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The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies

Identifieur interne : 000C91 ( Pmc/Checkpoint ); précédent : 000C90; suivant : 000C92

The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies

Auteurs : Aziz Bousfiha [Maroc] ; Leïla Jeddane [Maroc] ; Waleed Al-Herz [Koweït] ; Fatima Ailal [Maroc] ; Jean-Laurent Casanova [États-Unis, France] ; Talal Chatila [États-Unis] ; Mary Ellen Conley [États-Unis] ; Charlotte Cunningham-Rundles [États-Unis] ; Amos Etzioni [Israël] ; Jose Luis Franco [Colombie] ; H. Bobby Gaspar [Royaume-Uni] ; Steven M. Holland [États-Unis] ; Christoph Klein [Allemagne] ; Shigeaki Nonoyama [Japon] ; Hans D. Ochs [États-Unis] ; Eric Oksenhendler [France] ; Capucine Picard [États-Unis, France] ; Jennifer M. Puck [États-Unis] ; Kathleen E. Sullivan [États-Unis] ; Mimi L. K. Tang [Australie]

Source :

RBID : PMC:4659854

Abstract

There are now nearly 300 single-gene inborn errors of immunity underlying phenotypes as diverse as infection, malignancy, allergy, auto-immunity, and auto-inflammation. For each of these five categories, a growing variety of phenotypes are ascribed to Primary Immunodeficiency Diseases (PID), making PIDs a rapidly expanding field of medicine. The International Union of Immunological Societies (IUIS) PID expert committee (EC) has published every other year a classification of these disorders into tables, defined by shared pathogenesis and/or clinical consequences. In 2013, the IUIS committee also proposed a more user-friendly, phenotypic classification, based on the selection of key phenotypes at the bedside. We herein propose the revised figures, based on the accompanying 2015 IUIS PID EC classification.


Url:
DOI: 10.1007/s10875-015-0198-5
PubMed: 26445875
PubMed Central: 4659854


Affiliations:


