Lang-Over syndrome
Introduction
Introduction to Lang-Ove Syndrome Renda-Osler-Weber Syndrome is also called Herediatary Hemorrhagic Telangiectasin, Babington disease, Goldstein syndrome, a hereditary disease caused by abnormal development of the blood vessel wall. Disease, the typical lesion is the appearance of bright red or purple red capillaries or small blood vessels in the skin and mucous membranes, which causes skin and mucous membrane bleeding, which occurs in the mucosa of the digestive tract and causes bleeding in the digestive tract. basic knowledge The proportion of illness: the incidence rate is about 0.004% - 0.005% Susceptible people: good for young people Mode of infection: non-infectious Complications: AIDS
Cause
Cause of Lang-Aowei syndrome
The disease belongs to autosomal dominant inheritance. The basic pathological changes are capillaries of the skin and mucous membranes. The small arteries and small veins are thinned. Some parts are composed of only one layer of vascular endothelial cells, and there is no elastic connective tissue support around. As a result, the blood vessels are twisted and expanded, and hemangioma can be formed. The skin and mucous membranes of the whole body can be affected, and the visceral mucosal lesions are more common in the digestive tract.
Prevention
Lang-Ove Syndrome Prevention
There is no effective prevention method for this disease. Early detection and early treatment are the key to prevention.
Complication
Lang-Ove syndrome complications Complications AIDS
Lung and biliary system infections, AIDS.
Symptom
Lang-Ove syndrome symptoms common symptoms nodular bleeding tendency abdominal pain black stool hemoptysis repeated bleeding visceral bleeding gum bleeding hematuria
The disease occurs in young and middle-aged, both men and women can be onset, the typical lesions are bright red or purple red capillaries or small blood vessels in the skin and mucous membranes, diameter 1 ~ 3mm, lesions are needle-like, small nodular, Bulk or hemangioma shape, color disappears after pressurization, lesions often occur in the hands, feet, face, lips, mouth, nasal cavity and digestive tract, etc., dilated blood vessels can occur spontaneous bleeding or minor traumatic bleeding, often Repeated bleeding for the same part, nasal discharge, bleeding gum is a common symptom, visceral bleeding is more common in the digestive tract, manifested as recurrent black stool hematemesis, may have abdominal pain, clinically often misdiagnosed as ulcer disease or other reasons The resulting bleeding, a small number of hemoptysis, hematuria, menorrhagia and other organs bleeding tendency, bleeding can cause hemorrhagic anemia.
Examine
Lang-Ove syndrome examination
1. There is often no positive detection in laboratory tests, platelet count, normal coagulation function, prolongation of prothrombin time if there is a lack of hereditary coagulation factors.
2. A wrinkle microcirculation examination can reveal the tortuous expansion of the capillaries.
3. Patients with gastrointestinal bleeding, no positive findings in the gastrointestinal X-ray examination.
4. Gastroscopy or fiber colonoscopy can reveal dilated hemangioma lesions in the digestive tract mucosa.
Diagnosis
Diagnosis and diagnosis of Lang-Ove syndrome
diagnosis
In particular, unexplained recurrent gastrointestinal bleeding, combined with genetic history, should consider the possibility of the disease, and carry out relevant endoscopy to help confirm the diagnosis.
Differential diagnosis
Note the differentiation from coagulopathy.
