Lymphangiomyomatosis
Lymphangiomyomatosis
Lymphangiomyomatosis (pronounced lim-fan-je-o-my-o-ma-to-sis), often abbreviated as LAM, is a rare lung disease that primarily affects women of childbearing age.
Etymology
The term "Lymphangiomyomatosis" is derived from three Greek words: "lymph" meaning water, "angio" meaning vessel, "myoma" meaning muscle tumor, and "osis" meaning condition. Thus, the term literally translates to "condition of muscle tumors in the water vessels".
Definition
Lymphangiomyomatosis is a progressive disease characterized by an abnormal growth of smooth muscle cells, especially in the lungs, lymphatic system, and kidneys. This abnormal growth leads to the formation of cysts, obstructing the airways and causing a variety of symptoms.
Symptoms
The most common symptoms of Lymphangiomyomatosis include shortness of breath, cough, chest pain, and fatigue. In severe cases, it can lead to pneumothorax (collapsed lung) and chylous effusion (accumulation of lymphatic fluid in the pleural space).
Causes
The exact cause of Lymphangiomyomatosis is unknown. However, it is believed to be related to mutations in the TSC1 or TSC2 genes, which regulate cell growth and division.
Diagnosis
Diagnosis of Lymphangiomyomatosis is often challenging due to its rarity and non-specific symptoms. It typically involves a combination of clinical evaluation, imaging studies such as HRCT, and lung biopsy.
Treatment
There is currently no cure for Lymphangiomyomatosis. Treatment is aimed at managing symptoms and slowing disease progression. This may involve the use of medications such as sirolimus, lung transplantation in severe cases, and supportive care such as oxygen therapy and pulmonary rehabilitation.
Related Terms
- Tuberous sclerosis complex: A genetic disorder that causes non-cancerous (benign) tumors to grow in many parts of the body.
- Pulmonary Lymphangioleiomyomatosis: A form of LAM that affects the lungs.
External links
- Medical encyclopedia article on Lymphangiomyomatosis
- Wikipedia's article - Lymphangiomyomatosis
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