Status:

RECRUITING

Nebulized or Inhaled Albuterol for Lymphangioleiomyomatosis

Lead Sponsor:

National Heart, Lung, and Blood Institute (NHLBI)

Conditions:

Lymphangioleiomyomatosis

Eligibility:

FEMALE

18-100 years

Phase:

PHASE1

PHASE2

Brief 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 ...

Detailed Description

We have reported that approximately one third of patients with lymphangioleiomyomatosis (LAM) who have airflow obstruction respond to bronchodilators such as albuterol, a Beta2-adenergic receptor agon...

Eligibility Criteria

Inclusion

  • 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).

Exclusion

    Key Trial Info

    Start Date :

    June 10 2013

    Trial Type :

    INTERVENTIONAL

    Allocation :

    ESTIMATED

    End Date :

    November 1 2027

    Estimated Enrollment :

    100 Patients enrolled

    Trial Details

    Trial ID

    NCT01799538

    Start Date

    June 10 2013

    End Date

    November 1 2027

    Last Update

    December 24 2025

    Active Locations (1)

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    Page 1 of 1 (1 locations)

    1

    National Institutes of Health Clinical Center

    Bethesda, Maryland, United States, 20892

    Nebulized or Inhaled Albuterol for Lymphangioleiomyomatosis | DecenTrialz