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Lesions lips, patient with hemorrhagic hereditary telangiectasia. Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu disease and Osler–Weber–Rendu syndrome, is a rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and often in organs such as the lungs, liver, and brain.
Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu syndrome) Ataxia–telangiectasia Sturge–Weber syndrome , a nevus formation in the skin supplied by the trigeminal nerve and associated with facial port-wine stains, glaucoma , meningeal angiomas and intellectual disabilities
Hereditary hemorrhagic telangiectasia is a condition where there is direct connection between arterioles and venules without intervening capillary beds, at the mucocutaneous region and internal bodily organs. Those who are affected by this conditions usually do not experience any symptoms.
CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder.The acronym "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.
Here are links to possibly useful sources of information about Hereditary hemorrhagic telangiectasia. PubMed provides review articles from the past five years (limit to free review articles) The TRIP database provides clinical publications about evidence-based medicine. Other potential sources include: Centre for Reviews and Dissemination and CDC
The outcomes of microvascular occlusions are usually influenced by the degree, length, nature of the underlying cause, and prompt and effective treatment. [ 3 ] References
Symptoms of AVMs vary according to their location. Most neurological AVMs produce few to no symptoms.Often the malformation is discovered as part of an autopsy or during treatment of an unrelated disorder (an "incidental finding"); in rare cases, its expansion or a micro-bleed from an AVM in the brain can cause epilepsy, neurological deficit, or pain.
The management of ATE depends on the location and severity of the ischemia and the underlying etiology. The main goals of ATE management are to restore blood flow, prevent further thrombosis, and treat the underlying cause. The treatment options for ATE include antithrombotic therapy, revascularization procedures, and risk factor modification.