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51 Cards in this Set
- Front
- Back
What immunodeficiency is present in all infants at 3-6 months?
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Transient Hypogammaglobulinemia due to catbolism of placentally transferred IgG
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B cell tyrosine kinase (btk) lesion
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X-linked Agammaglobulinemia of Bruton
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Failure of B-cell precursors to mature into B cells
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X-linked Agammaglobulinemia of Bruton
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Heavy chains, but NOT light chains of Ab are formed
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X-linked Agammaglobulinemia of Bruton
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When does X-linked Agammaglobulinemia of Bruton manifest?
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6 months (maternal Igs have been depleted)
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Bacterial infections of the URT/LRT of what are common in X-linked Agammaglobulinemia of Bruton?
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H. influenzae, S. pneumoniae, S. aureus
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Why do H. influenzae, S. pneumoniae, S. aureus cause infections in X-linked Agammaglobulinemia of Bruton?
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They are encapsulated bacteria that need to be opsonized by Ab to be removed. Without Ab, no opsonization.
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What viruses, not handled well by T cells, may cause a problem in X-linked Agammaglobulinemia of Bruton?
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Enteroviruses (echo, polio, coxsackie). They require Ab to neutralize them.
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In what diseases may Giardia lamblia cause persistent infections, and why?
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X-linked Agammaglobulinemia of Bruton because there is an absence of secreted IgA and in CVID
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Mycoplasma arthritis in kids
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X-linked Agammaglobulinemia of Bruton
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Normal CD19, but no surface Igs
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X-linked Agammaglobulinemia of Bruton
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Rudimentary or underdeveloped germinal centers
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X-linked Agammaglobulinemia of Bruton
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ZERO plasma cells
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X-linked Agammaglobulinemia of Bruton
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What makes CVID different from Bruton?
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CVID is a later onset, normal # of B cells, and affects both sexes =
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B cells can proliferate in response to antigen, but do not produce antibodies
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CVID
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Infections similar to Bruton, with herpetic also
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CVID
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Enteroviral infections causing meningoecncephalitis
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CVID
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50 fold > in gastric carcinoma
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CVID
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Fairly common ID
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Isolated IgA
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Post-infectious (measles or toxoplasmosis)
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Isolated IgA
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What are the sx in Isolated IgA?
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Most are asymptomatic
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Where do you see infections in Isolated IgA?
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tubes
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High frequency of respiratory allergies
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Isolated IgA (lack of IgA in mucous secretions)
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CD40 is on what cell?
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B cell
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What Igs are on all naive B cells?
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M and D
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What is the cause for Hyper IgM
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Abnormal T cells fail to induce B cells to make Ab other than IgM
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What is the mutation in Hyper IgM?
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CD40L on Xq26
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Why are there pyogenic infections in Hyper IgM?
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opsonizing IgG is low
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P. jiroveci pneumonia
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Hyper IgM and SCID
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DiGeorge Syndrome is the failure of what to develop?
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3rd, 4th pharyngeal pouches
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What is the consequence of thymic agenesis in DiGeorge Syndrome?
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poor defense against fungal and viral infections
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Tetany
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DiGeorge Syndrome (parathyroid agenesis)
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Facial anomalies (big nose, cleft palate, retrognathia)
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DiGeorge Syndrome
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Is DiGeorge Syndrome a familial disorder?
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NO
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Where is the deletion in DiGeorge Syndrome?
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22q11
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CATCH (DiGeorge)
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Cardiac abnormality
Abnormal facies Thymic/T cell deficit Cleft Palate Hypocalcemia |
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What would the plasma cell level in lymphoid tissue be in DiGeorge Syndrome?
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Normal
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What would the Ig level be in DiGeorge Syndrome?
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Normal to reduced (depending upon the severity of T cell (CD4) deficiency
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Where would you see lymphoid depletion in DiGeorge Syndrome?
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Paractorical region of LN, periarteriolar sheaths of spleen
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Where is there always hypoplasia in SCID?
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T cell zone (may be B cell zone too)
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Defects in both humoral and cell-mediated immune responses
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SCID
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oral thrush, diaper rashes, failure to thrive
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SCID
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Rash on a newborn due to GVH
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SCID
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Gamma-c SCID has a defect in a receptor for what? (Most important)
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IL-7 (required for the lymphoid progenitors, particularily T cells)
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What does an ADA deficiency produce?
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accumulation of deoxyadenosine which is toxic to immature lymphocytes, esp T cells
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What is the Dx triad of Wiskott-Aldrich?
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Thrombocytopenia, eczema and recurrent infections (early death)
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What would the serum Ig level in WA?
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Low IgM, normal IgG, IgA and IgE > some
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What is the significance of a low IgM in WA?
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poor response to bacterial polysaccharides
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Do not make abys to bacterial polysaccharide antigens?
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WA
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Where is the lesion in WA?
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Xp 11.23 --> No WASP (CD43)
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Those with WA have a tendency to develop what?
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lymphomas
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