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Definition of primary immunodeficiency in 2011: a “trialogue” among friends

Authors

  • Mary Ellen Conley,

    1. University of Tennessee College of Medicine, St. Jude Children's Research Hospital, Memphis, Tennessee.
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  • Luigi D. Notarangelo,

    1. Division of Immunology and the Manton Center for Orphan Disease Research, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts.
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  • Jean-Laurent Casanova

    1. St. Giles Laboratory of Human Genetics of Infectious Disease, Rockefeller Branch, the Rockefeller University, New York, New York; and Laboratory of Human Genetics of Infectious Disease, Necker Branch, University Paris Descartes-INSERM U980, Paris, France
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  • Preferred citation: Conley, M.E., L.D. Notarangelo & J.-L. Casanova. 2011. Definition of primary immunodeficiency in 2011: a “trialogue” among friends. In “The Year in Human and Medical Genetics: Inborn Errors of Immunity I.” Jean-Laurent Casanova, Mary Ellen Conley & Luigi Notarangelo, Eds. Ann. N.Y. Acad. Sci.1238: 1–6.

Mary Ellen Conley, M. D., University of Tennessee College of Medicine, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105. maryellen.conley@stjude.org

Abstract

There is no clear consensus about the definition of the term primary immunodeficiency in 2011. Although there is general agreement that defects in both adaptive and innate immunity should be included, issues related to the frequency of primary immunodeficiencies, the modes of inheritance, the other types of cells involved, and the required clinical phenotype are more contentious. Three friends with an interest in both the clinical and scientific aspects of primary immunodeficiency carried out a discussion or trialogue to address some of these issues.

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