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7. Categories of Immune Disorders
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7.3 Deficiency states/Immunodeficiency Syndromes
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7.3.1 Primary immunodeficiencies
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Primary (B-cell) antibody deficiency
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Agammaglobulinemia of Bruton
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- X-linked
- Restricted to males
- Virtual absence of B-cells and immunoglobins
from serum
- Small amounts of IgG may be seen
- Normal number of pre-B cells in marrow
- Abnormal maturation
- Severe recurrent infections seen
begining ~ 8 months of age
- especially pyogenic organisms
- Staphylococcus
- Haemophilus
infuenzae
- Conjunctivitis
- Pharyngitis
- Otitis media
- Bronchitis
- Pneumonia
- Skin infections
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