gms | German Medical Science

87. Jahresversammlung der Deutschen Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e. V.

Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e. V.

04.05. - 07.05.2016, Düsseldorf

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Complications and causes of death in patients with hereditary haemorrhagic telangiectasia (HHT)

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  • corresponding author Freya Droege - Klinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie, Universitätsklinikum Essen, Essen, Germany
  • author Stephan Lang - Klinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie, Universitätsklinikum Essen, Essen, Germany
  • author Urban Geisthoff - Klinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie, Universitätsklinikum Essen, Essen, Germany

German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. 87th Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. Düsseldorf, 04.-07.05.2016. Düsseldorf: German Medical Science GMS Publishing House; 2016. Doc16hno27

doi: 10.3205/16hno27, urn:nbn:de:0183-16hno278

Veröffentlicht: 7. September 2016

© 2016 Droege et al.
Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). Lizenz-Angaben siehe http://creativecommons.org/licenses/by/4.0/.

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Text

Background: Few studies reported a reduced life expectancy in patients with hereditary haemorrhagic telangiectasia (HHT) due to severe comorbidities as well as complications [1], [2], [3], [4].

Methods: A total of 70 relatives of deceased patients with diagnosed hereditary haemorrhagic telangiectasia (HHT) completed a questionnaire about causes of death, symptoms and comorbidities patients developed. The data was compared to the general population.

Results: The mean age at death in patients with hereditary haemorrhagic telangiectasia (HHT) was lower than in the general population (64.0 years vs. 80.3 years respectively, T-Test: 34.9, p < 0.000). In many cases arteriovenous malformations led to severe comorbidities or even death of the patients. Unexpectedly the main causes of death were cardiac failure and infectious diseases causing a septic shock. Surprisingly just 11.5% of the patients with HHT died from a severe bleeding such as epistaxis and gastrointestinal bleeding.

Discussion: Patients with hereditary haemorrhagic telangiectasia (HHT) seem to have a lower life expectancy than the general population. Diagnosing patients at an early stage could prevent comorbidities and improve survival rates.

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References

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2.
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