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PMC:4659854

Le document en format XML

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<name sortKey="Conley, Mary Ellen" sort="Conley, Mary Ellen" uniqKey="Conley M" first="Mary Ellen" last="Conley">Mary Ellen Conley</name>
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<name sortKey="Klein, Christoph" sort="Klein, Christoph" uniqKey="Klein C" first="Christoph" last="Klein">Christoph Klein</name>
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<name sortKey="Nonoyama, Shigeaki" sort="Nonoyama, Shigeaki" uniqKey="Nonoyama S" first="Shigeaki" last="Nonoyama">Shigeaki Nonoyama</name>
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<name sortKey="Ochs, Hans D" sort="Ochs, Hans D" uniqKey="Ochs H" first="Hans D." last="Ochs">Hans D. Ochs</name>
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<name sortKey="Oksenhendler, Eric" sort="Oksenhendler, Eric" uniqKey="Oksenhendler E" first="Eric" last="Oksenhendler">Eric Oksenhendler</name>
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<name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name>
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<name sortKey="Puck, Jennifer M" sort="Puck, Jennifer M" uniqKey="Puck J" first="Jennifer M." last="Puck">Jennifer M. Puck</name>
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<name sortKey="Sullivan, Kathleen E" sort="Sullivan, Kathleen E" uniqKey="Sullivan K" first="Kathleen E." last="Sullivan">Kathleen E. Sullivan</name>
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<name sortKey="Tang, Mimi L K" sort="Tang, Mimi L K" uniqKey="Tang M" first="Mimi L. K." last="Tang">Mimi L. K. Tang</name>
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<name sortKey="Ailal, Fatima" sort="Ailal, Fatima" uniqKey="Ailal F" first="Fatima" last="Ailal">Fatima Ailal</name>
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<name sortKey="Casanova, Jean Aurent" sort="Casanova, Jean Aurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
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<region type="state">État de New York</region>
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<nlm:aff id="Aff5">Howard Hughes Medical Institute, New York, NY USA</nlm:aff>
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<region type="state">État de New York</region>
</placeName>
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<country xml:lang="fr">France</country>
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<region type="region">Île-de-France</region>
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<nlm:aff id="Aff7">Imagine Institute, University Paris Descartes, Paris, France</nlm:aff>
<country xml:lang="fr">France</country>
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<region type="region">Île-de-France</region>
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<nlm:aff id="Aff8">Pediatric Hematology & Immunology Unit, Necker Hospital for Sick Children, Paris, France</nlm:aff>
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<wicri:regionArea>Pediatric Hematology & Immunology Unit, Necker Hospital for Sick Children, Paris</wicri:regionArea>
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<name sortKey="Chatila, Talal" sort="Chatila, Talal" uniqKey="Chatila T" first="Talal" last="Chatila">Talal Chatila</name>
<affiliation wicri:level="2">
<nlm:aff id="Aff9">Division of Immunology, Children’s Hospital Boston, Boston, MA USA</nlm:aff>
<country>États-Unis</country>
<placeName>
<region type="state">Massachusetts</region>
</placeName>
<wicri:cityArea>Division of Immunology, Children’s Hospital Boston, Boston</wicri:cityArea>
</affiliation>
</author>
<author>
<name sortKey="Conley, Mary Ellen" sort="Conley, Mary Ellen" uniqKey="Conley M" first="Mary Ellen" last="Conley">Mary Ellen Conley</name>
<affiliation wicri:level="2">
<nlm:aff id="Aff4">St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY USA</nlm:aff>
<country>États-Unis</country>
<placeName>
<region type="state">État de New York</region>
</placeName>
<wicri:cityArea>St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York</wicri:cityArea>
</affiliation>
</author>
<author>
<name sortKey="Cunningham Undles, Charlotte" sort="Cunningham Undles, Charlotte" uniqKey="Cunningham Undles C" first="Charlotte" last="Cunningham-Rundles">Charlotte Cunningham-Rundles</name>
