Pericytes as targets in hereditary hemorrhagic telangiectasia
نویسندگان
چکیده
Defective paracrine Transforming Growth Factor-β (TGF-β) signaling between endothelial cells and the neighboring mural cells have been thought to lead to the development of vascular lesions that are characteristic of Hereditary Hemorrhagic Telangiectasia (HHT). This review highlights recent progress in our understanding of TGF-β signaling in mural cell recruitment and vessel stabilization and how perturbed TGF-β signaling might contribute to defective endothelial-mural cell interaction affecting vessel functionalities. Our recent findings have provided exciting insights into the role of thalidomide, a drug that reduces both the frequency and the duration of epistaxis in individuals with HHT by targeting mural cells. These advances provide opportunities for the development of new therapies for vascular malformations.
منابع مشابه
Hereditary benign Telangiectasia: Report of 6 cases in a family
Hereditary benign telangiectasia is an uncommon idiopathic skin disorder characterized by generalized telangiectases and angiomatous lesions of the skin. The diagnosis should be suspected in patients with such cutaneous lesions, positive family history, no associated bleeding problems, and no mucosal involvement. We present six cases of hereditary benign telangiectasia in three generations of o...
متن کاملAnesthetic considerations for the patient with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome).
Hereditary hemorrhagic telangiectasia (HHT), Osler-Weber-Rendu Syndrome, is an uncommon disease but may be present in many people who remain undiagnosed. It is an autosomal dominant disorder characterized by multiple arteriovenous malformations (AVMs) and telangiectases that affect multiple organ systems. Hereditary hemorrhagic telangiectasia patients have a propensity for bleeding, especially ...
متن کاملNeurovascular manifestations in hereditary hemorrhagic telangiectasia: imaging features and genotype-phenotype correlations.
BACKGROUND AND PURPOSE Hereditary hemorrhagic telangiectasia is an autosomal dominant disease that presents in 10%-20% of patients with various brain vascular malformations. We aimed to report the radiologic features (phenotype) and the genotype-phenotype correlations of brain vascular malformations in hereditary hemorrhagic telangiectasia. MATERIALS AND METHODS Demographic, clinical, genotyp...
متن کاملEndovascular treatment of epistaxis in patients with hereditary hemorrhagic telangiectasia.
BACKGROUND AND PURPOSE The treatment of epistaxis in patients with hereditary hemorrhagic telangiectasia can be very challenging. The purpose of our study was to evaluate our experience with endovascular epistaxis embolization in patients with hemorrhagic hereditary telangiectasia and to compare this with our experience in patients treated for idiopathic epistaxis. MATERIALS AND METHODS Over ...
متن کاملHereditary hemorrhagic telangiectasia patient presenting with brain abscess due to silent pulmonary arteriovenous malformation
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...
متن کامل