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30 Cards in this Set
- Front
- Back
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B cell deficiency in boys
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Brutons aka X linked agammaglobulinemia
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Brutons agammaglobinemia, Diagnosis
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Quantitative immunoglobulin levels; absent b cells, high T cells
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Brutons agammaglobinemia, Presentation
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*Recurrent infections, (s. pneumonia, hib, and pseudomonas) after SIX MONTHS
*Absent tonsils/other lymphoid tissue |
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Brutons agammaglobinemia, Treatment
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Prophylactic antibiotics
IVIG |
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Combined B and T cell defect
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Combined variable immunodeficiency
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Common Variable immunodeficiency, presentation
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Increase pyogenic upper and lower respiratory infections
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Common variable immunodeficiency, diagnosis
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quantitative immunoglobulin levels, decreased b and t cell
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Common variable immunodeficiency, treatment
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Treat with IVIG
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Most common immunodeficiency
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IgA deficiency
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IgA deficiency, presentation
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Usually asymptommatic;
Pts may develop recurrent respiratory or GI infections Anaphylactic transfusion reaction due to anti-IgA antibodies |
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IgA deficiency, treatment
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Treat infection
do NOT give immunoglobulins |
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DiGeorge Presentation
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C ardiac Abnormality (especially tetralogy of Fallot)
A bnormal facies T hymic aplasia C left palate H ypocalcemia. Delayed hypersensitivity skin testing |
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DiGeorge, infections
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Fungi
pneumocystis jiroveci |
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DiGeorge, treatment
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PCP prophylaxis
Bone Marrow Transplant IVIG Thymus transplant! |
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Defect in the ATM gene,for recognizing and correcting errors in duplicating DNA when cells divide, and in destroying the cells when the errors can't be corrected
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Ataxia-Telangiectasia
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Ataxia-Telangiectasia, Presentation
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*Oculucocutaneous telangiectasia
*Progressive cerebellar ataxia |
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Ataxia-Telangiectasia, Risks
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Increased incidence of non-Hodgkin's lymphoma
Leukemia Gastric Carcinoma |
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Ataxia-Telangiectasia, Treatment
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No specific treatment
May require IvIG |
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Severe lack of B and T cells
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Severe combined immunodeficiency (SCID)
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SCID, presentation
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*Severe, frequent bacterial infections
*Chronic Candiasis *Opportunistic organisms |
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SCID, treatment
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*Bone marrow transplant
*Stem Cell transplant *IVIG for antibody deficiency ****NEEDS PCP PROPHYLAXIS***** |
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X linked disorder with less severe B and T cell dysfunction
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Wiskott-Aldrich syndrome
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Wiskott-Aldrich syndrome, presentation
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*Eczema
*Bleeding *Recurrent otitis media |
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Wiskott-Aldrich syndrome, Infection/risk
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*Increase risk of atopic disorders
*Lymphoma/leukemia *S. Pneumonia, S. Aureus, HIB |
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Wiskott-Aldrich syndrome, Treatment
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Supportive: IVIG/ABX,
Severe-bone marrow transplant Don't usually live all that long |
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Wiskott-Aldrich, Diagnosis
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*Increased IgE/IgA
*Decreased IgM |
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Chronic Granulomatous Disease, Treatment
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*Daily with TMP-SMX
*Judicious use of ABX during infection *INF gamma can decrease incidence (newer therapies- bone marrow transplant and gene therapy) |
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CGD presentation
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Anemia
Lymphadenopathy hypergammaglobulinemia chronic SKIN, PULMONARY, GI, and UTI infections Granulomas of the skin Increased Risk of Aspergiluus |
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Leukocyte Adhesion deficiency, treatment
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Bone Marrow transplant is curative!!!!
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LAD, presentation
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No pus with minimal inflammation in wounds
Leukocytosis Omphalitis in the newborn; delayed separation of the umbilical cord |
