Since the original description of X-linked agammaglobulinemia in 1952, the number of independent primary immunodeficiency diseases (PIDs) has expanded to more than 100 entities. By definition, a PID is a genetically determined disorder resulting in enhanced susceptibility to infectious disease. Despite the heritable nature of these diseases, some PIDs are clinically manifested only after prerequisite environmental exposures but they often have associated malignant, allergic, or autoimmune manifestations. PIDs must be distinguished from secondary or acquired immunodeficiencies, which are far more common. In this review, we will place these immunodeficiencies in the context of both clinical and laboratory presentations as well as highlight the known genetic basis.
机构:
Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, IranUniv Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Aghamohammadi, A
Moin, M
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机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Moin, M
Farhoudi, A
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机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Farhoudi, A
Rezaei, N
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机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Rezaei, N
Pourpak, Z
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机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Pourpak, Z
Movahedi, M
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机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Movahedi, M
Gharagozlou, M
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机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Gharagozlou, M
Nabavi, M
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机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Nabavi, M
Shahrokhi, A
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机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Shahrokhi, A
[J].
FEMS IMMUNOLOGY AND MEDICAL MICROBIOLOGY,
2004,
40
(02):
: 113
-
118
机构:
Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, IranUniv Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Aghamohammadi, A
Moin, M
论文数: 0引用数: 0
h-index: 0
机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Moin, M
Farhoudi, A
论文数: 0引用数: 0
h-index: 0
机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Farhoudi, A
Rezaei, N
论文数: 0引用数: 0
h-index: 0
机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Rezaei, N
Pourpak, Z
论文数: 0引用数: 0
h-index: 0
机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Pourpak, Z
Movahedi, M
论文数: 0引用数: 0
h-index: 0
机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Movahedi, M
Gharagozlou, M
论文数: 0引用数: 0
h-index: 0
机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Gharagozlou, M
Nabavi, M
论文数: 0引用数: 0
h-index: 0
机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Nabavi, M
Shahrokhi, A
论文数: 0引用数: 0
h-index: 0
机构:Univ Tehran Med Sci, Immunol Asthma & Allergy Res Inst, Childrens Med Ctr, Dept Allergy, Tehran, Iran
Shahrokhi, A
[J].
FEMS IMMUNOLOGY AND MEDICAL MICROBIOLOGY,
2004,
40
(02):
: 113
-
118