There are many medical conditions that are related to lymphedema, or appear to develop in conjunction with LE. While I was researching this article, I realized that today there is more hope than ever. Scientists are researching the causes of LE and are trying to find appropriate treatments. This is the good news. The bad news is that LE is still misunderstood, misdiagnosed and underfunded in the research arena. We need your help! Step up to the plate by contacting your local, state and national representatives and tell them that you want funding for LE research. Unsure where to start? Contact the Lymphatic Research Foundation (http://www.lymphaticresearch.org), the National Lymphedema Network (http://www.lymphnet.org) for contact information and advice on how to be an active lymphedema advocate.
Milroy's Syndrome was used to describe hereditary congenital lymphedema in the past. The cause is believed to be from a vertical autosomal (from a non-sex chromosome) inheritance of a single gene, VEGFR3. http://www.iovs.org/cgi/content/full/43/... The condition usually is apparent at birth, with swelling of one or both legs. There is generally a family history of lymphedema. Lymphoscintigraphy is the way to verify the diagnosis. A radioactive tracer is injected into the limb and the findings are analyzed on a computer screen. This test will show where the lymphatic blockages are present in the affected limb.
Praecox is a type of primary hereditary lymphedema that usually shows up around puberty . It generally affects the legs and there is usually a family history of lymphedema. Diagnosis is made by lymphoscintigraphy. It is caused by a defect in the VEGFR3 gene and mutations in the FOXC2 gene. http://www.mutdb.org/cgi-bin/GetGene.py?... It is believed that a third gene is involved. Scientists are still trying to identify this gene.
Avasthey-Roy Syndrome: Usually staring in the teen years, this condition presents as lymphedema with conjunction with arteriovenous (tangled web of veins and arteries) malformations and pulmonary hypertension. (A rare blood vessel disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels.
Cholestatis Syndrome This lymphedema-related condition includes obstruction of the bile flow from the liver. Can progress to hepatic cirrhosis (nodules in the liver combined with fibrosis, interfering with blood flow through the liver) and hepatitis (liver disease caused by a virus) with fibrosis (accumulation of connective tissue) of the portal tracts in the liver.
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