provide adequate consent/assent will be excluded from the study.

Sponsors & Collaborators

• National Heart, Lung, and Blood Institute (NHLBI): lead

Study Sites (2)

National Institutes of Health Clinical Center, 9000 Rockville Pike

Bethesda, Maryland, 20892, United States

Location

Medical College of Wisconsin 8701 Watertown Plank Road Milwaukee, WI 53226

Milwaukee, Wisconsin, 53226, United States

Location

Related Publications (1)

• Frank DW. The exoenzyme S regulon of Pseudomonas aeruginosa. Mol Microbiol. 1997 Nov;26(4):621-9. doi: 10.1046/j.1365-2958.1997.6251991.x.

  PMID: 9427393 BACKGROUND

MeSH Terms

Conditions

Cystic Fibrosis; Pseudomonas Infections

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases; Lung Diseases; Respiratory Tract Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Infant, Newborn, Diseases; Gram-Negative Bacterial Infections; Bacterial Infections; Bacterial Infections and Mycoses; Infections

Study Design

• Study Type: observational
• Sponsor Type: NIH

Study Record Dates

• First Submitted: May 4, 2001
• First Posted: May 7, 2001
• Study Start: May 2, 2001
• Study Completion: April 21, 2008
• Last Updated: July 2, 2017

Record last verified: 2008-04-21

Locations

US (2)