림프관평활근종증에 대한 분무 또는 흡입 알부테롤

• 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).