Introduction
Hereditary hemorrhagic telangiectasiaAn autosomal dominant hereditary disease characterized by multiple capillaries of the skin, mucous membranes, and internal organs, or small arteries, venules, and repeated bleeding in the lesion. The liver is often affected.
Cause
The disease is an autosomal dominant hereditary disease, which can be inherited by both men and women. The lesion is located in the vessel wall and is characterized by telangiectasia, arteriovenous malformations, and aneurysms. Thinning of the vessel wall, lack of elastic fibers, lack of smooth muscle, capillary wall and small arterial wall consisting of only one layer of endothelial cells, vascular tortuosity or dilatation, sometimes only endothelial cells undergo degenerative changes, endothelial cell junction defects, diseased vessels It can be ruptured and bleeding due to slight external force or intravascular blood flow pressure.
symptom
[clinical manifestations]
More than 20 to 30 years old, some can occur in childhood.
The most prominent symptom is bleeding in the affected blood vessels, often repeated bleeding in the same site. In childhood, the nose is more common, and the sinus gradually improves in adolescence, while the chance of visceral bleeding increases. The most common gastrointestinal bleeding is hemorrhage, hematuria, fundus hemorrhage, menorrhagia, subarachnoid hemorrhage, etc. .
Hepatic involvement, hepatomegaly due to increased blood flow through the arteriovenous fistula, liver pain and a certain degree of tenderness, sometimes a pulsating mass can be touched, tremor can be touched, and continuity can be heard. Vascular noise.
The shunt of arteriovenous fistula can produce a high dynamic circulation state, and can produce high-discharge congestive heart failure, which can cause hypoxemia and secondary polycythemia due to arteriovenous fistula of the lung. Chronic blood loss or frequent and massive bleeding can cause iron deficiency anemia.
【diagnosis】
Positive family history, telangiectasia, and repeated bleeding at the same site. Due to the fragility of the vessel wall, the clinical beam arm test is often positive and has a prolonged bleeding time. Angiography has a diagnostic value.
diagnosis
Note that it must be differentiated from the following diseases:
1. Spider mites;
2, red dragonfly;
3. Vein expansion.
complication
A common complication of the disease is anemia.
treatment
[Treatment measures]
1. Hemostatic surface hemorrhage is mainly caused by compression and hemostasis. The visceral hemorrhage considers the use of Anluo blood to help small blood vessels to contract, and the vasopressin is used to reduce the pressure inside the visceral blood vessels.
2. Blood transfusion is only used for a large number of blood loss, but it should not be excessive, avoiding high blood pressure and making bleeding difficult.
3. Iron supplementation is suitable for patients with chronic hemorrhagic anemia.
4. Other hepatic artery embolization can be used to treat hepatic arteriovenous fistula. Beta-blockers improve high-powered circulation, reduce hepatic blood flow, and reduce fractional flow.
prevention
1. Pay attention to protect mucous membrane skin, avoid local trauma, excessive fatigue, cold, fever, and factors that can induce blood pressure and blood volume increase.
2. Care should also be taken when removing teeth and performing minor operations to avoid excessive bleeding.
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