View clinical trials related to Lymphangioleiomyomatosis.
Filter by:The purpose of this research study is to find out what effects RAD001 has on angiomyolipomas of a person with Tuberous Sclerosis Complex and to determine the safe dose of RAD001 without toxicity. The hypothesis is that the drug will inhibit the growth of the angiomyolipomas and possibly even cause regression.
The purpose of this study was to determine if rapamycin reduced angiomyolipomata volume in patients with tuberous sclerosis complex or lam.
Lymphangioleiomyomatosis (LAM) is a rare lung disease of women that is caused by genetic mutations. It results in the uncontrolled growth of an unusual type of smooth muscle cell in the lung. These cells invade lung tissue, including the airways, blood vessels, and lymph vessels, and restrict the flow of air, blood, and lymph, respectively. Respiratory failure, lung collapse (pneumothorax), and pleural effusions (chylothorax) are hallmarks of the disease. This study will evaluate the safety and effectiveness of sirolimus, an inhibitor of the mTOR pathway, in stabilizing or improving lung function in people with LAM.
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.
Lymphangioleiomyomatosis (LAM), a disease primarily of women of child-bearing age, is characterized by cystic lung disease and abdominal tumors (e.g., angiomyolipomas). Within the LAM patient population is a subset of patients who develop chylous effusions and lymphangioleiomyomas. Treatment of many of these symptoms has been ineffective. Previous studies with somatostatin and octreotide in other clinical settings have shown reduction in chylous effusions. This study assesses the effectiveness of octreotide in symptomatic patients with LAM, lymphangioleiomyomas and/or chylous effusions/ascites, peripheral lymphedema and chyluria.
To establish a registry of individuals with LAM by forming a consortium of six clinical centers and referring physicians who treat LAM patients.
Pulmonary lymphoangioleiomyomatosis (LAM) is a rare destructive lung disease typically affecting women of childbearing age. Currently, there is no effective therapy for the disease and the prognosis is poor. In order to better study this disease, the National Heart, Lung, and Blood Institute (NHLBI) has developed a registry to keep an official record of patients diagnosed with LAM. This research project will collect data from 6 health care centers as well as outside physicians. Researchers hope to provide valuable information about the rate of lung destruction and quality of life in patients with LAM. Patients participating in this study will be followed for 5 years. Tissue collected from these patients may contribute to the development of future studies on the disease processes of LAM.