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European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)

Hereditary haemorrhagic telangiectasia (HHT) is a multisystemic vascular dysplasia that leads to nosebleeds, anaemia due to blood loss, and arteriovenous malformations (AVMs) in organs such as the lungs, liver and brain. HHT is estimated to affect 85,000 European citizens, but most health care provi...

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Autores principales: Shovlin, Claire L., Buscarini, Elisabetta, Kjeldsen, Anette D., Mager, Hans Jurgen, Sabba, Carlo, Droege, Freya, Geisthoff, Urban, Ugolini, Sara, Dupuis-Girod, Sophie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6094583/
https://www.ncbi.nlm.nih.gov/pubmed/30111344
http://dx.doi.org/10.1186/s13023-018-0850-2
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author Shovlin, Claire L.
Buscarini, Elisabetta
Kjeldsen, Anette D.
Mager, Hans Jurgen
Sabba, Carlo
Droege, Freya
Geisthoff, Urban
Ugolini, Sara
Dupuis-Girod, Sophie
author_facet Shovlin, Claire L.
Buscarini, Elisabetta
Kjeldsen, Anette D.
Mager, Hans Jurgen
Sabba, Carlo
Droege, Freya
Geisthoff, Urban
Ugolini, Sara
Dupuis-Girod, Sophie
author_sort Shovlin, Claire L.
collection PubMed
description Hereditary haemorrhagic telangiectasia (HHT) is a multisystemic vascular dysplasia that leads to nosebleeds, anaemia due to blood loss, and arteriovenous malformations (AVMs) in organs such as the lungs, liver and brain. HHT is estimated to affect 85,000 European citizens, but most health care providers have limited prior HHT exposure or training. Outcome Measures were developed and implemented by the HHT Working Group of the European Reference Network for Rare Vascular Diseases (VASCERN), in order to maximise the number of patients receiving good care. The measures specifically target areas where optimal management reduces morbidity and mortality in HHT patients, and were designed to be robust to emerging new evidence. Thresholds are the percentage of patients in particular settings who have been recommended screening, or provided with written advice. The 5 Outcome Measures cover (1) pulmonary AVM screening; (2) written nosebleed advice, (3) assessment of iron deficiency; (4) antibiotic prophylaxis prior to dental and surgical procedures for patients with pulmonary AVMs, and (5) written advice on pregnancy. They are not a blueprint for detailed HHT management, but are suitable for all clinicians to be aware of and implement. In summary, these 5 Outcome Measures provide metrics to identify healthcare providers of good care, and encourage care improvement by all healthcare providers. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s13023-018-0850-2) contains supplementary material, which is available to authorized users.
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spelling pubmed-60945832018-08-24 European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT) Shovlin, Claire L. Buscarini, Elisabetta Kjeldsen, Anette D. Mager, Hans Jurgen Sabba, Carlo Droege, Freya Geisthoff, Urban Ugolini, Sara Dupuis-Girod, Sophie Orphanet J Rare Dis Position Statement Hereditary haemorrhagic telangiectasia (HHT) is a multisystemic vascular dysplasia that leads to nosebleeds, anaemia due to blood loss, and arteriovenous malformations (AVMs) in organs such as the lungs, liver and brain. HHT is estimated to affect 85,000 European citizens, but most health care providers have limited prior HHT exposure or training. Outcome Measures were developed and implemented by the HHT Working Group of the European Reference Network for Rare Vascular Diseases (VASCERN), in order to maximise the number of patients receiving good care. The measures specifically target areas where optimal management reduces morbidity and mortality in HHT patients, and were designed to be robust to emerging new evidence. Thresholds are the percentage of patients in particular settings who have been recommended screening, or provided with written advice. The 5 Outcome Measures cover (1) pulmonary AVM screening; (2) written nosebleed advice, (3) assessment of iron deficiency; (4) antibiotic prophylaxis prior to dental and surgical procedures for patients with pulmonary AVMs, and (5) written advice on pregnancy. They are not a blueprint for detailed HHT management, but are suitable for all clinicians to be aware of and implement. In summary, these 5 Outcome Measures provide metrics to identify healthcare providers of good care, and encourage care improvement by all healthcare providers. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s13023-018-0850-2) contains supplementary material, which is available to authorized users. BioMed Central 2018-08-15 /pmc/articles/PMC6094583/ /pubmed/30111344 http://dx.doi.org/10.1186/s13023-018-0850-2 Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Position Statement
Shovlin, Claire L.
Buscarini, Elisabetta
Kjeldsen, Anette D.
Mager, Hans Jurgen
Sabba, Carlo
Droege, Freya
Geisthoff, Urban
Ugolini, Sara
Dupuis-Girod, Sophie
European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)
title European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)
title_full European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)
title_fullStr European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)
title_full_unstemmed European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)
title_short European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)
title_sort european reference network for rare vascular diseases (vascern) outcome measures for hereditary haemorrhagic telangiectasia (hht)
topic Position Statement
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6094583/
https://www.ncbi.nlm.nih.gov/pubmed/30111344
http://dx.doi.org/10.1186/s13023-018-0850-2
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