Definition:
Lymphangioleiomyomatosis, LMA for brevity, is a rare lung disorder in which abnormal smooth muscle cells grow rapidly in lung and lymph tissue
Diagnosis:
In order to confirm the presence or absence of the LMA, the following are clinical diagnosis are used, to wit: Chest X-ray, pulmonary function tests, blood tests, computed tomography and lung biopsy.
Treatment:
Treatment with progestin in attempt to reduce the effects of the female hormone estrogen is used to those women who are in their premenopausal stage. In the case of those women who are at their childbearing age, treatments involving diminishing the effect of estrogen are employed. Treatment for LMA also includes therapeutic approaches. Respiratory care are also used to relieve shortness of breathing and if the disease becomes severe, lung transplantation may also be considered.
Symptoms and Signs:
Symptoms often includes shortness of breath or dyspnea, coughing, formation of cyst and pneumothorax or lung collapse, swollen lymph nodes, obstruction of ducts which can lead to lymph fluid or chyle leaking into the chest or abdomen and the presence of a non-cancerous tumor or an overgrowth of tissue called angiomyolipoma.
Causes:
The direct cause of LMA is not exactly known. It is however believed that the smooth muscle that occurs in patients with tuberous sclerosis, which are similar to those in renal angiomyolipomas, represents metastases of a benign tumor. It is said that there is a female preponderance to TSC-LAM (Henske EP. Metastasis of benign tumor cells in tuberous sclerosis complex. Genes, Chromosomes and Cancer. December 2003. 38 (4): 376-81).
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