TRACING THE CAUSE OF PULMONARY HYPERTENSION IN HEREDITARY HEMORRHAGIC TELANGIECTASIA
نویسندگان
چکیده
منابع مشابه
Hereditary hemorrhagic telangiectasia: rare cause of pulmonary hypertension?
A 73-year-old woman was admitted to the emergency room with predominantly right-sided heart failure and anemia. Following clinical and imagiological evaluation, a diagnosis of pulmonary hypertension (PH) associated with Hereditary Hemorrhagic Telangiectasia (HHT) was confirmed. The initial response to bosentan plus sildenafil was good, including improvement in functional class and reduction of ...
متن کاملPulmonary arterial hypertension in a patient with hereditary hemorrhagic telangiectasia.
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by the triad of epistaxis, telangiectasia and vascular malformations. Pulmonary vascular complications associated with this disease include pulmonary arteriovenous malformations (AVM) and, less frequently, pulmonary hypertension (PH). We report the case of a patient who presented multiple pulmonary AVM a...
متن کاملThalidomide for Hereditary Hemorrhagic Telangiectasia With Pulmonary Arterial Hypertension.
initiate thalidomide, which increases platelet-derived growth factor-B expression and downregulates vascular endothelial growth factor in endothelial cells, stimulating mural cell coverage and leading to normal vascular maturation.1,2 After the initiation of thalidomide (50 mg daily), the anemia was dramatically improved without blood transfusion (Figure H) and telangiectatic lesions in the ton...
متن کاملHereditary Hemorrhagic Telangiectasia: Patient with Pulmonary Hypertension and Hepatic Encephalopathy
Copyright: © 2013 Wong CW. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Abstract Hereditary hemorrhagic telangiectasia (HHT) is usually under recognized. It is a rare genetic disorder characterized by telan...
متن کاملPulmonary arterial hypertension and portal hypertension in a patient with hereditary hemorrhagic telangiectasia.
BACKGROUND AND OBJECTIVE Pulmonary arterial hypertension (PAH) is a rare disease that could be inherited with an autosomal dominant pattern. Mutations in BMPR2 gene are described in over 70% of cases, although other genes are involved in lesser extend in PAH. Hereditary hemorrhagic telangiectasia (HHT) is another rare autosomal dominant disease. PAH is a rare complication of HHT that occurs in ...
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ژورنال
عنوان ژورنال: Journal of the American College of Cardiology
سال: 2017
ISSN: 0735-1097
DOI: 10.1016/s0735-1097(17)35797-2