What is lympangioleiomymatosis?
Lympangioleiomymatosis is a very rare disease that causes the overgrowth of cells called smooth muscle cells. This type of cell is present in many organs and tissues of the body. This disease mainly affects the lungs and lymph nodes of the immune system and leads to dysfunction and possible destruction of these tissues. Lympangioleiomymatosis occurs mainly in women of premenopausal age and it is believed that female hormones are involved in the development of the disease. It is known that the overgrowth of smooth muscle tissue leads to a growth of several cysts of similar formations in lymph nodes, lungs and kidneys and causes more abdominal tumors. In the lungs, bronchiola cysts can prevent the airway obstacle and reduced lung capacity. Lymph nodes are defended and enlarged and abdominal tumors can cause digestive symptoms and massive fluid retention. Some patients also show signs of pneumothorax that occurs when air enters the chest cavity due to damaged or zhroa deceived plice. Pneumothorax is a life -threatening health and requires emergency treatment, but people with lympangioleiomyomatosis can show signs of pneumothorax without actually having a condition.
other possible symptoms of lympangioleiomymatosis include cracking and wheezing sounds in the lungs that can be heard after examination, and the accumulation of fluids in the lungs called pleural effusion. Over time, the fingertips can develop. This symptom in which the fingers are flattened and as a club is a sign of starvation of chronic oxygen.
This disease is so little understood that there is no astandard treatment regimen and no medicine is available. Most forms of treatment include the treatment of symptoms such as the accumulation of fluids in the lungs and abdomen and the use of drugs and supplementary oxygen to help breathe. It is assumed that female hormones are involved in the development of the disease, so some experimental summerPuts include hormonal therapy. In patients with severe lung diseases who are otherwise good health, lung transplantation can be considered.
Lympangioleiomymatosis eventually causes destruction of lung and kidney tissue, so it is always a fatal disease. Yet with improved diagnosis, treatment and understanding of the disease progression, the view for newly diagnosed people has become much more favorable. Statistics suggest that about 75 percent of patients with this condition will survive up to eight and a half years after diagnosis.