Lymphangioleiomyomatosis is also known as LAM. It is a rare medical condition which is characterized by cystic lung changes, angiomyolipomas and enlargement of the abdominal and pelvic lymphatics. This progressive disease affects lungs, kidneys and axial lymphatics. LAM tends to worsen over time. It leads to respiratory failure and death. This medical disorder was first reported by Lutembacher in 1918.
There are no known causes of LAM. It is observed that women (between menarche and menopause) are more prone to this medical condition. It is observed that more than seventy per cent of women with LAN are in the age group of 20 to 40 years. But experts have not been able to explain the reason behind this. Patients with tuberous sclerosis (TSC) are more likely to be affected by LAM. Thirty per cent of women with TSC are affected by LAM. Patients with LAM generally have abnormal TSC1 and TSC2 genes. These genes play a major role in producing proteins that control cell growth and movement in the body. Experts also believe that estrogen plays an important role in causing LAM.
Pneumothorax (collapsed lung) is caused when cysts rupture through the lining of the lungs. This results in collection of air in the space around the lungs, thereby restricting its expansion. Pleural effusions is a medical condition where body fluids collect between the lungs and the chest wall, making it difficult for the lungs to expand. Pneumothorax and pleural effusions are main symptoms of LAM.
Dyspnea (shortness of breath) is another symptom of LAM. During the initial stages of the disease, patients with LAM experience shortness of breath wile performing strenuous activities. But as the disease progresses this occurs even while performing normal activities. Patients with angiomyolipomas (tumors in the kidneys) may notice blood in the urine.
Patients with LAM experience chest pain while breathing in. Frequent cough is another common symptom of LAM. Blood-stained phlegm may be observed in some cases. Swelling is observed in legs, ankles and feet. Wheezing (whistling sound while breathing), painful swelling in the abdomen and enlarged lymph nodes are other common symptoms of LAM.
Patients with symptoms of LAM should consult a certified physician without delay for further medical care. There is an urgent need to create awareness about this medical condition.
References:
http://www.thelamfoundation.org/medical-providers/hrct-screening.html
http://www.nhlbi.nih.gov/health/dci/Diseases/lam/lam_whatis.html
http://lam.uc.edu/html/symptoms.html
http://www.pulmononline.org/lymphangio.html
