Human Primary Immunodeficiencies

作者: R.L. Fuleihan

DOI: 10.1016/B978-0-12-801238-3.00122-7

关键词:

摘要: Primary immune deficiency diseases are inherited resulting from genetic defects that affect the development or function of one more components system, leading to an increased susceptibility infection and, in some cases, cancer, autoimmune allergic disease. The main system is protection against invading microorganisms (bacteria, viruses, fungi, and parasites) cause disease (pathogenic pathogens). In addition, it must destroy any body's own cells develop into cancer. alter result enhanced inability kill cancer a dysregulation These immunodeficiency demonstrate importance each altered system. This article describes known diseases, which illustrates role different molecules cell populations immunity.

参考文章(60)
Hans D. Ochs, Mary Cosyns, Luigi Notarangelo, Jacov Levy, Fabio Facchetti, Adriana Weinberg, Anthony R. Hayward, Jean Yves Bonnefoy, Amos Etzioni, Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM. Journal of Immunology. ,vol. 158, pp. 977- 983 ,(1997)
Alexandre Alcaïs, Lluis Quintana‐Murci, David S Thaler, Erwin Schurr, Laurent Abel, Jean‐Laurent Casanova, None, Life-threatening infectious diseases of childhood: single-gene inborn errors of immunity? Annals of the New York Academy of Sciences. ,vol. 1214, pp. 18- 33 ,(2010) , 10.1111/J.1749-6632.2010.05834.X
Federico Serana, Alessandra Sottini, Marco Chiarini, Cinzia Zanotti, Claudia Ghidini, Arnalda Lanfranchi, Lucia Dora Notarangelo, Luigi Caimi, Luisa Imberti, The different extent of B and T cell immune reconstitution after hematopoietic stem cell transplantation and enzyme replacement therapies in SCID patients with adenosine deaminase deficiency. Journal of Immunology. ,vol. 185, pp. 7713- 7722 ,(2010) , 10.4049/JIMMUNOL.1001770
Fred S. Rosen, Sherwin V. Kevy, David Gitlin, Charles A. Janeway, Ezio Merler, RECURRENT BACTERIAL INFECTIONS AND DYSGAMMAGLOBULINEMIA: DEFICIENCY OF 7S GAMMA-GLOBULINS IN THE PRESENCE OF ELEVATED 19S GAMMA-GLOBULINS Report of Two Cases Pediatrics. ,vol. 28, pp. 182- 195 ,(1961)
Nicole M. Chase, James W. Verbsky, John M. Routes, Newborn screening for SCID: three years of experience Annals of the New York Academy of Sciences. ,vol. 1238, pp. 99- 105 ,(2011) , 10.1111/J.1749-6632.2011.06241.X
Donna L Mallery, Leo C James, William A McEwan, Jerry C H Tam, Ruth E Watkinson, Susanna R Bidgood, Intracellular antibody-bound pathogens stimulate immune signaling via the Fc receptor TRIM21 Nature Immunology. ,vol. 14, pp. 327- 336 ,(2013) , 10.1038/NI.2548
Ruslan Medzhitov, Charles A Janeway, Innate Immunity: The Virtues of a Nonclonal System of Recognition Cell. ,vol. 91, pp. 295- 298 ,(1997) , 10.1016/S0092-8674(00)80412-2
Alain Fischer, Salima Hacein-Bey-Abina, Marina Cavazzana-Calvo, Gene therapy for primary adaptive immune deficiencies The Journal of Allergy and Clinical Immunology. ,vol. 127, pp. 1356- 1359 ,(2011) , 10.1016/J.JACI.2011.04.030
Maria Garcia-Lloret, Sean McGhee, Talal A. Chatila, Immunoglobulin Replacement Therapy in Children Immunology and Allergy Clinics of North America. ,vol. 28, pp. 833- 849 ,(2008) , 10.1016/J.IAC.2008.07.001