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2篇 您的检索式:作者名="Sebastiaan Velthuis"
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1Pulmonary hypertension in hereditary haemorrhagic telangiectasia显示文摘Hereditary haemorrhagic telangiectasia(HHT) is an autosomal dominant inherited disorder characterised by vascular malformations in predominantly the brain,liverand lungs.Pulmonary hypertension(PH) is increasingly recognised as a severe complication of HHT.PH may be categorised into two distinct types in patients with HHT.Post-capillary PH most often results from a high pulmonary blood flow that accompanies the high cardiac output state associated with liver arteriovenous malformations.Less frequently,the HHT-related gene mutations in ENG or ACVRL1 appear to predispose patients with HHT to develop pre-capillary pulmonary arterial hypertension.Differentiation between both forms of PH by right heart catheterisation is essential,since both entities are associated with severe morbidity and mortality with different treatment options.Therefore all HHT patients should be referred to an HHT centre.Veronique MM Vorselaars Sebastiaan Velthuis Repke J Snijder Jan Albert Vos Johannes J Mager Martijn C Post 2015World Journal of Cardiology2015,7,5:4
2Hereditary hemorrhagic telangiectasia: How accurate are the clinical criteria?显示文摘Marco W.F. van Gent Sebastiaan Velthuis Martijn C. Post Repke J. Snijder Cornelis J.J. Westermann Tom G.W. Letteboer Johannes J. Mager 2013Am J Med Genet2013,,3:1
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