TY - JOUR
T1 - Primary immunodeficiencies
T2 - 2009 update
AU - Notarangelo, Luigi D.
AU - Fischer, Alain
AU - Geha, Raif S.
AU - Casanova, Jean Laurent
AU - Chapel, Helen
AU - Conley, Mary Ellen
AU - Cunningham-Rundles, Charlotte
AU - Etzioni, Amos
AU - Hammartröm, Lennart
AU - Nonoyama, Shigeaki
AU - Ochs, Hans D.
AU - Puck, Jennifer
AU - Roifman, Chaim
AU - Seger, Reinhard
AU - Wedgwood, Josiah
PY - 2009/12
Y1 - 2009/12
N2 - 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.
AB - 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.
KW - B cells
KW - DNA repair defects
KW - Primary immunodeficiencies
KW - T cells
KW - autoinflammatory disorders
KW - complement
KW - immune dysregulation syndromes
KW - innate immunity
KW - phagocytes
KW - predominantly antibody deficiencies
KW - severe combined immunodeficiency
UR - http://www.scopus.com/inward/record.url?scp=71449120471&partnerID=8YFLogxK
U2 - 10.1016/j.jaci.2009.10.013
DO - 10.1016/j.jaci.2009.10.013
M3 - Article
C2 - 20004777
AN - SCOPUS:71449120471
SN - 0091-6749
VL - 124
SP - 1161
EP - 1178
JO - Journal of Allergy and Clinical Immunology
JF - Journal of Allergy and Clinical Immunology
IS - 6
ER -