<affiliation wicri:level="2">
<nlm:aff id="Aff10">Department of Medicine and Pediatrics, Mount Sinai School of Medicine, New York, NY USA</nlm:aff>
<country>États-Unis</country>
<placeName>
<region type="state">État de New York</region>
</placeName>
<wicri:cityArea>Department of Medicine and Pediatrics, Mount Sinai School of Medicine, New York</wicri:cityArea>
</affiliation>
</author>
<author>
<name sortKey="Etzioni, Amos" sort="Etzioni, Amos" uniqKey="Etzioni A" first="Amos" last="Etzioni">Amos Etzioni</name>
<affiliation wicri:level="1">
<nlm:aff id="Aff11">Meyer Children’s Hospital‐Technion, Haifa, Israel</nlm:aff>
<country xml:lang="fr">Israël</country>
<wicri:regionArea>Meyer Children’s Hospital‐Technion, Haifa</wicri:regionArea>
<wicri:noRegion>Haifa</wicri:noRegion>
</affiliation>
</author>
<author>
<name sortKey="Franco, Jose Luis" sort="Franco, Jose Luis" uniqKey="Franco J" first="Jose Luis" last="Franco">Jose Luis Franco</name>
<affiliation wicri:level="1">
<nlm:aff id="Aff12">Group of Primary Immunodeficiencies, University of Antioquia, Medellin, Colombia</nlm:aff>
<country xml:lang="fr">Colombie</country>
<wicri:regionArea>Group of Primary Immunodeficiencies, University of Antioquia, Medellin</wicri:regionArea>
<wicri:noRegion>Medellin</wicri:noRegion>
</affiliation>
</author>
<author>
<name sortKey="Gaspar, H Bobby" sort="Gaspar, H Bobby" uniqKey="Gaspar H" first="H. Bobby" last="Gaspar">H. Bobby Gaspar</name>
<affiliation wicri:level="3">
<nlm:aff id="Aff13">UCL Institute of Child Health, London, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>UCL Institute of Child Health, London</wicri:regionArea>
<placeName>
<settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Holland, Steven M" sort="Holland, Steven M" uniqKey="Holland S" first="Steven M." last="Holland">Steven M. Holland</name>
<affiliation wicri:level="2">
<nlm:aff id="Aff14">Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD USA</nlm:aff>
<country>États-Unis</country>
<placeName>
<region type="state">Maryland</region>
</placeName>
<wicri:cityArea>Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda</wicri:cityArea>
</affiliation>
</author>
<author>
<name sortKey="Klein, Christoph" sort="Klein, Christoph" uniqKey="Klein C" first="Christoph" last="Klein">Christoph Klein</name>
<affiliation wicri:level="3">
<nlm:aff id="Aff15">Dr von Hauner Children’s Hospital, Ludwig‐Maximilians University Munich, Munich, Germany</nlm:aff>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Dr von Hauner Children’s Hospital, Ludwig‐Maximilians University Munich, Munich</wicri:regionArea>
<placeName>
<region type="land" nuts="1">Bavière</region>
<region type="district" nuts="2">District de Haute-Bavière</region>
<settlement type="city">Munich</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Nonoyama, Shigeaki" sort="Nonoyama, Shigeaki" uniqKey="Nonoyama S" first="Shigeaki" last="Nonoyama">Shigeaki Nonoyama</name>
<affiliation wicri:level="1">
<nlm:aff id="Aff16">Department of Pediatrics, National Defense Medical College, Saitama, Japan</nlm:aff>
<country xml:lang="fr">Japon</country>
<wicri:regionArea>Department of Pediatrics, National Defense Medical College, Saitama</wicri:regionArea>
<wicri:noRegion>Saitama</wicri:noRegion>
</affiliation>
</author>
<author>
<name sortKey="Ochs, Hans D" sort="Ochs, Hans D" uniqKey="Ochs H" first="Hans D." last="Ochs">Hans D. Ochs</name>
<affiliation wicri:level="2">
<nlm:aff id="Aff17">Department of Pediatrics, University of Washington and Seattle Children’s Research Institute, Seattle, WA USA</nlm:aff>
<country>États-Unis</country>
<placeName>
<region type="state">Washington (État)</region>
</placeName>
<wicri:cityArea>Department of Pediatrics, University of Washington and Seattle Children’s Research Institute, Seattle</wicri:cityArea>
</affiliation>
</author>
<author>
<name sortKey="Oksenhendler, Eric" sort="Oksenhendler, Eric" uniqKey="Oksenhendler E" first="Eric" last="Oksenhendler">Eric Oksenhendler</name>
<affiliation wicri:level="3">
<nlm:aff id="Aff18">Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique‐Hôpitaux de Paris, Paris, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique‐Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName>
<region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3">
<nlm:aff id="Aff19">Université Paris Diderot, Sorbonne Paris Cité, Paris, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris Diderot, Sorbonne Paris Cité, Paris</wicri:regionArea>
