The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies
Identifieur interne : 002770 ( Main/Exploration ); précédent : 002769; suivant : 002771The 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 :
- Journal of Clinical Immunology [ 0271-9142 ] ; 2015.
Descripteurs français
- KwdFr :
- MESH :
- génétique : Immunité.
- immunologie : Déficits immunitaires, Hypersensibilité, Infection, Inflammation, Tumeurs.
- Auto-immunité, Déficits immunitaires, Expertise, Humains, Phénotype.
English descriptors
- KwdEn :
- MESH :
- classification : Immunologic Deficiency Syndromes.
- genetics : Immunity.
- immunology : Hypersensitivity, Immunologic Deficiency Syndromes, Infection, Inflammation, Neoplasms.
- Autoimmunity, Expert Testimony, Humans, Phenotype.
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:
- Allemagne, Australie, Colombie, France, Israël, Japon, Koweït, Maroc, Royaume-Uni, États-Unis
- Angleterre, Bavière, Californie, District de Haute-Bavière, Grand Londres, Maryland, Massachusetts, Pennsylvanie, Victoria (État), Washington (État), État de New York, Île-de-France
- Londres, Melbourne, Munich, Paris
- Université de Melbourne
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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></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">26445875</idno><idno type="pmc">4659854</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4659854</idno><idno type="RBID">PMC:4659854</idno><idno type="doi">10.1007/s10875-015-0198-5</idno><date when="2015">2015</date><idno type="wicri:Area/Pmc/Corpus">000D45</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000D45</idno><idno type="wicri:Area/Pmc/Curation">000C06</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">000C06</idno><idno type="wicri:Area/Pmc/Checkpoint">000C91</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">000C91</idno><idno type="wicri:source">PubMed</idno><idno type="wicri:Area/PubMed/Corpus">002773</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">002773</idno><idno type="wicri:Area/PubMed/Curation">002704</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">002704</idno><idno type="wicri:Area/PubMed/Checkpoint">002704</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">002704</idno><idno type="wicri:Area/Ncbi/Merge">002B91</idno><idno type="wicri:Area/Ncbi/Curation">002B91</idno><idno type="wicri:Area/Ncbi/Checkpoint">002B91</idno><idno type="wicri:doubleKey">0271-9142:2015:Bousfiha A:the:iuis:phenotypic</idno><idno type="wicri:Area/Main/Merge">002770</idno><idno type="wicri:Area/Main/Curation">002770</idno><idno type="wicri:Area/Main/Exploration">002770</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies</title><author><name sortKey="Bousfiha, Aziz" sort="Bousfiha, Aziz" uniqKey="Bousfiha A" first="Aziz" last="Bousfiha">Aziz Bousfiha</name><affiliation wicri:level="1"><nlm:aff id="Aff1">Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco</nlm:aff><country xml:lang="fr">Maroc</country><wicri:regionArea>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca</wicri:regionArea><wicri:noRegion>Casablanca</wicri:noRegion></affiliation></author><author><name sortKey="Jeddane, Leila" sort="Jeddane, Leila" uniqKey="Jeddane L" first="Leïla" last="Jeddane">Leïla Jeddane</name><affiliation wicri:level="1"><nlm:aff id="Aff1">Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco</nlm:aff><country xml:lang="fr">Maroc</country><wicri:regionArea>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca</wicri:regionArea><wicri:noRegion>Casablanca</wicri:noRegion></affiliation></author><author><name sortKey="Al Herz, Waleed" sort="Al Herz, Waleed" uniqKey="Al Herz W" first="Waleed" last="Al-Herz">Waleed Al-Herz</name><affiliation wicri:level="1"><nlm:aff id="Aff2">Department of Pediatrics, Faculty of Medicine Kuwait University, Jabriya, Kuwait</nlm:aff><country xml:lang="fr">Koweït</country><wicri:regionArea>Department of Pediatrics, Faculty of Medicine Kuwait University, Jabriya</wicri:regionArea><wicri:noRegion>Jabriya</wicri:noRegion></affiliation><affiliation wicri:level="1"><nlm:aff id="Aff3">Allergy and Clinical Immunology Unit, Department of Pediatrics, Al-Sabah Hospital, Kuwait