Hereditary Hemorrhagic Telangiectasia (HHT) in Akita Prefecture, Japan.
نویسندگان
چکیده
منابع مشابه
A mouse model for hereditary hemorrhagic telangiectasia (HHT) type 2.
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal-dominant disorder characterized by the age-dependent development of focal arteriovenous malformations and telangiectases. HHT type 2 is caused by loss of function mutations in activin receptor-like kinase 1 (ACVRL1 or ALK1). However, the factors that initiate lesion formation and those that influence disease progression remain unknown....
متن کاملHereditary hemorrhagic telangiectasia/avastin.
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....
متن کاملHereditary Hemorrhagic Telangiectasia Management.
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...
متن کاملIntra-venous bevacizumab in hereditary hemorrhagic telangiectasia (HHT): A retrospective study of 46 patients
BACKGROUND Bevacizumab, an anti-VEGF monoclonal antibody, has recently emerged as a new option for severe forms of hereditary hemorrhagic telangiectasia (HHT). Its utilization in this orphan disease has rapidly spread despite the lack of randomized trials and international guidelines. The objective of this study is to report the main clinical data (baseline characteristics, dose schedule, effic...
متن کاملHereditary Hemorrhagic Telangiectasia (HHT, Osler–Weber–Rendu disease) — Symptoms and Diagnosis
Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu-Disease (OWRD) is a rare autosomal dominant disorder that affects blood vessels and consequently multiple systems resulting in a tendency to bleed. Also known as vascular dysplasia, the condition is more or less diagnosed clinically and has a variable prognosis depending on prompt recognition and severity. However, there is no cur...
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ژورنال
عنوان ژورنال: Internal Medicine
سال: 2000
ISSN: 0918-2918,1349-7235
DOI: 10.2169/internalmedicine.39.675