Guidelines for the management of hemophilia.
A. Srivastava,A. K. Brewer,Evelien P. Mauser-Bunschoten,Nigel S. Key,Steve Kitchen,Adolfo Llinás,C. A. Ludlam,Johnny Mahlangu,K. Mulder,M.-C. Poon,Alison Street +10 more
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TLDR
These evidence‐based guidelines offer practical recommendations on the diagnosis and general management of hemophilia, as well as the management of complications including musculoskeletal issues, inhibitors, and transfusion‐transmitted infections.Abstract:
Hemophilia is a rare disorder that is complex to diagnose and to manage. These evidence-based guidelines offer practical recommendations on the diagnosis and general management of hemophilia, as well as the management of complications including musculoskeletal issues, inhibitors, and transfusion-transmitted infections. By compiling these guidelines, the World Federation of Hemophilia aims to assist healthcare providers seeking to initiate and/or maintain hemophilia care programs, encourage practice harmonization around the world and, where recommendations lack adequate evidence, stimulate appropriate studies.read more
Citations
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Journal ArticleDOI
Management of severe perioperative bleeding Guidelines from the European Society of Anaesthesiology
Sibylle A. Kozek-Langenecker,Arash Afshari,Pierre Albaladejo,Cesar Aldecoa Alvarez Santullano,Edoardo De Robertis,Daniela Filipescu,Dietmar Fries,Thorsten Haas,Georgina Imberger,Matthias Jacob,Marcus D. Lancé,Juan V. Llau,Susan Mallett,Jens Meier,Niels Rahe-Meyer,Charles Marc Samama,Andrew Smith,Cristina Solomon,Philippe Van der Linden,Anne Wikkelsø,Patrick Wouters,Piet Wyffels +21 more
TL;DR: These guidelines are intended to provide an overview of current knowledge on the subject with an assessment of the quality of the evidence in order to allow anaesthetists throughout Europe to integrate this knowledge into daily patient care wherever possible.
Journal ArticleDOI
WFH Guidelines for the Management of Hemophilia, 3rd edition
Alok Srivastava,Elena Santagostino,Alison Dougall,Steve Kitchen,Megan Sutherland,Steven W. Pipe,Manuel Carcao,Johnny Mahlangu,Margaret V. Ragni,Jerzy Windyga,Adolfo Llinás,Nicholas J. Goddard,Richa Mohan,Pradeep M. Poonnoose,Brian M. Feldman,Sandra Zelman Lewis,H. Marijke van den Berg,Glenn F. Pierce +17 more
TL;DR: The WFH Guidelines for the Management of Hemophilia panelists and co-authors thank the panelists for their time and share their views on how to better understand and treat hemophilia.
Journal ArticleDOI
Management of severe perioperative bleeding: guidelines from the European Society of Anaesthesiology: First update 2016.
Sibylle A. Kozek-Langenecker,Aamer B Ahmed,Arash Afshari,Pierre Albaladejo,Cesar Aldecoa,Guidrius Barauskas,Edoardo De Robertis,David Faraoni,Daniela Filipescu,Dietmar Fries,Thorsten Haas,Matthias Jacob,Marcus D. Lancé,Juan V.L. Pitarch,Susan Mallett,Jens Meier,Zsolt Molnár,Niels Rahe-Meyer,Charles Marc Samama,Jakob Stensballe,Philippe Van der Linden,Anne Wikkelsø,Patrick Wouters,Piet Wyffels,Kai Zacharowski +24 more
TL;DR: This update includes revisions to existing recommendations with respect to the wording, or changes in the grade of recommendation, and also the addition of new recommendations.
Journal ArticleDOI
Hemophilia B Gene Therapy with a High-Specific-Activity Factor IX Variant.