<placeName>
<region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name>
<affiliation wicri:level="2">
<nlm:aff id="Aff5">Howard Hughes Medical Institute, New York, NY USA</nlm:aff>
<country>États-Unis</country>
<placeName>
<region type="state">État de New York</region>
</placeName>
<wicri:cityArea>Howard Hughes Medical Institute, New York</wicri:cityArea>
</affiliation>
<affiliation wicri:level="3">
<nlm:aff id="Aff20">Centre d’étude des déficits immunitaires (CEDI), Hôpital Necker‐Enfants Malades, AP-HP, Paris, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Centre d’étude des déficits immunitaires (CEDI), Hôpital Necker‐Enfants Malades, AP-HP, Paris</wicri:regionArea>
<placeName>
<region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Puck, Jennifer M" sort="Puck, Jennifer M" uniqKey="Puck J" first="Jennifer M." last="Puck">Jennifer M. Puck</name>
<affiliation wicri:level="2">
<nlm:aff id="Aff21">Department of Pediatrics, University of California San Francisco and UCSF Benioff Children’s Hospital, San Francisco, CA USA</nlm:aff>
<country>États-Unis</country>
<placeName>
<region type="state">Californie</region>
</placeName>
<wicri:cityArea>Department of Pediatrics, University of California San Francisco and UCSF Benioff Children’s Hospital, San Francisco</wicri:cityArea>
</affiliation>
</author>
<author>
<name sortKey="Sullivan, Kathleen E" sort="Sullivan, Kathleen E" uniqKey="Sullivan K" first="Kathleen E." last="Sullivan">Kathleen E. Sullivan</name>
<affiliation wicri:level="2">
<nlm:aff id="Aff22">Division of Allergy Immunology, Department of Pediatrics, The Children’s Hospital of Philadelphia, Philadelphia, PA USA</nlm:aff>
<country>États-Unis</country>
<placeName>
<region type="state">Pennsylvanie</region>
</placeName>
<wicri:cityArea>Division of Allergy Immunology, Department of Pediatrics, The Children’s Hospital of Philadelphia, Philadelphia</wicri:cityArea>
</affiliation>
</author>
<author>
<name sortKey="Tang, Mimi L K" sort="Tang, Mimi L K" uniqKey="Tang M" first="Mimi L. K." last="Tang">Mimi L. K. Tang</name>
<affiliation>
<nlm:aff id="Aff23">Murdoch Childrens Research Institute, Melbourne, VIC Australia</nlm:aff>
<wicri:noCountry code="subfield">VIC Australia</wicri:noCountry>
</affiliation>
<affiliation wicri:level="4">
<nlm:aff id="Aff24">Department of Paediatrics, University of Melbourne, Melbourne, VIC Australia</nlm:aff>
<orgName type="university">Université de Melbourne</orgName>
<country>Australie</country>
<placeName>
<settlement type="city">Melbourne</settlement>
<region type="état">Victoria (État)</region>
</placeName>
</affiliation>
<affiliation>
<nlm:aff id="Aff25">Department of Allergy and Immunology, Royal Children’s Hospital, Melbourne, VIC Australia</nlm:aff>
<wicri:noCountry code="subfield">VIC Australia</wicri:noCountry>
</affiliation>
</author>
</analytic>
<series>
<title level="j">Journal of Clinical Immunology</title>
<idno type="ISSN">0271-9142</idno>
<idno type="eISSN">1573-2592</idno>
<imprint>
<date when="2015">2015</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc>
<textClass></textClass>
</profileDesc>
</teiHeader>
<front>
<div type="abstract" xml:lang="en">
<p>There are now nearly 300 single-gene inborn errors of immunity underlying phenotypes as diverse as infection, malignancy, allergy, auto-immunity, and auto-inflammation. For each of these five categories, a growing variety of phenotypes are ascribed to Primary Immunodeficiency Diseases (PID), making PIDs a rapidly expanding field of medicine. The International Union of Immunological Societies (IUIS) PID expert committee (EC) has published every other year a classification of these disorders into tables, defined by shared pathogenesis and/or clinical consequences. In 2013, the IUIS committee also proposed a more user-friendly, phenotypic classification, based on the selection of key phenotypes at the bedside. We herein propose the revised figures, based on the accompanying 2015 IUIS PID EC classification.</p>
</div>
</front>
<back>
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</TEI>
<pmc article-type="research-article">
<pmc-dir>properties open_access</pmc-dir>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-ta">J Clin Immunol</journal-id>
<journal-id journal-id-type="iso-abbrev">J. Clin. Immunol</journal-id>
<journal-title-group>
