lymfiangioléiomyotosta

Lymphangioleiomyomatosis, often abbreviated as LAM, is a rare, progressive lung disease that primarily affects women. It is characterized by the abnormal growth of smooth muscle-like cells, called LAM cells, in the lungs. These cells can obstruct the airways and lymphatic vessels, leading to symptoms such as shortness of breath, coughing, and chest pain. LAM can also affect other parts of the body, including the kidneys and the lymphatic system, potentially causing tumors known as angiomyolipomas in these areas.

The exact cause of LAM is not fully understood, but it is known to be associated with mutations in the TSC1 or TSC2 genes. These genes are involved in the regulation of cell growth and division. In most cases, these mutations are sporadic, meaning they occur randomly. However, in some instances, LAM can be inherited as part of a genetic disorder called Tuberous Sclerosis Complex (TSC).

Diagnosis of LAM typically involves a combination of medical history, physical examination, imaging tests such as chest X-rays and CT scans, and sometimes a lung biopsy. There is no cure for LAM, but treatments are available to manage symptoms and slow disease progression. These may include medications to inhibit cell growth, procedures to remove excess air from the lungs, and in severe cases, lung transplantation. Research into new treatments for LAM is ongoing.