The value of screening for multiple arterio-venous malformations in hereditary hemorrhagic telangiectasia: a diagnostic study
Occult visceral arterio-venous malformations (AVMs) may be a constant threat to patients suffering from hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome (M. ROW). HHT patients predominantly become symptomatic through chronic, recurrent epistaxis, a symptom that c...
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creator | FOLZ, Benedikt J WOLLSTEIN, Ana Cerra ALFKE, Heiko DÜNNE, Anja A LIPPERT, Burkard M GÖRG, Konrad WAGNER, Hans-Joachim BIEN, Siegfried WERNER, Jochen A |
description | Occult visceral arterio-venous malformations (AVMs) may be a constant threat to patients suffering from hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome (M. ROW). HHT patients predominantly become symptomatic through chronic, recurrent epistaxis, a symptom that can alert physicians at an early stage of the disease. The purpose of this study was to investigate whether occult, visceral arterio-venous malformations could be detected by screening imaging studies in patients suffering from HHT. In a comprehensive diagnostic study, Rendu-Osler-Weber patients were examined for potential visceral arterio-venous malformations by physical examination and non-invasive imaging techniques. The Department of Otolaryngology of the Philipps University of Marburg is a major referral center and coordinated the screening procedures. Thirty-five individuals with the presumed diagnosis of HHT gave informed consent to the screening investigations. Eighteen of 35 individuals were found to suffer from visceral vascular malformations; most of the AVMs were diagnosed in the lung, but also the liver, spleen, brain and eyes were affected. Six patients could be treated preventively by arterial embolization for AVMs of the lung, liver and brain. Comprehensive screening for occult AVMs in HHT patients seems to be justified to avert potential complications in this group of patients. |
doi_str_mv | 10.1007/s00405-003-0719-3 |
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ROW). HHT patients predominantly become symptomatic through chronic, recurrent epistaxis, a symptom that can alert physicians at an early stage of the disease. The purpose of this study was to investigate whether occult, visceral arterio-venous malformations could be detected by screening imaging studies in patients suffering from HHT. In a comprehensive diagnostic study, Rendu-Osler-Weber patients were examined for potential visceral arterio-venous malformations by physical examination and non-invasive imaging techniques. The Department of Otolaryngology of the Philipps University of Marburg is a major referral center and coordinated the screening procedures. Thirty-five individuals with the presumed diagnosis of HHT gave informed consent to the screening investigations. Eighteen of 35 individuals were found to suffer from visceral vascular malformations; most of the AVMs were diagnosed in the lung, but also the liver, spleen, brain and eyes were affected. Six patients could be treated preventively by arterial embolization for AVMs of the lung, liver and brain. Comprehensive screening for occult AVMs in HHT patients seems to be justified to avert potential complications in this group of patients.</description><identifier>ISSN: 0937-4477</identifier><identifier>EISSN: 1434-4726</identifier><identifier>DOI: 10.1007/s00405-003-0719-3</identifier><identifier>PMID: 14685882</identifier><language>eng</language><publisher>Berlin: Springer</publisher><subject>Adolescent ; Adult ; Age Distribution ; Aged ; Aged, 80 and over ; Arteriovenous Malformations - diagnosis ; Arteriovenous Malformations - epidemiology ; Biological and medical sciences ; Blood and lymphatic vessels ; Cardiology. Vascular system ; Cerebral Angiography - methods ; Cohort Studies ; Comorbidity ; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous ; Female ; Humans ; Incidence ; Magnetic Resonance Imaging ; Male ; Mass Screening - methods ; Medical sciences ; Middle Aged ; Non tumoral diseases ; Otorhinolaryngology. Stomatology ; Prognosis ; Risk Assessment ; Sensitivity and Specificity ; Severity of Illness Index ; Sex Distribution ; Survival Rate ; Telangiectasia, Hereditary Hemorrhagic - diagnosis ; Telangiectasia, Hereditary Hemorrhagic - epidemiology ; Tomography, X-Ray Computed ; Ultrasonography, Doppler, Color ; Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology</subject><ispartof>European archives of oto-rhino-laryngology, 2004-10, Vol.261 (9), p.509-516</ispartof><rights>2004 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c327t-e8c89a154cb85542223312bd6101e6099a47b3de638af4767ffe4fc99297605e3</citedby><cites>FETCH-LOGICAL-c327t-e8c89a154cb85542223312bd6101e6099a47b3de638af4767ffe4fc99297605e3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=16209473$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/14685882$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>FOLZ, Benedikt J</creatorcontrib><creatorcontrib>WOLLSTEIN, Ana