title: Primary immunodeficiencies: 2009 update.
abstract: More than 50 years after Ogdeon Bruton's discovery of congenital agammaglobulinemia, human primary immunodeficiencies (PIDs) continue to unravel novel molecular and cellular mechanisms that govern development and function of the human immune system. This report provides the updated classification of PIDs that has been compiled by the International Union of Immunological Societies Expert Committee on Primary Immunodeficiencies after its biannual meeting in Dublin, Ireland, in June 2009. Since the appearance of the last classification in 2007, novel forms of PID have been discovered, and additional pathophysiology mechanisms that account for PID in human beings have been unraveled. Careful analysis and prompt recognition of these disorders is essential to prompt effective forms of treatment and thus to improve survival and quality of life in patients affected with PIDs.
authors.affiliation: Division of Immunology, Children's Hospital Boston and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA. luigi.notarangelo@childrens.harvard.edu
authors.forename: Luigi D
authors.initials: LD
authors.lastname: Notarangelo
authors.forename: Alain
authors.initials: A
authors.lastname: Fischer
authors.forename: Raif S
authors.initials: RS
authors.lastname: Geha
authors.forename: Jean-Laurent
authors.initials: JL
authors.lastname: Casanova
authors.forename: Helen
authors.initials: H
authors.lastname: Chapel
authors.forename: Mary Ellen
authors.initials: ME
authors.lastname: Conley
authors.forename: Charlotte
authors.initials: C
authors.lastname: Cunningham-Rundles
authors.forename: Amos
authors.initials: A
authors.lastname: Etzioni
authors.forename: Lennart
authors.initials: L
authors.lastname: Hammartröm
authors.forename: Shigeaki
authors.initials: S
authors.lastname: Nonoyama
authors.forename: Hans D
authors.initials: HD
authors.lastname: Ochs
authors.forename: Jennifer
authors.initials: J
authors.lastname: Puck
authors.forename: Chaim
authors.initials: C
authors.lastname: Roifman
authors.forename: Reinhard
authors.initials: R
authors.lastname: Seger
authors.forename: Josiah
authors.initials: J
authors.lastname: Wedgwood
mesh_terms: D015551:Autoimmunity / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology / Q000378:metabolism; D003165:Complement System Proteins / Q000276:immunology* / Q000378:metabolism; D016207:Cytokines / Q000276:immunology / Q000378:metabolism; D006801:Humans; D007113:Immunity, Innate; D007136:Immunoglobulins / Q000097:blood; D007153:Immunologic Deficiency Syndromes / Q000145:classification* / Q000276:immunology* / Q000628:therapy; D007694:Killer Cells, Natural / Q000276:immunology / Q000378:metabolism; D013601:T-Lymphocytes / Q000276:immunology / Q000378:metabolism
chemical_list: D016207:Cytokines; D007136:Immunoglobulins; D003165:Complement System Proteins