lymphangioleiomyomatosis ne demek?

Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease that primarily affects women of childbearing age. It is characterized by an abnormal growth of smooth muscle cells in the lungs, which can result in the destruction of lung tissue and the formation of cysts.

The exact cause of LAM is not fully understood, but it is believed to be related to mutations in the TSC1 or TSC2 genes, which control cell growth and division. LAM can occur sporadically or be inherited as part of tuberous sclerosis complex (TSC), a genetic disorder that causes tumors to form in various organs, including the brain, heart, and kidneys.

LAM typically presents with symptoms such as shortness of breath, wheezing, coughing, and chest pain. As the disease progresses, lung function declines, and patients may develop complications such as pneumothorax (collapsed lung) and respiratory failure. Treatment options include medications to improve lung function, oxygen therapy, and lung transplantation for severe cases.

Overall, LAM is a rare and complex disease that requires specialized care from a team of healthcare providers, including pulmonologists, genetic counselors, and social workers. With early diagnosis and appropriate treatment, individuals with LAM can have improved quality of life and better outcomes.