Cerra</creatorcontrib><creatorcontrib>ALFKE, Heiko</creatorcontrib><creatorcontrib>DÜNNE, Anja A</creatorcontrib><creatorcontrib>LIPPERT, Burkard M</creatorcontrib><creatorcontrib>GÖRG, Konrad</creatorcontrib><creatorcontrib>WAGNER, Hans-Joachim</creatorcontrib><creatorcontrib>BIEN, Siegfried</creatorcontrib><creatorcontrib>WERNER, Jochen A</creatorcontrib><title>The value of screening for multiple arterio-venous malformations in hereditary hemorrhagic telangiectasia: a diagnostic study</title><title>European archives of oto-rhino-laryngology</title><addtitle>Eur Arch Otorhinolaryngol</addtitle><description>Occult visceral arterio-venous malformations (AVMs) may be a constant threat to patients suffering from hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome (M. ROW). HHT patients predominantly become symptomatic through chronic, recurrent epistaxis, a symptom that can alert physicians at an early stage of the disease. The purpose of this study was to investigate whether occult, visceral arterio-venous malformations could be detected by screening imaging studies in patients suffering from HHT. In a comprehensive diagnostic study, Rendu-Osler-Weber patients were examined for potential visceral arterio-venous malformations by physical examination and non-invasive imaging techniques. The Department of Otolaryngology of the Philipps University of Marburg is a major referral center and coordinated the screening procedures. Thirty-five individuals with the presumed diagnosis of HHT gave informed consent to the screening investigations. Eighteen of 35 individuals were found to suffer from visceral vascular malformations; most of the AVMs were diagnosed in the lung, but also the liver, spleen, brain and eyes were affected. Six patients could be treated preventively by arterial embolization for AVMs of the lung, liver and brain. Comprehensive screening for occult AVMs in HHT patients seems to be justified to avert potential complications in this group of patients.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Age Distribution</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Arteriovenous Malformations - diagnosis</subject><subject>Arteriovenous Malformations - epidemiology</subject><subject>Biological and medical sciences</subject><subject>Blood and lymphatic vessels</subject><subject>Cardiology. Vascular system</subject><subject>Cerebral Angiography - methods</subject><subject>Cohort Studies</subject><subject>Comorbidity</subject><subject>Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous</subject><subject>Female</subject><subject>Humans</subject><subject>Incidence</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Mass Screening - methods</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Non tumoral diseases</subject><subject>Otorhinolaryngology. Stomatology</subject><subject>Prognosis</subject><subject>Risk Assessment</subject><subject>Sensitivity and Specificity</subject><subject>Severity of Illness Index</subject><subject>Sex Distribution</subject><subject>Survival Rate</subject><subject>Telangiectasia, Hereditary Hemorrhagic - diagnosis</subject><subject>Telangiectasia, Hereditary Hemorrhagic - epidemiology</subject><subject>Tomography, X-Ray Computed</subject><subject>Ultrasonography, Doppler, Color</subject><subject>Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology</subject><issn>0937-4477</issn><issn>1434-4726</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2004</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpFkE1rHDEMhk1paLZpf0AuxZf25kT-GHucWwj9gkAu6dl4PZpdh5nx1vYEcuh_r8Mu5CSBHgm9DyGXHK44gLkuAAo6BiAZGG6ZfEc2XEnFlBH6PdmAlYYpZcw5-VjKEwB0ysoP5Jwr3Xd9Lzbk3-Me6bOfVqRppCVkxCUuOzqmTOd1qvEwIfW5Yo6JPeOS1kJnP7Xx7GtMS6FxoXvMOMTq80tr55Tz3u9ioBUnv-wihupL9DfU0yH63ZJKbcNS1-HlEzkb_VTw86lekD8_vj_e_WL3Dz9_393esyCFqQz70FvPOxW2fdcpIYSUXGwHzYGjBmu9Mls5oJa9H5XRZhxRjcFaYY2GDuUF-Xa8e8jp74qlujmWgFP7D1sip7U1neGigfwIhpxKyTi6Q45zC-Y4uFfn7ujcNefu1bmTbefL6fi6nXF42zhJbsDXE-BLaO6yX0Isb5wWYJWR8j99Hovg</recordid><startdate>20041001</startdate><enddate>20041001</enddate><creator>FOLZ, Benedikt J</creator><creator>WOLLSTEIN, Ana Cerra</creator><creator>ALFKE, Heiko</creator><creator>DÜNNE, Anja A</creator><creator>LIPPERT, Burkard M</creator><creator>GÖRG, Konrad</creator><creator>WAGNER, Hans-Joachim</creator><creator>BIEN, Siegfried</creator><creator>WERNER, Jochen A</creator><general>Springer</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20041001</creationdate><title>The value of screening for multiple arterio-venous malformations in hereditary hemorrhagic telangiectasia: a diagnostic study</title><author>FOLZ, Benedikt J ; WOLLSTEIN, Ana Cerra ; ALFKE, Heiko ; DÜNNE, Anja A ; LIPPERT, Burkard M ; GÖRG, Konrad ; WAGNER, Hans-Joachim ; BIEN, Siegfried ; WERNER, Jochen A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c327t-e8c89a154cb85542223312bd6101e6099a47b3de638af4767ffe4fc99297605e3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2004</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Age