European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)
Abstract 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 c...
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| Format: | Article |
| Language: | English |
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BMC
2018-08-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13023-018-0850-2 |
| _version_ | 1818161953950924800 |
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| author | Claire L. Shovlin Elisabetta Buscarini Anette D. Kjeldsen Hans Jurgen Mager Carlo Sabba Freya Droege Urban Geisthoff Sara Ugolini Sophie Dupuis-Girod |
| author_facet | Claire L. Shovlin Elisabetta Buscarini Anette D. Kjeldsen Hans Jurgen Mager Carlo Sabba Freya Droege Urban Geisthoff Sara Ugolini Sophie Dupuis-Girod |
| author_sort | Claire L. Shovlin |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-11T16:25:58Z |
| format | Article |
| id | doaj.art-c73f4c12e562424b8cdccc41121327a4 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-11T16:25:58Z |
| publishDate | 2018-08-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-c73f4c12e562424b8cdccc41121327a42022-12-22T00:58:44ZengBMCOrphanet Journal of Rare Diseases1750-11722018-08-011311510.1186/s13023-018-0850-2European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)Claire L. Shovlin0Elisabetta Buscarini1Anette D. Kjeldsen2Hans Jurgen Mager3Carlo Sabba4Freya Droege5Urban Geisthoff6Sara Ugolini7Sophie Dupuis-Girod8Respiratory Medicine, and VASCERN HHT European Reference Centre, Hammersmith Hospital, Imperial College Healthcare NHS TrustGastroenterology Department and VASCERN HHT European Reference Centre, Maggiore Hospital, ASST CremaDepartment of Otorhinolaryngology and VASCERN HHT European Reference Centre, Odense University Hospital, University of Southern DenmarkDepartment of Pulmonology and VASCERN HHT European Reference Centre, St. Antonius HospitalCenter for Rare Diseases, “Frugoni” Internal Medicine Unit, Interdepartmental HHT Center, Interdisciplinary Department of Medicine and VASCERN HHT European Reference Centre, University of Bari School of MedicineDepartment of Otorhinolaryngology and VASCERN HHT European Reference Centre, Essen University HospitalDepartment of Otorhinolaryngology and VASCERN HHT European Reference Centre, Essen University HospitalDepartment of Otorhinolaryngology and VASCERN HHT European Reference Centre, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (I.R.C.C.S) Policlinico San Matteo, University of PaviaHospices Civils de Lyon, Hôpital Femme-Mère-Enfants, Service de Génétique, and VASCERN HHT European Reference Centre/ centre de Référence pour la maladie de Rendu-OslerAbstract 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.http://link.springer.com/article/10.1186/s13023-018-0850-2AnaemiaAntibiotic prophylaxisEpistaxisIron deficiencyNosebleedsPulmonary arteriovenous malformations |
| spellingShingle | Claire L. Shovlin Elisabetta Buscarini Anette D. Kjeldsen Hans Jurgen Mager Carlo Sabba Freya Droege Urban Geisthoff Sara Ugolini Sophie Dupuis-Girod European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT) Orphanet Journal of Rare Diseases Anaemia Antibiotic prophylaxis Epistaxis Iron deficiency Nosebleeds Pulmonary arteriovenous malformations |
| 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 | Anaemia Antibiotic prophylaxis Epistaxis Iron deficiency Nosebleeds Pulmonary arteriovenous malformations |
| url | http://link.springer.com/article/10.1186/s13023-018-0850-2 |
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