Lymphangioleiomyomatosis - Causes, Symptoms and Treatmentswhich many do not know. In additionLymphangioleiomyomatosisis a complex and rare lung disease that usually affects women of childbearing age. THELymphangioleiomyomatosistriggers the body to make an abnormal amount of muscle cells that are deposited in the organs of the body. Most commonly, these cells accumulate in the kidneys, lungs and lymph nodes.
As these cells do not play the role of the organ in which they have collected, the organ will not function efficiently and can cause serious complications for the patient. THELymphangioleiomyomatosisin the lungs causes a decrease in the amount of oxygen that can be transferred to the body resulting in hypoxia or reduction in the delivery of oxygen to the organs.
Causes of Lymphangioleiomyomatosis:There are two types ofLymphangioleiomyomatosis, but both have an unknown cause. Most cases occur in women between the ages of 20 and 40, and there is a higher occurrence in women who have a condition known as a tuberous sclerosis complex (TSC). Approximately 30% of women with TSC will develop
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Symptoms of Lymphangioleiomyomatosis:The uncontrolled growth ofLymphangioleiomyomatosis, is the cells and their effect on nearby body tissues causes the signs and symptoms ofLymphangioleiomyomatosis. The most common signs and symptoms are:
- Lack of airEspecially during physical activity. First, shortness of breath can occur only during high energy activities. Over time, you may have trouble breathing even during simple activities such as dressing and showering.
- Chest Pain or Aches:This pain can be worse when you breathe.
- Common Cough:This can occur with bloody phlegm.
Other signs and symptoms ofLymphangioleiomyomatosisinclude:.
- Pneumothorax: Or collapsed lung. This condition can occur if the lung cysts rupture through the lining of a lung. The air that accumulates in the space between the lung and the chest wall must be removed to renifulate the lung.
- Pleural Spills:This condition can occur if bodily fluids accumulate in the space between the lung and the chest wall. Often, the fluid contains a milky substance called chyle. Excess fluid in the chest can cause shortness of breath because the lung has less room to expand.
- Blood in Urine:This sign may occur in women who have kidney tumors called angiomyolipomas.
- Enlarged Lymph Nodes:These usually occur in the abdomen or chest. Very rarely, enlarged lymph nodes can occur in places where they can be felt, such as the neck or under the arms.
- Abdominal swelling:Sometimes with pain.
- Other Body Swelling:Like legs, ankles or feet.
Other diseases can also cause many of these signs and symptoms. It is important to see your doctor and find out what is causing these problems.
Diagnostic Testing:Providers will have to rule out other medical conditions such as asthma, bronchitis and emphysema to provide a diagnosis ofLymphangioleiomyomatosis. Patients can be treated by a pulmonologist, depending on the case. Using a combination of patient history, diagnosis and lung function testing are the common approach. Tests that may be used include:
• Pulmonary function studies.
• Chest X-ray.
• Pulse oximetry.
• High resolution computed tomography.
• Blood work.
• Lung biopsies.
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Treatment of lymphangioleiomyomatosis:No treatment available to stop cell growthLymphangioleiomyomatosison body. Treatment focuses on treating symptoms and preventing complications. Some approaches used to treatLymphangioleiomyomatosisinclude:
- Medications to open the lungs and move as much air as possible.
- Oxygen therapy.
- Procedures to remove fluid from the chest.
- Lung transplantation.
- Procedures for shrinking kidney tumors, if present.
- Hormone therapy, if appropriate.
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