This study is currently recruiting participants.
Number
13-H-0051
Sponsoring Institute
National Heart, Lung and Blood Institute (NHLBI)
Recruitment Detail
Type: Participants currently recruited/enrolled Gender: Female Min Age: 18 Years Max Age: 100 Years
Referral Letter Required
No
Population Exclusion(s)
Male;Children;Pregnant Women
Keywords
Albuterol; Bronchodilator; Nebulizer; Metered Dose Inhaler
Recruitment Keyword(s)
None
Condition(s)
Lymphangioleiomyomatosis
Investigational Drug(s)
Investigational Device(s)
Intervention(s)
Drug: albuterol inhaler Drug: albuterol nebulizer Procedure/Surgery: PFT
Supporting Site
National Heart, Lung and Blood Institute
- Lymphangioleiomyomatosis (LAM) is a rare type of lung disease that occurs almost exclusively in women. In LAM, muscle tissue grows in the lungs and starts to block the flow of air. It is a progressive disease, and in severe cases may require a lung transplant. One possible treatment to improve breathing in people with LAM is inhaled albuterol. Albuterol can be given in a metered dose inhaler (MDI) or with a nebulizer. Researchers want to compare these methods to see which method best improves lung function in women with LAM.
Objectives:
- To see whether a nebulizer or MDI can better improve lung function in women with LAM.
Eligibility:
- Women at least 18 years of age who have impaired lung function because of LAM.
Design:
- Participants will be screened with a physical exam and medical history. No lab tests will be needed for this study.
- Participants will have a 3-day overnight stay at the National Institutes of Health. Those who are using long-acting inhalers will have to stop taking these drugs 1 week before the study.
- Participants will receive either the nebulizer or two or four puffs of the inhaler. Four puffs of albuterol is a higher dose than is normally prescribed, and is being tested on this study.
- Participants will have each treatment around the same time of day on each of the 3 days. Before and after taking the albuterol, participants will have lung function tests.
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INCLUSION CRITERIA: -Diagnosis of LAM either by tissue biopsy, evidence of lung and other organ involvement (renal angiomyolipomas, chylous effusions, lymphangioleiomyomas), high serum levels of vascular endothelial growth factor D (VGEF-D)(1) or a diagnosis of TSC associated with cystic lung lesions. -Age 18 years or over -Evidence of airflow obstruction: FEV1/VC ratio < fifth percentile of predicted normal and an FEV(1) <80% predicted of the normal values. EXCLUSION CRITERIA: Subjects will be excluded from the study if they meet one or more of the following criteria: -History of hypersensitivity to albuterol or any of its components. -Moderate or large pleural effusions (chest x-ray and or CT scan procedure completed under Protocol 95-H-0186) -History of seizures other than during infancy -Inability to withhold bronchodilators for 24 hours -Cognitive Impairment -Age less than 18 years -Male sex -Status-post lung or kidney transplantation -Pregnant or breast feeding (women of childbearing potential will undergo a blood or urine pregnancy test under Protocol 95-H-0186). -Treatment with monoaminoxidase inhibitors, tricyclic antidepressants or Beta-adrenergic receptor antagonists or long acting anticholinergic bronchodilators who are unable to be discontinued for at least seven days before enrollment. -Patients with URI, uncontrolled hyperthyroidism or severe gastro-esophageal reflux. Major systemic diseases (i.e., malignancy; myocardial infarction or unstable angina; type 1 diabetes, severe hypertension; liver cirrhosis).
-Diagnosis of LAM either by tissue biopsy, evidence of lung and other organ involvement (renal angiomyolipomas, chylous effusions, lymphangioleiomyomas), high serum levels of vascular endothelial growth factor D (VGEF-D)(1) or a diagnosis of TSC associated with cystic lung lesions.
-Age 18 years or over
-Evidence of airflow obstruction: FEV1/VC ratio < fifth percentile of predicted normal and an FEV(1) <80% predicted of the normal values.
EXCLUSION CRITERIA:
Subjects will be excluded from the study if they meet one or more of the following criteria:
-History of hypersensitivity to albuterol or any of its components.
-Moderate or large pleural effusions (chest x-ray and or CT scan procedure completed under Protocol 95-H-0186)
-History of seizures other than during infancy
-Inability to withhold bronchodilators for 24 hours
-Cognitive Impairment
-Age less than 18 years
-Male sex
-Status-post lung or kidney transplantation
-Pregnant or breast feeding (women of childbearing potential will undergo a blood or urine pregnancy test under Protocol 95-H-0186).
-Treatment with monoaminoxidase inhibitors, tricyclic antidepressants or Beta-adrenergic receptor antagonists or long acting anticholinergic bronchodilators who are unable to be discontinued for at least seven days before enrollment.
-Patients with URI, uncontrolled hyperthyroidism or severe gastro-esophageal reflux. Major systemic diseases (i.e., malignancy; myocardial infarction or unstable angina; type 1 diabetes, severe hypertension; liver cirrhosis).
Principal Investigator
Referral Contact
For more information: