OBJECTIVES: I. Determine the prevalence of nontuberculous mycobacteria in sputum cultures from patients with cystic fibrosis.

II. Compare the clinical course of patients with negative versus positive cultures.

PROTOCOL OUTLINE: Participants are screened for nontuberculous mycobacteria (NTM) with 3 sputum acid-fast bacilli smears and mycobacterial cultures, with speciation of positive cultures, collected over 1 year. Demographic data, diagnostic criteria, clinical measurements, and other respiratory pathogen data are collected using the Cystic Fibrosis Foundation Patient Registry Questionnaire.

NTM-positive patients (cases) matched with NTM-negative controls identified in screening are followed every 3 months for 15 months. Cases are evaluated for NTM burden during the first 3 months.

Appropriate therapy is offered to consistently NTM-positive cases. Lung transplant recipients are removed from study.

PROTOCOL ENTRY CRITERIA:

Patients over age 10 with cystic fibrosis documented as follows:

• Pilocarpine sweat chloride test at least 60 mEq/L OR
• Genotyping or transepithelial potential difference AND
• Clinical symptoms of suppurative respiratory disease or pancreatic insufficiency

Able to produce at least 3 sputum samples

• Bronchoscopy specimens acceptable
• Throat/nasal specimens not acceptable

No Pseudomonas cepacia colonization