نتایج جستجو برای: hereditary hemorrhagic telangiectasia
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Introduction Hereditary hemorrhagic telangiectasia (HHT, M. Osler) is an autosomal-dominant hereditary disease that can lead to the development of arteriovenous malformation and skin mucous membranes. The leading symptom epistaxis, severity which vary greatly from patient patient. Therapy only symptomatically oriented. In addition surgical medicinal therapy approaches, adequate care nasal mucos...
We describe a report of an elderly lady who was hospitalized with progressive worsening of breathlessness and fatigue of one month's duration. Clinical evaluation of the patient revealed hereditary hemorrhagic telangiectasia, interstitial lung disease, pulmonary hypertension without left heart failure, and bilateral gluteal calcinosis cutis. Initially, CREST (calcinosis, Raynaud's phenomenon, e...
Hereditary hemorrhagic telangiectasia is a rare autosomal dominant inherited disease that is usually complicated by visceral vascular malformations. Patients harboring such malformations are at increased risk of brain abscess formation, which despite advances in diagnostic and surgical methods remains a life threatening medical emergency with high mortality and morbidity rates. In the present r...
This is the first scientific report of hereditary hemorrhagic telangiectasia (HHT) epistaxis treatment by intranasal spraying of the vascular endothelial growth factor (VEGF) inhibitor bevacizumab (Avastin). Epistaxis in patients with HHT is a morbid, mortal condition that is difficult and unpleasant to manage. Nasal telangiectasia growth is modulated by VEGF, which is elevated in HHT patients....
BACKGROUND Hereditary hemorrhagic telangiectasia is characterized by angiodysplastic lesions. So far, knowledge is limited on the vascular architecture and rate of occult manifestation of telangiectases. Contact endoscopy has not been used for this task before. METHODS Eleven patients with hereditary hemorrhagic telangiectasia were examined with contact endoscopy to identify occult telangiect...
CASE DESCRIPTION Five-year-old female patient with hereditary hemorrhagic telangiectasia. CLINICAL FINDINGS Deterioration of cardiopulmonary function with higher oxygen requirements secondary to pulmonary arteriovenous shunts, epistaxis. TREATMENT AND OUTCOME The patient was treated with the monoclonal antibody bevacizumab, which inhibits the vascular endothelial growth factor, with good cl...
Hereditary hemorrhagic telangiectasia (HHT) is an inherited disorder that can lead to frequent and severe sequelae. Although the condition has no cure, many of its physical symptoms can be managed to improve the quality of life and lower the risk of life-threatening complications. This article discusses HHT presentation, genetic screening, diagnosis, and management. The management of HHT can in...
BACKGROUND AND PURPOSE Brain AVM, cerebral abscess, and ischemic stroke are among the well known neurologic manifestations of hereditary hemorrhagic telangiectasia. However, recently reported data suggest an additional association with malformations of cortical development. The purpose of this study was to determine the prevalence of malformations of cortical development in a population of pedi...
Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu syndrome is an inherited disorder characterized by vascular dysplasias leading to hemorrhages. If affects approximately 1 in 10,000 Caucasian people. The most common presentation chronic and recurrent epistaxis whereas bleeding from other sites can lead life-threatening complications.
BACKGROUND Hereditary hemorrhagic telangiectasia is a rare autosomal dominant inherited disease characterized by vascular dysplasia. To the best of our knowledge, we report the first case in the literature of definite hereditary hemorrhagic telangiectasia diagnosed in western Kenya, a resource-limited setting with limited treatment options. CASE PRESENTATION A 60-year-old black Kenyan woman w...
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