Lymphangioleiomyomatosis

Lymphangioleiomyomatosis

lymphangioleiomyomatosis is a very rare disease that leads to overgrowth of cells called smooth muscle cells. this cell type are present in many of the body’s organs and tissues. This disease affects mainly the lungs and lymph nodes of the immune system, leading to dysfunction and the ultimate destruction of these tissues. lymphangioleiomyomatosis occurs mainly in women of premenopausal age, and it is thought that female hormones are involved in the development of the disease.
Other possible symptoms include lymphangioleiomyomatosis crackling and whistling sounds in the lungs, which can be heard on the exam, and builds up fluid in the lungs, called pleural effusion. over time, clubbing of the fingers develop. this symptom, where the fingertips are flatter and club-like, is a sign of chronic hypoxia.
so little is understood about this disease as a standard regimen does not exist, and no cure is available. Most forms of treatment involves treating the symptoms of the disease, which builds up fluid in the lungs and stomach, and the use of medication and extra oxygen to help workers in breathing. female hormones are believed to be involved in the development of the disease, as some experimental treatments involve hormone therapy. for patients with severe lung disease who are in otherwise good general health, a lung transplantation considered.
lymphangioleiomyomatosis eventually causes destruction of lung and kidney tissue, so there always is a fatal disease. even so, with improved diagnosis, treatment and understanding of disease progression, the prospects for newly diagnosed people become much more favorable. Statistics show that about 75 percent of patients with this condition survive as long as eight-and-a-half years after diagnosis.

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