Life expectancy and comorbidities in patients with hereditary hemorrhagic telangiectasia
There are only a few published studies that demonstrate associations between life expectancy, severe comorbidities, and their complications in patients with hereditary hemorrhagic telangiectasia (HHT). Relatives of 73 deceased patients with suspected HHT completed a questionnaire about causes of dea...
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| Veröffentlicht in: | Vascular medicine (London, England) England), 2018-08, Vol.23 (4), p.377-383 |
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| creator | Droege, Freya Thangavelu, Kruthika Stuck, Boris A Stang, Andreas Lang, Stephan Geisthoff, Urban |
| description | There are only a few published studies that demonstrate associations between life expectancy, severe comorbidities, and their complications in patients with hereditary hemorrhagic telangiectasia (HHT). Relatives of 73 deceased patients with suspected HHT completed a questionnaire about causes of death, and symptoms and comorbidities that the patients had developed. We compared the data for 55 cases where HHT had been clinically confirmed with the general population. Patients suffering from HHT lost, on average, 19 years (SD 11 years) of potential life compared to the general population. Among the deceased HHT patients, 35% (95% CI: 23–48%) died from sepsis, 26% (95% CI: 16–38%) from cardiac failure, 20% (95% CI: 9–28%) from a severe bleeding episode, and 13% (95% CI: 6–24%) from terminal cancer. Congestive heart failure (69%, 95% CI: 56–80%) and pulmonary hypertension (23%, 95% CI: 14–36%) were the main non-fatal comorbidities in patients with HHT. Patients with HHT appear to have a lower life expectancy than the general population. Sepsis and cardiac failure were the main causes of death. Optimized and targeted screening programs for the most frequent comorbidities followed by improved management of infectious complications may increase life expectancy. |
| doi_str_mv | 10.1177/1358863X18767761 |
| format | Article |
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Relatives of 73 deceased patients with suspected HHT completed a questionnaire about causes of death, and symptoms and comorbidities that the patients had developed. We compared the data for 55 cases where HHT had been clinically confirmed with the general population. Patients suffering from HHT lost, on average, 19 years (SD 11 years) of potential life compared to the general population. Among the deceased HHT patients, 35% (95% CI: 23–48%) died from sepsis, 26% (95% CI: 16–38%) from cardiac failure, 20% (95% CI: 9–28%) from a severe bleeding episode, and 13% (95% CI: 6–24%) from terminal cancer. Congestive heart failure (69%, 95% CI: 56–80%) and pulmonary hypertension (23%, 95% CI: 14–36%) were the main non-fatal comorbidities in patients with HHT. Patients with HHT appear to have a lower life expectancy than the general population. Sepsis and cardiac failure were the main causes of death. Optimized and targeted screening programs for the most frequent comorbidities followed by improved management of infectious complications may increase life expectancy.</description><identifier>ISSN: 1358-863X</identifier><identifier>EISSN: 1477-0377</identifier><identifier>DOI: 10.1177/1358863X18767761</identifier><identifier>PMID: 29781402</identifier><language>eng</language><publisher>London, England: SAGE Publications</publisher><subject>Adult ; Aged ; Aged, 80 and over ; Bleeding ; Cancer ; Cause of Death ; Comorbidity ; Complications ; Congestive heart failure ; Fatalities ; Female ; Heart ; Heart Failure - mortality ; Hemorrhage ; Hemorrhage - mortality ; Hereditary hemorrhagic telangiectasia ; Humans ; Hypertension ; Hypertension, Pulmonary - mortality ; Life Expectancy ; Life span ; Male ; Middle Aged ; Patients ; Risk Factors ; Sepsis ; Sepsis - mortality ; Surveys and Questionnaires ; Telangiectasia, Hereditary Hemorrhagic - diagnosis ; Telangiectasia, Hereditary Hemorrhagic - mortality</subject><ispartof>Vascular medicine (London, England), 2018-08, Vol.23 (4), p.377-383</ispartof><rights>The Author(s) 