{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,4,1]],"date-time":"2022-04-01T22:42:00Z","timestamp":1648852920731},"reference-count":30,"publisher":"Georg Thieme Verlag KG","issue":"05","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Laryngorhinootologie"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>\n          Hintergrund\u2002Die heredit\u00e4re h\u00e4morrhagische Teleangiektasie (HHT) ist eine seltene, systemische Erbkrankheit, die durch mukokutane und viszerale Gef\u00e4\u00dfmalformationen gekennzeichnet ist. Ein interdisziplin\u00e4res Behandlungskonzept in HHT-Zentren wird empfohlen.<\/jats:p><jats:p>\n          Material und Methoden\u2002In einer retrospektiven Studie wurden Strukturen, Prozesse und die Daten aller Patienten analysiert, die von April 2014 bis August 2019 in unserem neu gegr\u00fcndeten HHT-Zentrum behandelt wurden.<\/jats:p><jats:p>\n          Ergebnisse\u2002Innerhalb des Universit\u00e4tsklinikums Essen wurde zun\u00e4chst ein Netzwerk von \u00fcber 20 verschiedenen Fachabteilungen zur Behandlung von Patienten mit HHT aufgebaut. Von den 282 Patienten, die sich seit der Zentrumsgr\u00fcndung mit m\u00f6glicher HHT im Westdeutschen Morbus-Osler-Zentrum vorstellten, wurde bei 261 Patienten (93\u200a%) die Diagnose als m\u00f6glich angesehen. Die meisten Patienten wiesen multiple Symptome auf (Epistaxis und\/oder Teleangiektasien: je\u00a0&gt;\u200a80\u200a%, viszerale Beteiligung: 65\u200a%), welche h\u00e4ufiger eine Behandlung im interdisziplin\u00e4ren Rahmen erforderten. \u00dcber 900 E-Mails fielen j\u00e4hrlich f\u00fcr die direkte Behandlung der Patienten mit HHT, deren Koordination, Kooperationen und \u00d6ffentlichkeitsarbeit an. Durch den internationalen Austausch innerhalb des Europ\u00e4ischen Referenznetzwerks f\u00fcr multisystemische vaskul\u00e4re Erkrankungen (VASCERN) ist eine Behandlung auf aktuellem Stand auch bei komplexen Fragestellungen gew\u00e4hrleistet.<\/jats:p><jats:p>\n          Schlussfolgerungen\u2002Ein HHT-Zentrum beinhaltet ein interdisziplin\u00e4res Netzwerk von medizinischen Fachrichtungen, bei denen die Abl\u00e4ufe durch Prozesse definiert und durch eine wirksame \u00d6ffentlichkeitsarbeit, Schulungs- und Ver\u00f6ffentlichungst\u00e4tigkeit gekennzeichnet sind.<\/jats:p>","DOI":"10.1055\/a-1402-0543","type":"journal-article","created":{"date-parts":[[2021,3,16]],"date-time":"2021-03-16T07:35:54Z","timestamp":1615880154000},"page":"372-381","source":"Crossref","is-referenced-by-count":0,"title":["Aufbau und Entwicklung eines interdisziplin\u00e4ren Zentrums f\u00fcr\u00a0heredit\u00e4re h\u00e4morrhagische Teleangiektasie"],"prefix":"10.1055","volume":"100","author":[{"given":"Freya","family":"Droege","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julia","family":"Dingemann","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kruthika","family":"Thangavelu","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Gie\u00dfen und Marburg \u2013 Standort\u00a0Marburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cornelius H. L.","family":"Kuerten","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philipp Marius","family":"Dahlfrancis","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christina","family":"Kaiser","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Friederike","family":"Kaster","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eleni","family":"Zioga","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Corinna","family":"Meyer","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolin","family":"Lueb","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ulrich","family":"Sure","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Neurochirurgie, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephan","family":"Lang","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Urban","family":"Geisthoff","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Universit\u00e4tsklinikum Gie\u00dfen und Marburg \u2013 Standort\u00a0Marburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2021,3,15]]},"reference":[{"key":"ref2","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3389\/fgene.2015.00001","article-title":"Hereditary hemorrhagic telangiectasia: genetics and molecular diagnostics in a new era","volume":"6","author":"J McDonald","year":"2015","journal-title":"Front Genet"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"3487","DOI":"10.1172\/jci39482","article-title":"Real-time imaging of de novo arteriovenous malformation in a mouse model of hereditary hemorrhagic telangiectasia","volume":"119","author":"S O Park","year":"2009","journal-title":"The Journal of clinical investigation"},{"key":"ref4","first-page":"3597","article-title":"[Hereditary hemorrhagic telangiectasia. 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