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Primary immune deficiencies - principles of care

Chapel, Helen ; Prevot, Johan ; Gaspar, Hubert Bobby ; Español, Teresa ; Bonilla, Francisco A ; Solis, Leire ; Drabwell, Josina

Frontiers in immunology, 2014-12, Vol.5, p.627-627 [Periódico revisado por pares]

Switzerland: Frontiers Media S.A

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  • Título:
    Primary immune deficiencies - principles of care
  • Autor: Chapel, Helen ; Prevot, Johan ; Gaspar, Hubert Bobby ; Español, Teresa ; Bonilla, Francisco A ; Solis, Leire ; Drabwell, Josina
  • Assuntos: Awareness ; diagnosis ; Immunology ; Management ; Primary Immunodeficiencies ; treatments ; worldwide
  • É parte de: Frontiers in immunology, 2014-12, Vol.5, p.627-627
  • Notas: ObjectType-Article-2
    SourceType-Scholarly Journals-1
    ObjectType-Feature-3
    content type line 23
    ObjectType-Review-1
    Edited by: Guzide Aksu, Ege University School of Medicine, Turkey
    Reviewed by: Klaus Warnatz, University of Freiburg, Germany; Laurence E. Cheng, University of California San Francisco, USA
    Contributors: M. Ballow (Children’s Hospital of Buffalo, USA); L. Brown (UCL Institute of Child Health, London, UK); B. T. Costa-Carvalho (Federal University of São Paulo, Brazil); J. D. Edgar (United Kingdom Primary Immunodeficiency Network, UK); W. Egner (British Society for Immunology, UK); S. Ehl (Center for Chronic Immunodeficiency, Freiburg, Germany); M. Esser (University of Stellenbosch, South Africa); A. Gardulf (Karolinska Institute at Huddinge University Hospital, Sweden); A. Gennery (University of Newcastle upon Tyne, Newcastle, UK); J. Kerr (Paul-Ehrlich-Institut, Langen, Germany); C. Kinnon (UCL Institute of Child Health, London, UK), Y. L. Lau (The University of Hong Kong, Hong Kong, China); J. Litzman (St. Anne’s University Hospital Brno, Czech Republic); H. J. Longhurst (Barts Health NHS Trust, London, UK); N. Mahlaoui (Centre de Référence Déficits Immunitaires Héréditaires – CEREDIH, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, France); J. S. Orange (Texas Children’s Hospital and Baylor College of Medicine, USA); C. Picard (Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, France); J. Puck (University of California San Francisco, UCSF Benioff Children’s Hospital, San Francisco, USA); S. Sánchez Ramon (Hospital Clínico San Carlos, Madrid, Spain); R. E. Schmidt (Medical University Hanover, Hanover, Germany); S. Singh (Advanced Pediatrics Center, PGIMER, Chandigarh, India); G. Spickett (University of Newcastle upon Tyne, Newcastle, UK); K. Sullivan (Children’s Hospital of Philadelphia, USA); A. Symes (Royal Free Hospital NHS Trust, London, UK)
    This article was submitted to Primary Immunodeficiencies, a section of the journal Frontiers in Immunology.
  • Descrição: Primary immune deficiencies (PIDs) are a growing group of over 230 different disorders caused by ineffective, absent or an increasing number of gain of function mutations in immune components, mainly cells and proteins. Once recognized, these rare disorders are treatable and in some cases curable. Otherwise untreated PIDs are often chronic, serious, or even fatal. The diagnosis of PIDs can be difficult due to lack of awareness or facilities for diagnosis, and management of PIDs is complex. This document was prepared by a worldwide multi-disciplinary team of specialists; it aims to set out comprehensive principles of care for PIDs. These include the role of specialized centers, the importance of registries, the need for multinational research, the role of patient organizations, management and treatment options, the requirement for sustained access to all treatments including immunoglobulin therapies and hematopoietic stem cell transplantation, important considerations for developing countries and suggestions for implementation. A range of healthcare policies and services have to be put into place by government agencies and healthcare providers, to ensure that PID patients worldwide have access to appropriate and sustainable medical and support services.
  • Editor: Switzerland: Frontiers Media S.A
  • Idioma: Inglês
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