Nebulized or Inhaled Albuterol for Lymphangioleiomyomatosis

Summary

Background: \- 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.

Eligibility

* 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).

Conditions2

Interventions2

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