Protocol Number: 01-H-0163

Title:

Tissue Collection Protocol for Individuals with Cystic Fibrosis

Number:

01-H-0163

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.

Sponsoring Institute:

National Heart, Lung and Blood Institute (NHLBI)

Recruitment Detail

Type: Active Accrual Of New Subjects

Gender: Male & Female

Referral Letter Required: Yes

Population Exclusion(s): None

Eligibility Criteria:

INCLUSION CRITERIA:

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.

EXCLUSION CRITERIA:

There are no exclusion criteria for subjects in the transplant portion of the study.

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:

1. Cardiovascular instability;

2. Severe anemia (hematocrit less than 28 percent, hemoglobin less than10gm/ml);

3. Thrombocytopenia (Platelets less than 50,000);

4. Inadequate venous access in the upper extremities (No central venous catheters will be used);

5. Severe coagulation disorder;

6. Positive serology for hepatitis B, C, or HIV;

7. Pregnancy;

8. Pulmonary hypertension;

9. Respiratory exacerbation requiring intravenous antibiotics;

10. 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.

Special Instructions: Currently Not Provided

Keywords:

Apheresis

Transplantation

Type III Secretion Pathway

Exotoxins

Pseudomonas Aeruginosa

Recruitment Keywords:

Cystic Fibrosis

CF

Pseudomonas Aeruginosa

Conditions:

Cystic Fibrosis

Investigational Drug(s):

None

Investigational Device(s):

None

Contacts:

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citations:

Early bacteriologic, immunologic, and clinical courses of young infants with cystic fibrosis identified by neonatal screening

Mechanism of action of Pseudomonas aeruginosa exotoxin A-adenosine diphosphate-ribosylation of mammalian elongation factor 2 in vitro and in vivo

The exoenzyme S regulon of Pseudomonas aeruginosa

• Questions about participating in a study, please contact the Patient Recruitment and Public Liaison Office, CC.
• Technical questions regarding the Clinical Center web site, please contact the Department of Networks and Applications, CC.

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Warren Grant Magnuson Clinical Center (CC)
National Institutes of Health (NIH)
Bethesda, Maryland 20892. Last update: 10/15/2004