Pathology

Lymphangioleiomyomatosis Lam Diagnosis and Treatment



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The mysterious disease known as Lymphangioleiomyomatosis has perplexed the medical world since it was first discovered and identified around 1918, and later reported in a medical journal in 1937. Better known as LAM, this rare disease primarily attacks the lungs of women, normally those of child bearing age. Because it is so rare, and similar in symptoms to various other lung diseases, it is sometimes extremely difficult to diagnose.

The disease is caused by muscle cells which grow in the interior of the lung, form into clusters, and eventually cut off airways and the ability to provide oxygen to the rest of the body. While It is estimated that only about 1,000 women in the United States are afflicted with this disease, it is also believed that many others may be affected and as yet undiagnosed. Those who suffer from tuberous sclerosis, a genetic disorder, demonstrate similar symptoms, and some have suggested that there may be some connection between the two. Early signs of the disease are shortness of breath after exertion, and eventually shortness of breath even at rest. Since the clusters of muscle cells can rupture air sacs in the lung, there may sometimes be signs of blood in the sputum, and the disease may cause the collapse of a lung, producing chest pains.

Most of the symptoms of this disease are similar to other lung diseases such as asthma and emphysema, so a diagnosis generally requires an X-ray, CT scan, or, in some cases a biopsy. CT scans are by and large more accurate than X-rays, and the final diagnosis is generally obtained only after extensive testing by a pulmonary physician.

According to the LAM Foundation, treatment for this disease is performed on a strictly individual basis, since each patient responds differently to various methods. Based on the fact that most cases occur in women between puberty and menopause, it is suspected that estrogen may have some effect, however there is no definite proof. In some cases, oxygen therapy is used, and in the most severe, lung transplants may prove necessary.

Patients with this disease are, of course, advised to lead the healthiest lifestyles possible. They should follow a routine of exercise, proper nutrition, and proper rest. They should also avoid smoking or being in areas that may contain second hand smoke.

While there is no definite determined cause for this disease, research is underway to find a treatment, and to more readily diagnose the problem in its initial stages.



http://www.medscape.com/viewarticle/518317

http://www.thelamfoundation.org/







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