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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Bibliographic Details
Published in:Orphanet journal of rare diseases 2018-08, Vol.13 (1), p.136-136, Article 136
Main Authors: Shovlin, Claire L, Buscarini, Elisabetta, Kjeldsen, Anette D, Mager, Hans Jurgen, Sabba, Carlo, Droege, Freya, Geisthoff, Urban, Ugolini, Sara, Dupuis-Girod, Sophie
Format: Article
Language:English
Subjects:
Anaemia
Anemia
Antibiotic prophylaxis
Antibiotics
Blood transfusion
Clinical medicine
Epistaxis
Gastroenterology
Health care
Iron
Iron deficiency
Medical research
Nosebleeds
Patients
Position Statement
Pulmonary arteriovenous malformations
Rare diseases
Vascular diseases
Working groups
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Summary: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.
ISSN:1750-1172
1750-1172
DOI:10.1186/s13023-018-0850-2