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15 Cards in this Set
- Front
- Back
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BRUTON'S AGAMMAGLOBULINEMIA
- inheritance? - susceptible population group? |
- XLR
- Boys (after 6 months) |
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BRUTON'S AGAMMAGLOBULINEMIA
- Defect in what gene? - What kind of gene is this? - Defect has what effect on Immune? |
- BTK
- Tyrosine Kinase gene Blocks B-cell - Maturation - Differentiation |
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What Dz causes Recurrent Bacterial Infections AFTER 6 months of age?
Why after 6 months? What process is defective? |
- Bruton's Agammaglobulinemia
- Decrease/loss of Maternal IgG - Opsonization defect |
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BRUTON'S AGAMMAGLOBULINEMIA
- Labs show what normal? - Labs how what abnormal? x3 |
Normal = Progenitor B-cells
Abnormal Decrease - Maturation of B-cells - Number of B-cells - Immunoglobulins of ALL classes |
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Hyper-IgM Syndrome
- what is the etiology defect? - defect is seen on what cells? - defect has what effect on Immune? |
- Defective CD40 Ligand
- on Helper T-cells - Inability to class switch |
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What Dz presents with Severe PYOGENIC infections Early in Life?
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- Hyper-IgM syndrome
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Hyper-IgM Syndrome
- Labs show what Normal? - Labs show what Abnormal? |
Abnormal Increase
- IgM Abnormal Decrease - IgA - IgG - IgE |
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Selective Ig Deficiency
- what is the etiology defect? - defect has what effect on Immune? |
- Defect in Isotype switching
- Deficiency in Specific Class of Ig |
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Selective Ig Deficiency
- possible presentations |
- Sinus Infections
- Lung Infections - Allergy to MILK & Diarrhea - Anaphylaxis on exposure to Blood products with IgA |
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What Dz may present with Anaphylaxis on exposure to Blood Products with IgA?
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- Selective IgA Deficiency
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Selective Ig Deficiency
- Labs show what Normal? - Labs show what Abnormal? |
Abnormal Decrease
- specific Immunoglobulin |
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Selective Ig Deficiency
- which type is MC - this MC type affects what cell & how? - causes what immune effect? |
- Selective IgA deficiency
- B-cell Failure to Mature into Plasma cells - Decreased Secretory IgA |
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Common Variable Immunodeficiency (CVID)
- etiology defect? - defect has what effect on immune |
- many causes
- Defect in B-cell MATURATION |
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Common Variable Immunodeficiency (CVID)
- can be acquired commonly in what population group? - causes an increased risk in what Dz's? x3 |
- 20's to 30's
- Autoimmune Dz - Lymphoma - Sinopulmonary infections |
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Common Variable Immunodeficiency (CVID)
- Labs show normal what? - Labs show abnormal what? |
Normal
- number of B-cells Abnormal Decrease - Plasma cells - Immunoglobulins |
