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Hereditary haemorrhagic telangiectasia: pathophysiology, diagnosis and treatment.

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Title: Hereditary haemorrhagic telangiectasia: pathophysiology, diagnosis and treatment.
Authors: Shovlin, CL
Item Type: Journal Article
Issue Date: 1-Nov-2010
Date of Acceptance: 25-Sep-2010
URI: http://hdl.handle.net/10044/1/22167
DOI: https://dx.doi.org/10.1016/j.blre.2010.07.001
ISSN: 1532-1681
Publisher: Elsevier
Start Page: 203
End Page: 219
Journal / Book Title: Blood Reviews
Volume: 24
Issue: 6
Copyright Statement: © 2010, Elsevier. Licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International http://creativecommons.org/licenses/by-nc-nd/4.0/
Notes: Writing this systematic review took two years, requiring generation of new data; synthesis of materials from diverse fields; discussions with major UK stakeholders on screening; and incorporation of international guidelines that were then still ’ in press’, but were already four years out of date. Between April –October 2011, when the website for this prestigious haematology journal listed download rates, the article was in the all time top 10 downloaded articles. this is translating to a rapid rise in citations. The manuscript provides a conceptual framework for the whole patient, incorporating familial, population and longitudinal concepts that are difficult to appreciate from conventional peer reviewed articles with a particular focus. The manuscript refocuses pathophysiolgy in a disease that was at risk of being defined by over-simplistic concepts, phenotypes defined in mouse models not always related to HHT, and pharmaceutical hype regarding therapies lacking an appropriate evidence base. The manuscript educates clinicians (most of whom are organ-specific specialists or sub-specialists), to improve management in this complex and highly challenging disorder. For the NHS, the manuscript used systematic review to highlight that recommendations for intensive screening programmes derived in other healthcare cultures may be neither affordable, nor appropriate, for the UK.
Publication Status: Published
Appears in Collections:National Heart and Lung Institute
Faculty of Medicine



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