Protocol Number: 87-DK-0029

Title:

Diagnosis and Treatment of Patients with Cystic Fibrosis and Other Disorders of the Respiratory System and Pancreas

Number:

87-DK-0029

Summary:

Cystic fibrosis is a disease that affects many parts of the body, particularly the lungs and pancreas. The main purpose of this study is to further understand the natural history, clinical presentation, and genetics of cystic fibrosis.

Patients with cystic fibrosis will be asked to participate in this study by undergoing standard medical tests and procedures. Patients will have a history taken and have a physical examination as well as blood tests, and a sweat test (a test for cystic fibrosis of the pancreas in which electrolytes are measured in collected sweat). Patients may also be asked to provide samples of urine, stool, and sputum for additional tests. More complicated procedures such as bronchoscopy or bronchoalveolar lavage may be required for diagnosis, treatment, or research purposes.

Patients will receive appropriate treatment with antibiotics, pancreatic enzymes, vitamins, physiotherapy, and other agents. Medications may be given by mouth or injected into a vein.

This study will provide patients with information on the prognosis of the disease as well as recommendations for management of cystic fibrosis. In addition this study will provide information to researchers which may be useful in other studies of cystic fibrosis.

Sponsoring Institute:

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

Recruitment Detail

Type: Participants currently recruited/enrolled

Gender: Male & Female

Referral Letter Required: No

Population Exclusion(s): None

Eligibility Criteria:

INCLUSION CRITERIA

Patients and family members with known or suspected CF and related disorders of the respiratory tract or pancreas as defined by clinical presentation, sweat testing and/or genotyping.

Willingness of patients, or guardian to give informed consent.

EXCLUSION CRITERIA

Age less than 4 years.

Concomitant medical, psychiatric or other problems which might complicate interpretation of studies of CF, or for which we are unable to provide adequate care.

Special Instructions:

Only a limited number of patients are being recruited at the present time. If interested, send a written complete copy of your medical history (physical exam and laboratory data) to the primary investigator for evaluation to participate in this protocol, prior to the screening visit.

Keywords:

Cystic Fibrosis

Pancreatic Insufficiency

Bronchiectasis

Recruitment Keyword(s):

None

Condition(s):

Bronchiectasis

Cystic Fibrosis

Pancreatic Insufficiency

Investigational Drug(s):

None

Investigational Device(s):

None

Intervention(s):

None

Supporting Site:

National Institute of Diabetes and Digestive and Kidney Diseases

Contact(s):

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

Citation(s):

Welsh MJ, Fick RB. Cystic fibrosis. J Clin Invest. 1987 Dec;80(6):1523-6.

Collins FS. Cystic fibrosis: molecular biology and therapeutic implications. Science. 1992 May 8;256(5058):774-9.

Roberts DD, Rose MC, Wang WT, Chernick MS, Frates RC Jr. Isolation and Characterization of Mucin from the Serum of Cystic Fibrosis Patients. Am J Respir Cell Mol Biol. 1990 Apr;2(4):373-9.

• Questions about participating in a study, please contact the Patient Recruitment and Public Liaison Office, CC.
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