<journal-title>Journal of Clinical Immunology</journal-title>
</journal-title-group>
<issn pub-type="ppub">0271-9142</issn>
<issn pub-type="epub">1573-2592</issn>
<publisher>
<publisher-name>Springer US</publisher-name>
<publisher-loc>New York</publisher-loc>
</publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">26445875</article-id>
<article-id pub-id-type="pmc">4659854</article-id>
<article-id pub-id-type="publisher-id">198</article-id>
<article-id pub-id-type="doi">10.1007/s10875-015-0198-5</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Original Research</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author" corresp="yes">
<name>
<surname>Bousfiha</surname>
<given-names>Aziz</given-names>
</name>
<address>
<email>profbousfiha@gmail.com</email>
</address>
<xref ref-type="aff" rid="Aff1"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Jeddane</surname>
<given-names>Leïla</given-names>
</name>
<xref ref-type="aff" rid="Aff1"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Al-Herz</surname>
<given-names>Waleed</given-names>
</name>
<xref ref-type="aff" rid="Aff2"></xref>
<xref ref-type="aff" rid="Aff3"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Ailal</surname>
<given-names>Fatima</given-names>
</name>
<xref ref-type="aff" rid="Aff1"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Casanova</surname>
<given-names>Jean‐Laurent</given-names>
</name>
<xref ref-type="aff" rid="Aff4"></xref>
<xref ref-type="aff" rid="Aff5"></xref>
<xref ref-type="aff" rid="Aff6"></xref>
<xref ref-type="aff" rid="Aff7"></xref>
<xref ref-type="aff" rid="Aff8"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Chatila</surname>
<given-names>Talal</given-names>
</name>
<xref ref-type="aff" rid="Aff9"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Conley</surname>
<given-names>Mary Ellen</given-names>
</name>
<xref ref-type="aff" rid="Aff4"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Cunningham‐Rundles</surname>
<given-names>Charlotte</given-names>
</name>
<xref ref-type="aff" rid="Aff10"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Etzioni</surname>
<given-names>Amos</given-names>
</name>
<xref ref-type="aff" rid="Aff11"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Franco</surname>
<given-names>Jose Luis</given-names>
</name>
<xref ref-type="aff" rid="Aff12"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Gaspar</surname>
<given-names>H. Bobby</given-names>
</name>
<xref ref-type="aff" rid="Aff13"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Holland</surname>
<given-names>Steven M.</given-names>
</name>
<xref ref-type="aff" rid="Aff14"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Klein</surname>
<given-names>Christoph</given-names>
</name>
<xref ref-type="aff" rid="Aff15"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Nonoyama</surname>
<given-names>Shigeaki</given-names>
</name>
<xref ref-type="aff" rid="Aff16"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Ochs</surname>
<given-names>Hans D.</given-names>
</name>
<xref ref-type="aff" rid="Aff17"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Oksenhendler</surname>
<given-names>Eric</given-names>
</name>
<xref ref-type="aff" rid="Aff18"></xref>
<xref ref-type="aff" rid="Aff19"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Picard</surname>
<given-names>Capucine</given-names>
</name>
<xref ref-type="aff" rid="Aff5"></xref>
<xref ref-type="aff" rid="Aff20"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Puck</surname>
<given-names>Jennifer M.</given-names>
</name>
<xref ref-type="aff" rid="Aff21"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Sullivan</surname>
<given-names>Kathleen E.</given-names>
</name>
<xref ref-type="aff" rid="Aff22"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Tang</surname>
<given-names>Mimi L. K.</given-names>
</name>
<xref ref-type="aff" rid="Aff23"></xref>
<xref ref-type="aff" rid="Aff24"></xref>
<xref ref-type="aff" rid="Aff25"></xref>
</contrib>
<aff id="Aff1">
<label></label>
Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco</aff>
<aff id="Aff2">
<label></label>
Department of Pediatrics, Faculty of Medicine Kuwait University, Jabriya, Kuwait</aff>
<aff id="Aff3">
<label></label>
Allergy and Clinical Immunology Unit, Department of Pediatrics, Al-Sabah Hospital, Kuwait City, Kuwait</aff>
<aff id="Aff4">
<label></label>
St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY USA</aff>