2018</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c407t-168426d07ba557da2775d247c667bd3cde4bc5aea16f9259c44668954c803a973</citedby><cites>FETCH-LOGICAL-c407t-168426d07ba557da2775d247c667bd3cde4bc5aea16f9259c44668954c803a973</cites><orcidid>0000-0001-8811-507X</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://journals.sagepub.com/doi/pdf/10.1177/1358863X18767761$$EPDF$$P50$$Gsage$$H</linktopdf><linktohtml>$$Uhttps://journals.sagepub.com/doi/10.1177/1358863X18767761$$EHTML$$P50$$Gsage$$H</linktohtml><link.rule.ids>314,776,780,21798,27901,27902,43597,43598</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29781402$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Droege, Freya</creatorcontrib><creatorcontrib>Thangavelu, Kruthika</creatorcontrib><creatorcontrib>Stuck, Boris A</creatorcontrib><creatorcontrib>Stang, Andreas</creatorcontrib><creatorcontrib>Lang, Stephan</creatorcontrib><creatorcontrib>Geisthoff, Urban</creatorcontrib><title>Life expectancy and comorbidities in patients with hereditary hemorrhagic telangiectasia</title><title>Vascular medicine (London, England)</title><addtitle>Vasc Med</addtitle><description>There are only a few published studies that demonstrate associations between life expectancy, severe comorbidities, and their complications in patients with hereditary hemorrhagic telangiectasia (HHT). Relatives of 73 deceased patients with suspected HHT completed a questionnaire about causes of death, and symptoms and comorbidities that the patients had developed. We compared the data for 55 cases where HHT had been clinically confirmed with the general population. Patients suffering from HHT lost, on average, 19 years (SD 11 years) of potential life compared to the general population. Among the deceased HHT patients, 35% (95% CI: 23–48%) died from sepsis, 26% (95% CI: 16–38%) from cardiac failure, 20% (95% CI: 9–28%) from a severe bleeding episode, and 13% (95% CI: 6–24%) from terminal cancer. Congestive heart failure (69%, 95% CI: 56–80%) and pulmonary hypertension (23%, 95% CI: 14–36%) were the main non-fatal comorbidities in patients with HHT. Patients with HHT appear to have a lower life expectancy than the general population. Sepsis and cardiac failure were the main causes of death. Optimized and targeted screening programs for the most frequent comorbidities followed by improved management of infectious complications may increase life expectancy.</description><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Bleeding</subject><subject>Cancer</subject><subject>Cause of Death</subject><subject>Comorbidity</subject><subject>Complications</subject><subject>Congestive heart failure</subject><subject>Fatalities</subject><subject>Female</subject><subject>Heart</subject><subject>Heart Failure - mortality</subject><subject>Hemorrhage</subject><subject>Hemorrhage - mortality</subject><subject>Hereditary hemorrhagic telangiectasia</subject><subject>Humans</subject><subject>Hypertension</subject><subject>Hypertension, Pulmonary - mortality</subject><subject>Life Expectancy</subject><subject>Life span</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Patients</subject><subject>Risk Factors</subject><subject>Sepsis</subject><subject>Sepsis - mortality</subject><subject>Surveys and Questionnaires</subject><subject>Telangiectasia, Hereditary Hemorrhagic - diagnosis</subject><subject>Telangiectasia, Hereditary Hemorrhagic - mortality</subject><issn>1358-863X</issn><issn>1477-0377</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kEtLAzEUhYMotlb3riTgxs1oksnkZpZSfEHBjUJ3QybJtCmdh8kM2n9vSqtCwVUOnO-e3HsQuqTkllKAO5pmUop0TiUIAEGP0JhygISkAMdRRzvZ-iN0FsKKEAIip6doxHKQlBM2RvOZqyy2X53VvWr0BqvGYN3WrS-dcb2zAbsGdyqqpg_40_VLvLTeRk_5TZSR9Eu1cBr3dq2ahdsGBafO0Uml1sFe7N8Jen98eJs-J7PXp5fp_SzRnECfUCE5E4ZAqbIMjGIAmWEctBBQmlQby0udKauoqHKW5ZpzIWSecS1JqnJIJ-hml9v59mOwoS9qF7Rdx11sO4SCEc5YKimkEb0-QFft4Ju4XaQAmJCC80iRHaV9G4K3VdF5V8djC0qKbevFYetx5GofPJS1Nb8DPzVHINkBQS3s36__Bn4DM3qJ8A</recordid><startdate>201808</startdate><enddate>201808</enddate><creator>Droege, Freya</creator><creator>Thangavelu, Kruthika</creator><creator>Stuck, Boris A</creator><creator>Stang, Andreas</creator><creator>Lang, Stephan</creator><creator>Geisthoff, Urban</creator><general>SAGE Publications</general><general>Sage Publications Ltd</general><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>8FD</scope><scope>FR3</scope><scope>K9.