City, Kuwait</nlm:aff><country xml:lang="fr">Koweït</country><wicri:regionArea>Allergy and Clinical Immunology Unit, Department of Pediatrics, Al-Sabah Hospital, Kuwait City</wicri:regionArea><wicri:noRegion>Kuwait City</wicri:noRegion></affiliation></author><author><name sortKey="Ailal, Fatima" sort="Ailal, Fatima" uniqKey="Ailal F" first="Fatima" last="Ailal">Fatima Ailal</name><affiliation wicri:level="1"><nlm:aff id="Aff1">Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco</nlm:aff><country xml:lang="fr">Maroc</country><wicri:regionArea>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca</wicri:regionArea><wicri:noRegion>Casablanca</wicri:noRegion></affiliation></author><author><name sortKey="Casanova, Jean Aurent" sort="Casanova, Jean Aurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</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><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="Aff6">Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Necker Hospital for Sick Children, Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Necker Hospital for Sick Children, 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="Aff7">Imagine Institute, University Paris Descartes, Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Imagine Institute, University Paris Descartes, 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="Aff8">Pediatric Hematology & Immunology Unit, Necker Hospital for Sick Children, Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Pediatric Hematology & Immunology Unit, Necker Hospital for Sick Children, 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="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><keywords scheme="KwdEn" xml:lang="en"><term>Autoimmunity</term><term>Expert Testimony</term><term>Humans</term><term>Hypersensitivity (immunology)</term><term>Immunity (genetics)</term><term>Immunologic Deficiency Syndromes (classification)</term><term>Immunologic Deficiency Syndromes (immunology)</term><term>Infection (immunology)</term><term>Inflammation (immunology)</term><term>Neoplasms (immunology)</term><term>Phenotype</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Auto-immunité</term><term>Déficits immunitaires ()</term><term>Déficits immunitaires (immunologie)</term><term>Expertise</term><term>Humains</term><term>Hypersensibilité (immunologie)</term><term>Immunité (génétique)</term><term>Infection (immunologie)</term><term>Inflammation (immunologie)</term><term>Phénotype</term><term>Tumeurs (immunologie)</term></keywords><keywords scheme="MESH" qualifier="classification" xml:lang="en"><term>Immunologic Deficiency Syndromes</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Immunity</term></keywords><keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Immunité</term></keywords><keywords scheme="MESH" qualifier="immunologie" xml:lang="fr"><term>Déficits immunitaires</term><term>Hypersensibilité</term><term>Infection</term><term>Inflammation</term><term>Tumeurs</term></keywords><keywords scheme="MESH" qualifier="immunology" xml:lang="en"><term>Hypersensitivity</term><term>Immunologic Deficiency Syndromes</term><term>Infection</term><term>Inflammation</term><term>Neoplasms</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Autoimmunity</term><term>Expert Testimony</term><term>Humans</term><term>Phenotype</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Auto-immunité</term><term>Déficits immunitaires</term><term>Expertise</term><term>Humains</term><term>Phénotype</term></keywords></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><div1 type="bibliography"><listBibl><biblStruct></biblStruct><biblStruct><analytic><author><name sortKey="Bousfiha, Aa" uniqKey="Bousfiha A">AA Bousfiha</name></author><author><name sortKey="Jeddane, L" uniqKey="Jeddane L">L Jeddane</name></author><author><name sortKey="Ailal, F" uniqKey="Ailal F">F Ailal</name></author><author><name sortKey="Benhsaien, I" uniqKey="Benhsaien I">I Benhsaien</name></author><author><name sortKey="Mahlaoui, N" uniqKey="Mahlaoui N">N Mahlaoui</name></author><author><name sortKey="Casanova, Jl" uniqKey="Casanova J">JL Casanova</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Ciancanelli, Mj" uniqKey="Ciancanelli