Role of Helicobacter Pylori and Its Toxins in Lung and Digestive System Diseases
Condition: Pulmonary Disease; Oropharyngeal Disease; Lymphangioleiomyomatosis; Pulmonary Fibrosis; Asthma; Sarcoidosis
MeSH terms: Lymphangioleiomyomatosis; Lung Diseases; Pulmonary Fibrosis; Sarcoidosis; Fibrosis
Study type: Observational

Summary: 
      This study will examine bacteria and toxins in the mouth, lung and digestive system that may
      be the cause of various diseases or symptoms. H. pylori is a bacterium that produces various
      toxins that may contribute to lung problems. This study will examine specimens collected from
      the mouth, teeth, lung, digestive tract and blood to measure H. pylori and its toxins and
      their effects on cells.

      People 18 years of age and older with or without gastrointestinal disease may be eligible for
      this study. These include people without a history of lung disease as well as patients with
      any of the following: lymphangioleiomyomatosis, asthma, sarcoidosis, other chronic or genetic
      lung disease (e.g., chronic obstructive pulmonary disease, cystic fibrosis or eosinophilic
      granuloma).

      Participants may undergo the following tests:

        -  Blood and urine tests, chest x-ray.

        -  Measurement of arterial blood gases: A small needle is placed in an artery in the
           forearm to collect arterial blood.

        -  Lung function tests: Subjects breathe deeply and occasionally hold their breath. They
           may also receive a medication that expands the airways.

        -  Fiberoptic bronchoscopy with lavage and bronchial brushing: The subject's mouth and
           throat are numbed with lidocaine; a sedative may be given for comfort. A thin flexible
           tube called a bronchoscope is advanced through the nose or mouth into the lung airways
           to examine the airways. Saline (salt water) is then injected through the bronchoscope
           into the air passage and then removed by gentle suction. Next, a small brush is passed
           through the bronchoscope and an area of the airway is brushed to collect some cells for
           examination.

        -  Mouth rinsing or teeth brushing to collect cells.

        -  Endoscopy: A small needle and catheter (thin plastic tube) are placed into an arm vein
           to administer fluids and medications through the vein. A sedative may be given. The
           throat is numbed with lidocaine and a thin flexible tube called an endoscope is inserted
           through the mouth and down the esophagus into the stomach and upper part of the small
           intestine to examine those areas.
    

Detailed description: 
      Vacuolating cytotoxin A (VacA toxin), an 88-kDa multifunctional protein, and other toxins are
      produced by Helicobacter pylori. We hypothesize that H. pylori, VacA toxin, and other toxins
      within the gastrointestinal tract and/or oropharynx are also found in the lung and may
      contribute to decline in lung function. Analyses of gastrointestinal, oropharyngeal, lung and
      blood specimens will improve the understanding of H. pylori, VacA toxin, and other toxins as
      well as their potential role in pathophysiology of disease. The objectives of this
      exploratory protocol are to procure gastrointestinal, oropharyngeal, lung and/or blood
      specimens from healthy research volunteers and subjects with lung disease (e.g.,
      lymphangioleiomyomatosis, asthma, sarcoidosis, pulmonary fibrosis) and to analyze these
      specimens for H. pylori, VacA toxin, and other toxins. We hypothesize that the toxins may
      have a role in the pathogenesis of lung disease and in the subclinical decline in lung
      function seen with aging.
    

Observational model: Case-Control
Time perspective: Prospective
Sampling method: Non-Probability Sample
Study population: 
        139 patient volunteers and 18 healthy research volunteers have participated in this
        protocol at the NIH. 133 female patients with lymphangioleiomyomatosis (age range at
        accrual from 22 to 72 years old: 121 white, 7 black, 4 asian, and 1 unknown). 2 female
        patients with other chronic/genetic lung diseases (age range at accrual from 47 to 71: 2
        white). 4 male patients with pulmonary fibrosis (age range at accrual from 67 to 74: 4
        white). 18 research volunteers without lung disease (age range at accrual from 19 to 67: 6
        white males, 5 white females, 5 black females, 1 hispanic multi-race male, and 1 hispanic
        unknown male.
      
Keywords: Vac A Toxin; Lung; Bronchoscopy; Endoscopy; Cytotoxin; Pulmonary Disease; Lung Disease; Genetic Disease; Oropharyngeal Disease; Pulmonary Fibrosis; Asthma