LEADER 05759nam 2201441z- 450 001 9910557578003321 005 20220111 035 $a(CKB)5400000000043869 035 $a(oapen)https://directory.doabooks.org/handle/20.500.12854/76272 035 $a(oapen)doab76272 035 $a(EXLCZ)995400000000043869 100 $a20202201d2021 |y 0 101 0 $aeng 135 $aurmn|---annan 181 $ctxt$2rdacontent 182 $cc$2rdamedia 183 $acr$2rdacarrier 200 00$aHereditary Hemorrhagic Telangiectasia$eRecent Advances and Future Challenges 210 $aBasel, Switzerland$cMDPI - Multidisciplinary Digital Publishing Institute$d2021 215 $a1 online resource (228 p.) 311 08$a3-0365-0590-3 311 08$a3-0365-0591-1 330 $aHereditary hemorrhagic telangiectasia (HHT) is an inherited disease that affects the blood vessels, and is characterized by direct connections between arteries and veins with no intervening capillaries. These abnormal vessels may appear in the skin as tiny red dilated blood vessels in the mouth, lips, fingers and toes. The presence of these vascular lesions in the mucosa can lead to spontaneous and recurrent nose bleeding, typically beginning in mid-childhood, and this is the most common clinical manifestation of HHT, occurring in over 90% of patients. Gastrointestinal bleeding, derived from mucocutaneous vascular lesions, affects approximately 25% of patients, almost always presenting after the age of 50. Chronic nasal and gastrointestinal bleeding can cause iron-deficiency anemia, and current therapeutic strategies are trying to minimize iron and blood transfusions. HHT patients also present large vascular lesions, known as arteriovenous malformations, that occur in internal organs like lungs, liver, and brain, and may result in life-threatening complications often related to the shunting of blood. This book not only highlights the current knowledge regarding diagnosis and treatment of HHT, but also the newest insights in the molecular basis of HHT, the understanding of which is essential for the development of new medicines or therapeutic strategies. 517 $aHereditary Hemorrhagic Telangiectasia 606 $aResearch and information: general$2bicssc 610 $aactivin receptor-like kinase 1 (ALK1) 610 $aactivin-receptor-like kinase 1 610 $aACVRL1 610 $aALK1 610 $aangiogenesis 610 $aantiangiogenic properties 610 $aanticoagulants 610 $aantiplatelets 610 $aantithrombotic therapy 610 $aarteriovenous malformation 610 $aarteriovenous malformation (AVM) 610 $aarteriovenous malformations (AVMs) 610 $aAVM 610 $abazedoxifene 610 $abevacizumab 610 $abiomarker 610 $abiomarkers 610 $ableeding 610 $abone morphogenetic protein (BMP) 610 $acatheter based embolization therapy 610 $acell adhesion 610 $acerebral ischemic lesions 610 $acontrast enhanced magnetic resonance angiography 610 $aendoglin 610 $aEndoglin 610 $aendothelial cells 610 $aENG 610 $aepistaxis 610 $aepistaxis severity score 610 $aetamsylate 610 $aFK506 610 $agastrointestinal bleeding 610 $agenetic disease 610 $agenotype-phenotype correlation 610 $aguidelines 610 $ahereditary hemorrhagic 610 $ahereditary hemorrhagic telangiectasia 610 $aHereditary hemorrhagic telangiectasia 610 $aHereditary Hemorrhagic Telangiectasia 610 $ahereditary hemorrhagic telangiectasia (HHT) 610 $ahereditary hemorrhagic telangiectasia (HHT), second-hit 610 $ahereditary hemorrhagic telangiectasia/HHT/osler's disease 610 $aHHT 610 $ainflammation 610 $alife expectancy 610 $aliver 610 $along non-coding RNAs 610 $amechanical damage 610 $amicroRNA 610 $amicroRNAs 610 $aMRI 610 $aN-acetylcysteine 610 $anasal endoscopy 610 $anasal ointment 610 $anon-coding RNAs 610 $anosebleeds 610 $aOsler-Weber-Rendu 610 $apediatrics 610 $aplasma 610 $apropranolol 610 $apropranolol gel 610 $apulmonary arteriovenous malformation 610 $apulmonary arteriovenous malformations 610 $araloxifene 610 $arare disease 610 $arare diseases 610 $asafety 610 $ascreening 610 $ashear stress 610 $aSmad pathway 610 $aSmad4 610 $aSMAD4 610 $asomatic mutation 610 $asun-induced trauma 610 $asurvival 610 $atacrolimus 610 $atelangiectases 610 $atelangiectasia 610 $atelangiectasis 610 $atranexamic acid 610 $atranscatheter embolotherapy 610 $atransforming growth factor beta (TGF-?) 610 $atransforming growth factor-beta (TGF-?) 610 $aultrasound 610 $avascular endothelial growth factor (VEGF) 610 $avascular injury 610 $avascular malformations 615 7$aResearch and information: general 700 $aMager$b Hans-Jurgen$4edt$01299262 702 $aBernabeu$b Carmelo$4edt 702 $aPost$b Marco$4edt 702 $aMager$b Hans-Jurgen$4oth 702 $aBernabeu$b Carmelo$4oth 702 $aPost$b Marco$4oth 906 $aBOOK 912 $a9910557578003321 996 $aHereditary Hemorrhagic Telangiectasia$93025091 997 $aUNINA