M">MJ Ciancanelli</name></author><author><name sortKey="Huang, Sx" uniqKey="Huang S">SX Huang</name></author><author><name sortKey="Luthra, P" uniqKey="Luthra P">P Luthra</name></author><author><name sortKey="Garner, H" uniqKey="Garner H">H Garner</name></author><author><name sortKey="Itan, Y" uniqKey="Itan Y">Y Itan</name></author><author><name sortKey="Volpi, S" uniqKey="Volpi S">S Volpi</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Troedson, C" uniqKey="Troedson C">C Troedson</name></author><author><name sortKey="Wong, M" uniqKey="Wong M">M Wong</name></author><author><name sortKey="Dalby Payne, J" uniqKey="Dalby Payne J">J Dalby-Payne</name></author><author><name sortKey="Wilson, M" uniqKey="Wilson M">M Wilson</name></author><author><name sortKey="Dexter, M" uniqKey="Dexter M">M Dexter</name></author><author><name sortKey="Rice, Gi" uniqKey="Rice G">GI Rice</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Sewell, Gw" uniqKey="Sewell G">GW Sewell</name></author><author><name sortKey="Rahman, Fz" uniqKey="Rahman F">FZ Rahman</name></author><author><name sortKey="Levine, Ap" uniqKey="Levine A">AP Levine</name></author><author><name sortKey="Jostins, L" uniqKey="Jostins L">L Jostins</name></author><author><name sortKey="Smith, Pj" uniqKey="Smith P">PJ Smith</name></author><author><name sortKey="Walker, Ap" uniqKey="Walker A">AP Walker</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Bousfiha, Aa" uniqKey="Bousfiha A">AA Bousfiha</name></author><author><name sortKey="Jeddane, L" uniqKey="Jeddane L">L Jeddane</name></author><author><name sortKey="Ailal, F" uniqKey="Ailal F">F Ailal</name></author><author><name sortKey="Al Herz, W" uniqKey="Al Herz W">W Al Herz</name></author><author><name sortKey="Conley, Me" uniqKey="Conley M">ME Conley</name></author><author><name sortKey="Cunningham Rundles, C" uniqKey="Cunningham Rundles C">C Cunningham-Rundles</name></author></analytic></biblStruct><biblStruct></biblStruct><biblStruct><analytic><author><name sortKey="Ochs, Hd" uniqKey="Ochs H">HD Ochs</name></author><author><name sortKey="Hagin, D" uniqKey="Hagin D">D Hagin</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Federici, S" uniqKey="Federici S">S Federici</name></author><author><name sortKey="Martini, A" uniqKey="Martini A">A Martini</name></author><author><name sortKey="Gattorno, M" uniqKey="Gattorno M">M Gattorno</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Modell, V" uniqKey="Modell V">V Modell</name></author><author><name sortKey="Knaus, M" uniqKey="Knaus M">M Knaus</name></author><author><name sortKey="Modell, F" uniqKey="Modell F">F Modell</name></author><author><name sortKey="Roifman, C" uniqKey="Roifman C">C Roifman</name></author><author><name sortKey="Orange, J" uniqKey="Orange J">J Orange</name></author><author><name sortKey="Notarangelo, Ld" uniqKey="Notarangelo L">LD Notarangelo</name></author></analytic></biblStruct><biblStruct></biblStruct></listBibl></div1></back></TEI><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><tree><country name="Maroc"><noRegion><name sortKey="Bousfiha, Aziz" sort="Bousfiha, Aziz" uniqKey="Bousfiha A" first="Aziz" last="Bousfiha">Aziz Bousfiha</name></noRegion><name sortKey="Ailal, Fatima" sort="Ailal, Fatima" uniqKey="Ailal F" first="Fatima" last="Ailal">Fatima Ailal</name><name sortKey="Jeddane, Leila" sort="Jeddane, Leila" uniqKey="Jeddane L" first="Leïla" last="Jeddane">Leïla Jeddane</name></country><country name="Koweït"><noRegion><name sortKey="Al Herz, Waleed" sort="Al Herz, Waleed" uniqKey="Al Herz W" first="Waleed" last="Al-Herz">Waleed Al-Herz</name></noRegion><name sortKey="Al Herz, Waleed" sort="Al Herz, Waleed" uniqKey="Al Herz W" first="Waleed" last="Al-Herz">Waleed Al-Herz</name></country><country name="États-Unis"><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><country name="Colombie"><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></region></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></noRegion></country><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>Pour manipuler ce document sous Unix (Dilib)
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