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18 Cards in this Set
- Front
- Back
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Bruton's Agammaglobulinemia
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X linked (Boys)
B cell deficiency Defective tyrosine kinase gene low levels of all Ig's After 6 months (end passive immunity) recurrent Bacterial infections. |
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Thymic Aplasia
(DiGeorge Syndrome) |
*3rd & 4th endodermal pouches fail to develop
1. No Thymus = No Tcells 2. No Parathyroid glands = Hypocalcemia = Tetaly (Chaustic sign) Recurrent viral, fungal & protozoal infections. Often associated with ch 22q11 deletion "catch 22" = congenital heart & great vessel defects |
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Severe Combined Immunodeficiency (SCID)
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Defect in early stem cell differentiation (no B or T cell)
*Adenosine Deaminase Deficiency* Last defense = NK cells Triad: 1. Severe recurrent infections (Candidiasis, Viral dx, PCP pneumonia) 2. Chonic diarrhea 3. Failure to thrive & NO thymic shadow on child's XRay. |
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Chronic Mucocutaneous Candidiasis
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T cell dysfunction
= overgrowth and inability to kill C. albicans Treat w/ prophylactic Ketoconazole |
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Wiskott Aldrich Immunodeficiency
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WAITER.
Thrombocytopenia & purpura Eczema (Trunkal!) Recurrent pyogenic infection. No IgM against capsular polysaccharides of bacteria (G- & Listeria) low IgM, High IgA. X-linked. |
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Ataxia-Telangiectasia
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DNA repair deficiency.
IgA deficiency Cerebellar Ataxia & poor smooth pursuit of moving target with eyes Telangectasia (spider vessels) of face > 5 yrs Increased Cancer risk: - Lymphoma - acute leukemia Radiation sensitivity (avoid Xray) May have increased aFetoprotein >8 mo. Death on avg = 25yrs |
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IgA deficiency
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most common selective Ig deficit
- appear mostly healthy - sinus / lung infections more freq - asthma, allergies, etc May proceed to anaphylaxis with blood transfusion or use of blood products. |
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IL 12 Receptor deficiency
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Mycobacterial infection susceptibility.
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What disease states may be present in a child whose X-ray shows no thymic shadow?
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Severe combined immunodeficiency
DiGeorge Syndrome |
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A child with tetany, candidiasis & a history of recurrent infections has a deficiency in which cell type?
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T-Cells.
This is DiGeorge syndrome. Tetany = low Ca++ = no PTh recurrent infect & candidiasis = immunosupression & lack of t-cells from thymic aplasia |
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2 year old boy with recurrent lung infections & granulomatous disease. What is the defect in his his neutrophils?
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Deficient in NADPH oxidase.
Susceptible to catalase+ infections. Chronic Granulomatous Disease. |
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Child with a history of frequent and recurrent viral and fungal infections. Blood work states child is hypocalcemic. What is the germ cell responsible for the missing structure?
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Endoderm.
DiGeorge Syndrome = Thymic aplasia - missing 3rd and 4th pouches. |
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Immune disorder in children with repeat cold Staphylococcus abscesses.
What is deficient/improperly working in this child? what is the disease? |
Helper T cells (type 1) are not producing IFN gamma.
PMN's are not able to chemotaxis in response to C5a, LTB4 or IL8. Therefore abcesses are cold because inflammation cannot occur. Hyper IgE syndrom or Job's synd. |
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Chronic Granulomatous Dx
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Lack of NADPH oxidase activity = impotent phagocytes.
(can engulf bacteria but no O free radicals) Susceptible to Catalase + organisms. Diagnosis with Nitroblue Tetrazolium dye test (negative = yellow dye is not oxidized to blue-black = no free radicals) Treat with prophylactic TMP-SMX or INF-g |
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Name 5 micro-organisms that pts with
Chronic Granulomatous Disease are particularly suscepitble to. |
Staph Aureus
E. Coli Klebsiella Aspergillus Candida |
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Chediak Higashi Disease
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Defective (lysosomal transport) gene.
= defect phagosyte lysosome = giant cytoplasmic granules in pmn. Triad: - partial albinism - recurrent respiratory and skin infections - neurologic disorders. |
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Hyper IgE syndrome
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(Job syndrome)
Deficient INF-g = PMNs fail to respond to chemotaxis stim (C5a, IL8, LT B4) High IgE, lots of eosinophils Triad: Eczema Recurrent cold Staph abscesses (boils) Course facial features & Retained primary teeth = 2 rows of teeth. |
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leukocyte adhesion deficiency syndrome
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Abnormal integrins = ina bility of phagocyte to exit circulation (and integrate with tissue)
Delayed seperation of umbilicus |
