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Hereditary Hemorrhagic Telangiectasia - Symptom, Causes, Treatment


Hereditary hemorrhagic telangiectasia is characterized by the presence of multiple arteriovenous malformations that lack intervening capillaries and result in direct connections between arteries and veins. Too much clotting can block blood vessels that are not bleeding consequently, the body has control mechanisms to limit clotting and dissolve clots that are no longer needed. Hereditary hemorrhagic telangiectasia is a condition characterized by abnormal blood vessels which are delicate and prone to bleeding. A child with HHT, also known as Osler-Weber-Rendu Syndrome, tends to form blood vessels that lack the capillaries between an artery and vein. It is usually referred to as the Osler-Weber-Rendu disorder, or simply the OWR. It is a rare inherited disorder that affects blood vessels throughout the body and is characterised by a tendency for bleeding in particular recurrent epistaxis, and skin telangiectasia.

Hereditary haemorrhagic telangiectasia is also known as Osler-Rendu-Weber syndrome. Telangiectasias are abnormally dilated blood vessels associated with a number of diseases. In this condition, without capillaries, arterial blood under high pressure flows directly into a vein. When clotting is uncontrolled, small blood vessels in critical places can become clogged with clots. Large AVMs often cause symptoms when they occur in the brain, lungs, or gastrointestinal tract complications from bleeding or shunting may be sudden and catastrophic.

Causes of Hereditary Hemorrhagic Telangiectasia

Common causes and risk factor's of Hereditary Hemorrhagic Telangiectasia include the following :

  • Hereditary hemorrhagic telangiectasia.
  • Ataxia - telangiectasia.
  • Spider angioma.
  • Cutis marmorata telangiectatica congenita.
  • Bloom syndrome.
  • Klippel-Trenaunay-Weber syndrome.
  • Sturge-Weber disease.
  • Xeroderma pigmentosa.
  • Nevus flammeus such as port-wine stain.

Symptoms of Hereditary Hemorrhagic Telangiectasia

Some of the common sign and symptoms of the disease Hereditary Hemorrhagic Telangiectasia as follows:

  • Anxiety.
  • Lower back pain.
  • Fatigue.
  • Fever.

Treatment of Hereditary Hemorrhagic Telangiectasia

Find effective treatment methods of treating Hereditary Hemorrhagic Telangiectasia :

  • Life-threatening GI bleeds can be effectively treated by segmental bowel resection.
  • Embolization, ligation, or surgical excision is indicated for enlarging or symptomatic pulmonary AV fistulas or for intrahepatic fistulas that cause high-output cardiac failure.
  • Pulsed dye laser treatment can be used to photocoagulate telangiectases in the nasal mucosa.
  • Radiosurgery can be used to treat smaller cerebral AVMs.
  • Humidification and the daily application of nasal lubricants by the individual may be helpful

 

 

 

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