<aff id="Aff5">
<label></label>
Howard Hughes Medical Institute, New York, NY USA</aff>
<aff id="Aff6">
<label></label>
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Necker Hospital for Sick Children, Paris, France</aff>
<aff id="Aff7">
<label></label>
Imagine Institute, University Paris Descartes, Paris, France</aff>
<aff id="Aff8">
<label></label>
Pediatric Hematology & Immunology Unit, Necker Hospital for Sick Children, Paris, France</aff>
<aff id="Aff9">
<label></label>
Division of Immunology, Children’s Hospital Boston, Boston, MA USA</aff>
<aff id="Aff10">
<label></label>
Department of Medicine and Pediatrics, Mount Sinai School of Medicine, New York, NY USA</aff>
<aff id="Aff11">
<label></label>
Meyer Children’s Hospital‐Technion, Haifa, Israel</aff>
<aff id="Aff12">
<label></label>
Group of Primary Immunodeficiencies, University of Antioquia, Medellin, Colombia</aff>
<aff id="Aff13">
<label></label>
UCL Institute of Child Health, London, UK</aff>
<aff id="Aff14">
<label></label>
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD USA</aff>
<aff id="Aff15">
<label></label>
Dr von Hauner Children’s Hospital, Ludwig‐Maximilians University Munich, Munich, Germany</aff>
<aff id="Aff16">
<label></label>
Department of Pediatrics, National Defense Medical College, Saitama, Japan</aff>
<aff id="Aff17">
<label></label>
Department of Pediatrics, University of Washington and Seattle Children’s Research Institute, Seattle, WA USA</aff>
<aff id="Aff18">
<label></label>
Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique‐Hôpitaux de Paris, Paris, France</aff>
<aff id="Aff19">
<label></label>
Université Paris Diderot, Sorbonne Paris Cité, Paris, France</aff>
<aff id="Aff20">
<label></label>
Centre d’étude des déficits immunitaires (CEDI), Hôpital Necker‐Enfants Malades, AP-HP, Paris, France</aff>
<aff id="Aff21">
<label></label>
Department of Pediatrics, University of California San Francisco and UCSF Benioff Children’s Hospital, San Francisco, CA USA</aff>
<aff id="Aff22">
<label></label>
Division of Allergy Immunology, Department of Pediatrics, The Children’s Hospital of Philadelphia, Philadelphia, PA USA</aff>
<aff id="Aff23">
<label></label>
Murdoch Childrens Research Institute, Melbourne, VIC Australia</aff>
<aff id="Aff24">
<label></label>
Department of Paediatrics, University of Melbourne, Melbourne, VIC Australia</aff>
<aff id="Aff25">
<label></label>
Department of Allergy and Immunology, Royal Children’s Hospital, Melbourne, VIC Australia</aff>
</contrib-group>
<pub-date pub-type="epub">
<day>7</day>
<month>10</month>
<year>2015</year>
</pub-date>
<pub-date pub-type="pmc-release">
<day>7</day>
<month>10</month>
<year>2015</year>
</pub-date>
<pub-date pub-type="ppub">
<year>2015</year>
</pub-date>
<volume>35</volume>
<issue>8</issue>
<fpage>727</fpage>
<lpage>738</lpage>
<history>
<date date-type="received">
<day>11</day>
<month>8</month>
<year>2015</year>
</date>
<date date-type="accepted">
<day>16</day>
<month>9</month>
<year>2015</year>
</date>
</history>
<permissions>
<copyright-statement>© Springer Science+Business Media New York 2015</copyright-statement>
</permissions>
<abstract id="Abs1">
<p>There are now nearly 300 single-gene inborn errors of immunity underlying phenotypes as diverse as infection, malignancy, allergy, auto-immunity, and auto-inflammation. For each of these five categories, a growing variety of phenotypes are ascribed to Primary Immunodeficiency Diseases (PID), making PIDs a rapidly expanding field of medicine. The International Union of Immunological Societies (IUIS) PID expert committee (EC) has published every other year a classification of these disorders into tables, defined by shared pathogenesis and/or clinical consequences. In 2013, the IUIS committee also proposed a more user-friendly, phenotypic classification, based on the selection of key phenotypes at the bedside. We herein propose the revised figures, based on the accompanying 2015 IUIS PID EC classification.</p>
</abstract>
<kwd-group xml:lang="en">
<title>Keywords</title>
<kwd>Primary immunodeficiencies</kwd>
<kwd>classification</kwd>
<kwd>IUIS PID expert committee</kwd>
</kwd-group>
<custom-meta-group>
<custom-meta>
<meta-name>issue-copyright-statement</meta-name>
<meta-value>© Springer Science+Business Media New York 2015</meta-value>
</custom-meta>
</custom-meta-group>
</article-meta>
</front>
</pmc>
<affiliations>
<list>
<country>