Lindsey A. George,Spencer K. Sullivan,Adam Giermasz,John E.J. Rasko,John E.J. Rasko,Benjamin J. Samelson-Jones,Jonathan M. Ducore,Adam Cuker,Lisa M. Sullivan,Suvankar Majumdar,Jerome M. Teitel,Catherine E. McGuinn,Margaret V. Ragni,Alvin Luk,Daniel Hui,J. Fraser Wright,Yifeng Chen,Yun Liu,Katie Wachtel,Angela Winters,Stefan Tiefenbacher,Valder R. Arruda,Johannes C.M. Van der Loo,Olga Zelenaia,Daniel Takefman,Marcus E. Carr,Linda B. Couto,Xavier M. Anguela,Katherine A. High +28 more
TL;DR: Transgene‐derived factor IX coagulant activity enabled the termination of baseline prophylaxis and the near elimination of bleeding and factor use in 10 participants with hemophilia who received the same vectors.
Journal ArticleDOI
AAV5–Factor VIII Gene Transfer in Severe Hemophilia A
Savita Rangarajan,Liron Walsh,Will Lester,David Perry,Bella Madan,Michael Laffan,Hua Yu,Christian Vettermann,Glenn F. Pierce,Wing Yen Wong,K. John Pasi +10 more
TL;DR: The infusion of AAV5‐hFVIII‐SQ was associated with the sustained normalization of factor VIII activity level over a period of 1 year in six of seven participants who received a high dose, with stabilization of hemostasis and a profound reduction in factor VIII use in all seven participants.
References
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Journal ArticleDOI
Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia.
Marilyn J. Manco-Johnson,Marilyn J. Manco-Johnson,Thomas C. Abshire,Amy D. Shapiro,Brenda Riske,Michele R. Hacker,Ray F. Kilcoyne,J. David Ingram,M L Manco-Johnson,Sharon Funk,Linda J. Jacobson,Leonard A. Valentino,W. Keith Hoots,George R. Buchanan,Donna DiMichele,Michael Recht,Deborah L Brown,Cindy A. Leissinger,Shirley Bleak,Alan R. Cohen,Prasad Mathew,Alison Matsunaga,Desiree Medeiros,Diane J. Nugent,Gregory Thomas,Alexis A. Thompson,Kevin McRedmond,J. Michael Soucie,Harlan Austin,Bruce L. Evatt +29 more
TL;DR: Prophylaxis with recombinant factor VIII can prevent joint damage and decrease the frequency of joint and other hemorrhages in young boys with severe hemophilia A.
Journal ArticleDOI
Twenty-five years' experience of prophylactic treatment in severe haemophilia A and B
TL;DR: It appears to be possible to prevent haemophilic arthropathy by giving effective continuous prophylaxis from an early age, and preventing the VIII:C or IX:C concentration from falling below 1% of normal.
Journal ArticleDOI
Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis.
Gilbert C. White,Frits R. Rosendaal,Louis M. Aledort,Jeanne M. Lusher,Chantal Rothschild,Jørgen Ingerslev +5 more
TL;DR: Definitions in Hemophilia - Recommendation of the Scientific Subcommittee on Factor VIII and Factor IX and the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis.
Journal ArticleDOI
Guidelines for the use of fresh-frozen plasma, cryoprecipitate and cryosupernatant.
D F O'Shaughnessy,C Atterbury,P. Bolton Maggs,Michael F. Murphy,Dafydd Thomas,S Yates,Lorna M. Williamson +6 more
TL;DR: Fresh‐frozen plasma (FFP), cryoprecipitate and cryosupernatant plasma are very limited and should not be used to reverse warfarin anticoagulation in the absence of severe bleeding, and PRP may be used as an alternative to FFP.
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Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia.
Marilyn J. Manco-Johnson,Marilyn J. Manco-Johnson,Thomas C. Abshire,Amy D. Shapiro,Brenda Riske,Michele R. Hacker,Ray F. Kilcoyne,J. David Ingram,M L Manco-Johnson,Sharon Funk,Linda J. Jacobson,Leonard A. Valentino,W. Keith Hoots,George R. Buchanan,Donna DiMichele,Michael Recht,Deborah L Brown,Cindy A. Leissinger,Shirley Bleak,Alan R. Cohen,Prasad Mathew,Alison Matsunaga,Desiree Medeiros,Diane J. Nugent,Gregory Thomas,Alexis A. Thompson,Kevin McRedmond,J. Michael Soucie,Harlan Austin,Bruce L. Evatt +29 more