Tuesday, May 10, 2016

Hereditary Hemorrhagic Telangiectasia

Hereditary Hemorrhagic Telangiectasia






This is a hereditary disorder in which certain abnormalities develop in the blood vessels to make them bleed. Normally, blood is pumped by the heart with great pressure so that it reaches the smaller blood vessels and capillaries.


But when the blood reaches the capillaries, its pressure gets reduced so that the blood returns to the heart through the veins. But in people with hereditary hemorrhagic telangiectasia, some arterial blood vessels flow into veins directly rather than into capillaries. When such abnormalities in the blood vessels is seen near the skin surface, they become red visibly, known as telangiectasia.










Types :


There are totally 3 types of telangiectasia. People with Type 1 and 2 will show symptoms early and type 2 and 3 will have liver abnormalities to large extent. Nosebleeds are quite common in all people with this disorder and it becomes serious when there is internal bleeding in liver or lungs or brain. In another type of Juvenile telangiectasia there will be a tendency for the growth of polyps in the gastrointestinal tract.


Symptoms and Causes :


Symptoms of this disorder vary with the type and organ affected. It is reported that one out of every 5,000 people are affected with telangiectasia. Since this disorder is hereditary, the only cause is chromosomes that are passed on to the offspring from the parent.


This disorder is also called Osler Weber Rendu syndrome when the blood vessels create abnormalities in arterio-venous vessels in many parts of the body and in case it affects the skin it is called telangiectasia.


Some of the common symptoms are bleeding of nose, port wine stain, Gastro-intestine bleeding and seizures or small Strokes.


Tests :


Your doctor would examine the physical condition for the symptoms and will concentrate more on the skin. Echocardiogram, endoscopy and blood test and genetic testing are done.


Pictures of Hereditary Hemorrhagic Telangiectasia :


Images, Pics, Pictures and Photos of Hereditary Hemorrhagic Telangiectasia


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Treatment :


If needed, surgery is done to stop bleeding in internal organs. For reducing nosebleeding, electrocautery procedure is done. For some patients, estrogen injection is given for reducing bleeding. In case of abnormal blood vessels, your doctor may perform endovascular embolization.


Otherwise, people with HHT can lead a normal life with close monitor on their blood vessels and other internal organs. If there is shortness of breath it can be due to heart failure or internal pressure in the lungs.











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