Lymphangioleiomyomatosis
What is it?
Lymphangioleiomyomatosis (LAM) is a genetic lung disease characterized by an abnormal growth of smooth muscle cells, which causes holes or cysts formation in the lung. It is typically diagnosed in women between the ages 20-40, 30% of them suffer from tuberous sclerosis. In some cases the disease is hereditary and there is also a non hereditary form. People who have this condition suffer from breathing difficulties as the gas exchange becomes less effective and have greater risk for developing pneumothorax. Furthermore, they have a tendency to develop angiolipomas, which are growth in the kidneys, which are harmless unless they become enlarged and cause bleeding.
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Signs & symptoms
Symptoms may include shortness of breath, chest pain, cough, wheezing, pneumothorax, and pleural effusion.
Diagnosis
Diagnosis is based upon findings from medical history and physical examination. Further evaluation may include chest CT scan, lung function tests, VEGF-D blood tests, to measure the level of this hormone in the blood, which is elevated in LAM and a lung biopsy.
Treatment
There is no cure for lymphangioleiomyomatosis but there is a treatment to prevent it from getting worse which is called Sirolimus. Other treatments include oxygen therapy, inhaled medications, procedures to remove fluid from the lungs and lung transplant in severe cases.
☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.
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