<li>Allemagne</li>
<li>Australie</li>
<li>Colombie</li>
<li>France</li>
<li>Israël</li>
<li>Japon</li>
<li>Koweït</li>
<li>Maroc</li>
<li>Royaume-Uni</li>
<li>États-Unis</li>
</country>
<region>
<li>Angleterre</li>
<li>Bavière</li>
<li>Californie</li>
<li>District de Haute-Bavière</li>
<li>Grand Londres</li>
<li>Maryland</li>
<li>Massachusetts</li>
<li>Pennsylvanie</li>
<li>Victoria (État)</li>
<li>Washington (État)</li>
<li>État de New York</li>
<li>Île-de-France</li>
</region>
<settlement>
<li>Londres</li>
<li>Melbourne</li>
<li>Munich</li>
<li>Paris</li>
</settlement>
<orgName>
<li>Université de Melbourne</li>
</orgName>
</list>
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</noRegion>
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<name sortKey="Jeddane, Leila" sort="Jeddane, Leila" uniqKey="Jeddane L" first="Leïla" last="Jeddane">Leïla Jeddane</name>
</country>
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<region name="État de New York">
<name sortKey="Casanova, Jean Aurent" sort="Casanova, Jean Aurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
</region>
<name sortKey="Casanova, Jean Aurent" sort="Casanova, Jean Aurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
<name sortKey="Chatila, Talal" sort="Chatila, Talal" uniqKey="Chatila T" first="Talal" last="Chatila">Talal Chatila</name>
<name sortKey="Conley, Mary Ellen" sort="Conley, Mary Ellen" uniqKey="Conley M" first="Mary Ellen" last="Conley">Mary Ellen Conley</name>
<name sortKey="Cunningham Undles, Charlotte" sort="Cunningham Undles, Charlotte" uniqKey="Cunningham Undles C" first="Charlotte" last="Cunningham-Rundles">Charlotte Cunningham-Rundles</name>
<name sortKey="Holland, Steven M" sort="Holland, Steven M" uniqKey="Holland S" first="Steven M." last="Holland">Steven M. Holland</name>
<name sortKey="Ochs, Hans D" sort="Ochs, Hans D" uniqKey="Ochs H" first="Hans D." last="Ochs">Hans D. Ochs</name>
<name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name>
<name sortKey="Puck, Jennifer M" sort="Puck, Jennifer M" uniqKey="Puck J" first="Jennifer M." last="Puck">Jennifer M. Puck</name>
<name sortKey="Sullivan, Kathleen E" sort="Sullivan, Kathleen E" uniqKey="Sullivan K" first="Kathleen E." last="Sullivan">Kathleen E. Sullivan</name>
</country>
<country name="France">
<region name="Île-de-France">
<name sortKey="Casanova, Jean Aurent" sort="Casanova, Jean Aurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
</region>
<name sortKey="Casanova, Jean Aurent" sort="Casanova, Jean Aurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
<name sortKey="Casanova, Jean Aurent" sort="Casanova, Jean Aurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
<name sortKey="Oksenhendler, Eric" sort="Oksenhendler, Eric" uniqKey="Oksenhendler E" first="Eric" last="Oksenhendler">Eric Oksenhendler</name>
<name sortKey="Oksenhendler, Eric" sort="Oksenhendler, Eric" uniqKey="Oksenhendler E" first="Eric" last="Oksenhendler">Eric Oksenhendler</name>
<name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name>
</country>
<country name="Israël">
<noRegion>
<name sortKey="Etzioni, Amos" sort="Etzioni, Amos" uniqKey="Etzioni A" first="Amos" last="Etzioni">Amos Etzioni</name>
</noRegion>
</country>
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<noRegion>
<name sortKey="Franco, Jose Luis" sort="Franco, Jose Luis" uniqKey="Franco J" first="Jose Luis" last="Franco">Jose Luis Franco</name>
</noRegion>
</country>
<country name="Royaume-Uni">
<region name="Angleterre">
<name sortKey="Gaspar, H Bobby" sort="Gaspar, H Bobby" uniqKey="Gaspar H" first="H. Bobby" last="Gaspar">H. Bobby Gaspar</name>
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</country>
<country name="Allemagne">
<region name="Bavière">
<name sortKey="Klein, Christoph" sort="Klein, Christoph" uniqKey="Klein C" first="Christoph" last="Klein">Christoph Klein</name>
</region>
</country>
<country name="Japon">
<noRegion>
<name sortKey="Nonoyama, Shigeaki" sort="Nonoyama, Shigeaki" uniqKey="Nonoyama S" first="Shigeaki" last="Nonoyama">Shigeaki Nonoyama</name>
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<country name="Australie">
<region name="Victoria (État)">
<name sortKey="Tang, Mimi L K" sort="Tang, Mimi L K" uniqKey="Tang M" first="Mimi L. K." last="Tang">Mimi L. K. Tang</name>
</region>
</country>
</tree>
</affiliations>
</record>

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