</scope><scope>M7Z</scope><scope>P64</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0001-8811-507X</orcidid></search><sort><creationdate>201808</creationdate><title>Life expectancy and comorbidities in patients with hereditary hemorrhagic telangiectasia</title><author>Droege, Freya ; Thangavelu, Kruthika ; Stuck, Boris A ; Stang, Andreas ; Lang, Stephan ; Geisthoff, Urban</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c407t-168426d07ba557da2775d247c667bd3cde4bc5aea16f9259c44668954c803a973</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Bleeding</topic><topic>Cancer</topic><topic>Cause of Death</topic><topic>Comorbidity</topic><topic>Complications</topic><topic>Congestive heart failure</topic><topic>Fatalities</topic><topic>Female</topic><topic>Heart</topic><topic>Heart Failure - mortality</topic><topic>Hemorrhage</topic><topic>Hemorrhage - mortality</topic><topic>Hereditary hemorrhagic telangiectasia</topic><topic>Humans</topic><topic>Hypertension</topic><topic>Hypertension, Pulmonary - mortality</topic><topic>Life Expectancy</topic><topic>Life span</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Patients</topic><topic>Risk Factors</topic><topic>Sepsis</topic><topic>Sepsis - mortality</topic><topic>Surveys and Questionnaires</topic><topic>Telangiectasia, Hereditary Hemorrhagic - diagnosis</topic><topic>Telangiectasia, Hereditary Hemorrhagic - mortality</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Droege, Freya</creatorcontrib><creatorcontrib>Thangavelu, Kruthika</creatorcontrib><creatorcontrib>Stuck, Boris A</creatorcontrib><creatorcontrib>Stang, Andreas</creatorcontrib><creatorcontrib>Lang, Stephan</creatorcontrib><creatorcontrib>Geisthoff, Urban</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Biochemistry Abstracts 1</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Vascular medicine (London, England)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Droege, Freya</au><au>Thangavelu, Kruthika</au><au>Stuck, Boris A</au><au>Stang, Andreas</au><au>Lang, Stephan</au><au>Geisthoff, Urban</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Life expectancy and comorbidities in patients with hereditary hemorrhagic telangiectasia</atitle><jtitle>Vascular medicine (London, England)</jtitle><addtitle>Vasc Med</addtitle><date>2018-08</date><risdate>2018</risdate><volume>23</volume><issue>4</issue><spage>377</spage><epage>383</epage><pages>377-383</pages><issn>1358-863X</issn><eissn>1477-0377</eissn><abstract>There are only a few published studies that demonstrate associations between life expectancy, severe comorbidities, and their complications in patients with hereditary hemorrhagic telangiectasia (HHT). Relatives of 73 deceased patients with suspected HHT completed a questionnaire about causes of death, and symptoms and comorbidities that the patients had developed. We compared the data for 55 cases where HHT had been clinically confirmed with the general population. Patients suffering from HHT lost, on average, 19 years (SD 11 years) of potential life compared to the general population. Among the deceased HHT patients, 35% (95% CI: 23–48%) died from sepsis, 26% (95% CI: 16–38%) from cardiac failure, 20% (95% CI: 9–28%) from a severe bleeding episode, and 13% (95% CI: 6–24%) from terminal cancer. Congestive heart failure (69%, 95% CI: 56–80%) and pulmonary hypertension (23%, 95% CI: 14–36%) were the main non-fatal comorbidities in patients with HHT. Patients with HHT appear to have a lower life expectancy than the general population. Sepsis and cardiac failure were the main causes of death. Optimized and targeted screening programs for the most frequent comorbidities followed by improved management of infectious complications may increase life expectancy.</abstract><cop>London, England</cop><pub>SAGE Publications</pub><pmid>29781402</pmid><doi>10.1177/1358863X18767761</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0001-8811-507X</orcidid><oa>free_for_read</oa></addata></record> |
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| subjects | Adult Aged Aged, 80 and over Bleeding Cancer Cause of Death Comorbidity Complications Congestive heart failure Fatalities Female Heart Heart Failure - mortality Hemorrhage Hemorrhage - mortality Hereditary hemorrhagic telangiectasia Humans Hypertension Hypertension, Pulmonary - mortality Life Expectancy Life span Male Middle Aged Patients Risk Factors Sepsis Sepsis - mortality Surveys and Questionnaires Telangiectasia, Hereditary Hemorrhagic - diagnosis Telangiectasia, Hereditary Hemorrhagic - mortality |
| title | Life expectancy and comorbidities in patients with hereditary hemorrhagic telangiectasia |
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