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19 Cards in this Set
- Front
- Back
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Herditary Angioedema
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C1 esterase inhibitor deficiency
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WHat is contraind in C1 esterase inhib def?
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Ace Inhib - Bradykinin is high
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Sinus and URI, Sysceptible to TYpe 3 HSRx like glomerulonephritis
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C3 deficiency
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Recurrent Neisseria bacteremia
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C5-9 deficiency
Can't form MAC |
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PNH
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DAF GPI anchored enzyme deficiency
COmplement mediated lysis of RBCs |
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Recurrent Bacterial infections, decrease immunoglobulins of all classes, No germinal centers
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X-Linked Bruton's Agammaglobulinemia
- Defect in BTK tyrosine kinase gene - decreased B cell maturation |
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Sinopulmonary infections, GI infections, autoimmune disease, anaphylaxis to blood transfusion
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Selective IgA deficiency
- also false pos BHCG test |
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20-30 y.o. with autoimmune disease, lymphoma, sinopulmonary infections, decreased plasma cell and immunoglobulin
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CVID
- defect in B cell maturation |
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Cleft lip, Absent thymic shadow, Tetany, congenital heart defect, recurrent viral and fungal infections
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DiGeorge
- Thymus and parathyroids fail to develop from 3rd and 4th pharyngeal pouches |
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Disseminated mycobacterial infection, Decreased IFN-y
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IL-12 receptor deficiency
- Decrease TH1 response --> decrease IFN-y --> decrease macrophages |
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2 rows of teeth, Abscesses, increased IgE, Ezcema
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Job's / Hyper IgE SYndrome
- TH1 cells don't produce IFN-y --> Neutrophils can't respond to chemotactic stimuli |
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Recurrent candida infections
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Chronic Mucocutaneous Candiasis
- T cell dysfunction |
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FAilure to thrive, DIarrhea, Recurrent viral, bacterial, fungal, and protozoal infection.
Absent thymic shadow and germinal centers. |
SCID
- Adenosine deaminase deficiency - treat with bone marrow transplant |
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Ataxia, Spider angiomas, High AFP, low IgA
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Ataxia-Telangiectasia
- Defect in ATM gene which codes for DNA repair enzymes - risk for lymphoma, radiation sensitivity |
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Severe pyogenic infections early in life
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Hyper IgM
- IgM is high and everything else is low - Defective CD40L --> inability to class switch |
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Pyogenic Infections, TCP purpura, Ezcema.
Low IgM, High IgE and IgA |
Wiskott-Aldrich
- X linked - WAS gene - T cells can't recognize actin cytoskeleton |
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Delayed umbilical separation, no pus formed, bacterial infections
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Leucocyte Adhesion Deficiency
- Defect in LFA-1 Integrin CD18 on phagocytes - Phagocytes can't extravasate |
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Pyogenic infections with Step and Staph, Albinism, Peripheral neuropathy, Giant granules in neutrophils
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Chediak Higashi:
- Defect in LYST lysosonmal trafficking regulator gene - Microtubule dysfunction in phagosome lysosome fusion - Phagocytose things but then can't break them down |
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Susceptibility to S Aureus, E Coli, Aspergillus, Klebsiella, Candida, Pseudomonas
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Chronic Granulomatous Disease
- Lack of NADPH Oxidase - decrease respiratory burst in neutrophils - Susceptible to catalase positive - Nitroblue tetrazolium dye abnormal test - Dihydrorhodamine DHR flow cytometry test |