Distribution</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Arteriovenous Malformations - diagnosis</topic><topic>Arteriovenous Malformations - epidemiology</topic><topic>Biological and medical sciences</topic><topic>Blood and lymphatic vessels</topic><topic>Cardiology. Vascular system</topic><topic>Cerebral Angiography - methods</topic><topic>Cohort Studies</topic><topic>Comorbidity</topic><topic>Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous</topic><topic>Female</topic><topic>Humans</topic><topic>Incidence</topic><topic>Magnetic Resonance Imaging</topic><topic>Male</topic><topic>Mass Screening - methods</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Non tumoral diseases</topic><topic>Otorhinolaryngology. Stomatology</topic><topic>Prognosis</topic><topic>Risk Assessment</topic><topic>Sensitivity and Specificity</topic><topic>Severity of Illness Index</topic><topic>Sex Distribution</topic><topic>Survival Rate</topic><topic>Telangiectasia, Hereditary Hemorrhagic - diagnosis</topic><topic>Telangiectasia, Hereditary Hemorrhagic - epidemiology</topic><topic>Tomography, X-Ray Computed</topic><topic>Ultrasonography, Doppler, Color</topic><topic>Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>FOLZ, Benedikt J</creatorcontrib><creatorcontrib>WOLLSTEIN, Ana Cerra</creatorcontrib><creatorcontrib>ALFKE, Heiko</creatorcontrib><creatorcontrib>DÜNNE, Anja A</creatorcontrib><creatorcontrib>LIPPERT, Burkard M</creatorcontrib><creatorcontrib>GÖRG, Konrad</creatorcontrib><creatorcontrib>WAGNER, Hans-Joachim</creatorcontrib><creatorcontrib>BIEN, Siegfried</creatorcontrib><creatorcontrib>WERNER, Jochen A</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>European archives of oto-rhino-laryngology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>FOLZ, Benedikt J</au><au>WOLLSTEIN, Ana Cerra</au><au>ALFKE, Heiko</au><au>DÜNNE, Anja A</au><au>LIPPERT, Burkard M</au><au>GÖRG, Konrad</au><au>WAGNER, Hans-Joachim</au><au>BIEN, Siegfried</au><au>WERNER, Jochen A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The value of screening for multiple arterio-venous malformations in hereditary hemorrhagic telangiectasia: a diagnostic study</atitle><jtitle>European archives of oto-rhino-laryngology</jtitle><addtitle>Eur Arch Otorhinolaryngol</addtitle><date>2004-10-01</date><risdate>2004</risdate><volume>261</volume><issue>9</issue><spage>509</spage><epage>516</epage><pages>509-516</pages><issn>0937-4477</issn><eissn>1434-4726</eissn><abstract>Occult visceral arterio-venous malformations (AVMs) may be a constant threat to patients suffering from hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome (M. ROW). HHT patients predominantly become symptomatic through chronic, recurrent epistaxis, a symptom that can alert physicians at an early stage of the disease. The purpose of this study was to investigate whether occult, visceral arterio-venous malformations could be detected by screening imaging studies in patients suffering from HHT. In a comprehensive diagnostic study, Rendu-Osler-Weber patients were examined for potential visceral arterio-venous malformations by physical examination and non-invasive imaging techniques. The Department of Otolaryngology of the Philipps University of Marburg is a major referral center and coordinated the screening procedures. Thirty-five individuals with the presumed diagnosis of HHT gave informed consent to the screening investigations. Eighteen of 35 individuals were found to suffer from visceral vascular malformations; most of the AVMs were diagnosed in the lung, but also the liver, spleen, brain and eyes were affected. Six patients could be treated preventively by arterial embolization for AVMs of the lung, liver and brain. Comprehensive screening for occult AVMs in HHT patients seems to be justified to avert potential complications in this group of patients.</abstract><cop>Berlin</cop><pub>Springer</pub><pmid>14685882</pmid><doi>10.1007/s00405-003-0719-3</doi><tpages>8</tpages></addata></record> |
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subjects | Adolescent Adult Age Distribution Aged Aged, 80 and over Arteriovenous Malformations - diagnosis Arteriovenous Malformations - epidemiology Biological and medical sciences Blood and lymphatic vessels Cardiology. Vascular system Cerebral Angiography - methods Cohort Studies Comorbidity Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous Female Humans Incidence Magnetic Resonance Imaging Male Mass Screening - methods Medical sciences Middle Aged Non tumoral diseases Otorhinolaryngology. Stomatology Prognosis Risk Assessment Sensitivity and Specificity Severity of Illness Index Sex Distribution Survival Rate Telangiectasia, Hereditary Hemorrhagic - diagnosis Telangiectasia, Hereditary Hemorrhagic - epidemiology Tomography, X-Ray Computed Ultrasonography, Doppler, Color Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology |
title | The value of screening for multiple arterio-venous malformations in hereditary hemorrhagic telangiectasia: a diagnostic study |
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