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  • Rodeghiero, FrancescoOspedale San Bortolo (författare)

Fundamentals for a Systematic Approach to Mild and Moderate Inherited Bleeding Disorders : An EHA Consensus Report

  • Artikel/kapitelEngelska2019

Förlag, utgivningsår, omfång ...

  • 2019

Nummerbeteckningar

  • LIBRIS-ID:oai:lup.lub.lu.se:ed1980ee-a25c-4b10-8d9d-f91c190bd7d2
  • https://lup.lub.lu.se/record/ed1980ee-a25c-4b10-8d9d-f91c190bd7d2URI
  • https://doi.org/10.1097/HS9.0000000000000286DOI

Kompletterande språkuppgifter

  • Språk:engelska
  • Sammanfattning på:engelska

Ingår i deldatabas

Klassifikation

  • Ämneskategori:art swepub-publicationtype
  • Ämneskategori:ref swepub-contenttype

Anmärkningar

  • Healthy subjects frequently report minor bleedings that are frequently 'background noise' of normality rather than a true disorder. Nevertheless, unexpected or unusual bleeding may be alarming. Thus, the distinction between normal and pathologic bleeding is critical. Understanding the underlying pathologic mechanism in patients with an excessive bleeding is essential for their counseling and treatment. Most of these patients with significant bleeding will result affected by non-severe inherited bleeding disorders (BD), collectively denominated mild or moderate BD for their relatively benign course. Unfortunately, practical recommendations for the management of these disorders are still lacking due to the current state of fragmented knowledge of pathophysiology and lack of a systematic diagnostic approach. To address this gap, an International Working Group (IWG) was established by the European Hematology Association (EHA) to develop consensus-based guidelines on these disorders. The IWG agreed that grouping these disorders by their clinical phenotype under the single category of mild-to-moderate bleeding disorders (MBD) reflects current clinical practice and will facilitate a systematic diagnostic approach. Based on standardized and harmonized definitions a conceptual unified framework is proposed to distinguish normal subjects from affected patients. The IWG proposes a provisional comprehensive patient-centered initial diagnostic approach that will result in classification of MBD into distinct clinical-pathological entities under the overarching principle of clinical utility for the individual patient. While we will present here a general overview of the global management of patients with MBD, this conceptual framework will be adopted and validated in the evidence-based, disease-specific guidelines under development by the IWG.

Ämnesord och genrebeteckningar

Biuppslag (personer, institutioner, konferenser, titlar ...)

  • Pabinger, IngridMedical University of Vienna (författare)
  • Ragni, MargaretUniversity of Pittsburgh Medical Center (författare)
  • Abdul-Kadir, RezanUniversity College London (författare)
  • Berntorp, ErikLund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups(Swepub:lu)medf-ebe (författare)
  • Blanchette, VictorHospital for Sick Children, Toronto (författare)
  • Bodó, ImreSemmelweis University (författare)
  • Casini, AlessandroGeneva University Hospital (författare)
  • Gresele, PaoloUniversity of Perugia (författare)
  • Lassila, RiittaHelsinki University Central Hospital (författare)
  • Leebeek, FrankErasmus University Medical Center (författare)
  • Lillicrap, DavidQueen's University at Kingston (författare)
  • Mezzano, DiegoPontifical Catholic University of Chile (författare)
  • Noris, PatriziaUniversity of Pavia (författare)
  • Srivastava, AlokChristian Medical College & Hospital (författare)
  • Tosetto, AlbertoOspedale San Bortolo (författare)
  • Windyga, JerzyInstitute of Haematology and Transfusion Medicine, Warsaw (författare)
  • Zieger, BarbaraUniversity Medical Center Freiburg (författare)
  • Makris, MikeRoyal Hallamshire Hospital (författare)
  • Key, NigelUniversity of North Carolina (författare)
  • Ospedale San BortoloMedical University of Vienna (creator_code:org_t)

Sammanhörande titlar

  • Ingår i:HemaSphere3:52572-9241

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