We report a case of severe, refractory gastrointestinal(GI) bleeding in a patient with hereditary hemorrhagic telangiectasia(HHT) whose massive transfusion dependence was lifted shortly after treatment with bevacizuma...We report a case of severe, refractory gastrointestinal(GI) bleeding in a patient with hereditary hemorrhagic telangiectasia(HHT) whose massive transfusion dependence was lifted shortly after treatment with bevacizumab, an anti-vascular endothelial growth factor. The patient's bleeding had been refractory to repeated endoscopic interventions, tranexamic acid, and tamoxifen. However, following treatment with bevacizumab at 5 mg/kg every other week, nearly 300 units of packed red blood cell transfusions were avoided in one year's time. Despite its relatively high cost, bevacizumab may have a more active role in the management of severe GI bleeding in HHT if such remarkable response can be consistently demonstrated.展开更多
Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disorder with variable expressivity. We present a 62-year-old patient with a rare, late-onset disease course featuring a novel mutation in ACVRL1, a...Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disorder with variable expressivity. We present a 62-year-old patient with a rare, late-onset disease course featuring a novel mutation in ACVRL1, a signal transducer in the TGFβ/BMP pathway.展开更多
Pulmonary arteriovenous malformations （AVM） lung is defined by an abnormal communication between pulmonary artery（ies） and vein（s） responsible for a right-left shunt. Congenital forms are most common and usually...Pulmonary arteriovenous malformations （AVM） lung is defined by an abnormal communication between pulmonary artery（ies） and vein（s） responsible for a right-left shunt. Congenital forms are most common and usually associated with Rendu-Osler disease （ROD）. Inversely, 15-45% of patients with ROD present lung AVM Nowadays, embolization is preferred to surgical resection in the majority of cases. Except for certain cases where surgery is indicated. This paper shows the role of surgery in AVMs.展开更多
文摘We report a case of severe, refractory gastrointestinal(GI) bleeding in a patient with hereditary hemorrhagic telangiectasia(HHT) whose massive transfusion dependence was lifted shortly after treatment with bevacizumab, an anti-vascular endothelial growth factor. The patient's bleeding had been refractory to repeated endoscopic interventions, tranexamic acid, and tamoxifen. However, following treatment with bevacizumab at 5 mg/kg every other week, nearly 300 units of packed red blood cell transfusions were avoided in one year's time. Despite its relatively high cost, bevacizumab may have a more active role in the management of severe GI bleeding in HHT if such remarkable response can be consistently demonstrated.
文摘Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disorder with variable expressivity. We present a 62-year-old patient with a rare, late-onset disease course featuring a novel mutation in ACVRL1, a signal transducer in the TGFβ/BMP pathway.
文摘Pulmonary arteriovenous malformations （AVM） lung is defined by an abnormal communication between pulmonary artery（ies） and vein（s） responsible for a right-left shunt. Congenital forms are most common and usually associated with Rendu-Osler disease （ROD）. Inversely, 15-45% of patients with ROD present lung AVM Nowadays, embolization is preferred to surgical resection in the majority of cases. Except for certain cases where surgery is indicated. This paper shows the role of surgery in AVMs.
