01442nas 2200493-a 450 99633575600331620221208213020.01945-1555(OCoLC)46762163(CKB)110978979784237(CONSER)--2008214697(EXLCZ)9911097897978423720010417a19939999 s-- aengurcnu||||||||txtrdacontentcrdamediacrrdacarrierCritical care alertAtlanta, GA American Health ConsultantsRefereed/Peer-reviewed1067-9502 Crit. care alertIntensive care nursingPeriodicalsCritical care medicinePeriodicalsCritical CareNursing CareCritical care medicinefast(OCoLC)fst00883647Intensive care nursingfast(OCoLC)fst00975924Periodicals.fastNursingIntensive care nursingCritical care medicineCritical Care.Nursing Care.Critical care medicine.Intensive care nursing.362American Health Consultants.Health Reference Center.JOURNAL996335756003316Critical care alert2183853UNISA05759nam 2201441z- 450 991055757800332120220111(CKB)5400000000043869(oapen)https://directory.doabooks.org/handle/20.500.12854/76272(oapen)doab76272(EXLCZ)99540000000004386920202201d2021 |y 0engurmn|---annantxtrdacontentcrdamediacrrdacarrierHereditary Hemorrhagic TelangiectasiaRecent Advances and Future ChallengesBasel, SwitzerlandMDPI - Multidisciplinary Digital Publishing Institute20211 online resource (228 p.)3-0365-0590-3 3-0365-0591-1 Hereditary 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.Hereditary Hemorrhagic Telangiectasia Research and information: generalbicsscactivin receptor-like kinase 1 (ALK1)activin-receptor-like kinase 1ACVRL1ALK1angiogenesisantiangiogenic propertiesanticoagulantsantiplateletsantithrombotic therapyarteriovenous malformationarteriovenous malformation (AVM)arteriovenous malformations (AVMs)AVMbazedoxifenebevacizumabbiomarkerbiomarkersbleedingbone morphogenetic protein (BMP)catheter based embolization therapycell adhesioncerebral ischemic lesionscontrast enhanced magnetic resonance angiographyendoglinEndoglinendothelial cellsENGepistaxisepistaxis severity scoreetamsylateFK506gastrointestinal bleedinggenetic diseasegenotype-phenotype correlationguidelineshereditary hemorrhagichereditary hemorrhagic telangiectasiaHereditary hemorrhagic telangiectasiaHereditary Hemorrhagic Telangiectasiahereditary hemorrhagic telangiectasia (HHT)hereditary hemorrhagic telangiectasia (HHT), second-hithereditary hemorrhagic telangiectasia/HHT/osler's diseaseHHTinflammationlife expectancyliverlong non-coding RNAsmechanical damagemicroRNAmicroRNAsMRIN-acetylcysteinenasal endoscopynasal ointmentnon-coding RNAsnosebleedsOsler-Weber-Rendupediatricsplasmapropranololpropranolol gelpulmonary arteriovenous malformationpulmonary arteriovenous malformationsraloxifenerare diseaserare diseasessafetyscreeningshear stressSmad pathwaySmad4SMAD4somatic mutationsun-induced traumasurvivaltacrolimustelangiectasestelangiectasiatelangiectasistranexamic acidtranscatheter embolotherapytransforming growth factor beta (TGF-β)transforming growth factor-beta (TGF-β)ultrasoundvascular endothelial growth factor (VEGF)vascular injuryvascular malformationsResearch and information: generalMager Hans-Jurgenedt1299262Bernabeu CarmeloedtPost MarcoedtMager Hans-JurgenothBernabeu CarmeloothPost MarcoothBOOK9910557578003321Hereditary Hemorrhagic Telangiectasia3025091UNINA