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Immunodeficiencies.
Day 2
| Question | Answer |
|---|---|
| x-linked, b cell deficiency (defective tyrosine kinase gene), recurrent bacterial infections after 6 mos | bruton's aggamaglobulinemia |
| 3rd and 4th pouches fail to develop--> no thymus-->no t cells; congenital defects in heart/great vessels | thymic aplasia (diGeorge) |
| recurrent viral, fungal, and protozoal infections with 22q11 deletion | thymic aplasia (diGeorge) |
| no thymic shadow on CXR (2 diseases) | thymic aplasia (diGeorge) and SCID (severe combined immunodeficiency) |
| adenosine deaminiase deficiency causing defect in early stem cell differentiation | SCID |
| no b cells or t cells so only defense is NK cells | SCID |
| triad: 1. severe recurrent infections with chronic mucocutaneous candidiasis; fatal or recurrent RSV,VZV, HSV, measles, flu, parainfluenza; PCP pneumonia 2. chronic diarrhea 3. failure to thrive | SCID |
| pneumocystis jiroveci in a young child | SCID |
| what drug is used to treat chronic mucocutaneous candidiasis? | ketoconazole |
| What is the WAITER of Wiskott-Aldrich? | WA=Wiskott Aldrich I= Immunodeficiency (decreased IgM) T= Thrombocytopenia and purpura E= Eczema R= Recurrent pyogenic infections (no IgM to fight capsular polysaccarides of bacteria, low IgM and high IgA, X-linked) |
| truncal eczema in a child | Wiskott-Aldrich |
| IgA deficiency, cerebellar ataxia, elevated AFP in a 1 year old, and poor smooth pursuit of moving target with eyes | ataxia-telangiectasia (also see telangiectasias on face, obviously) |
| ataxia-telangiectasia carries an increased risk of developing which malignancies? | lymphoma and acute leukemias (ALL, AML) |
| most common selective immunoglobulin deficiency that presents with sinus and lung infections in otherwise healthy person | IgA deficiency (may also have atopy, asthma) |
| why is it important to diagnose IgA deficiency in a person who is otherwise healthy? | danger of anaphylaxis to blood transfusions and blood products |
| IL-12 receptor deficiency predisposes to what type of infections? | mycobaterial infections |
| delayed separation of umbilical cord | leukocyte adhesion deficiency |
| abnormal integrins so phagocytes can't exit circulation | leukocyte adhesion deficiency |
| hyperimmunoglobulin E syndrome is also known as ________ syndrome | job syndrome |
| triad: eczema, recurrent cold staph aureus abscesses, hgih levels of IgE and eosinophils | job syndrome/hyperimmunoglobulin E syndrome |
| defective LYST gene | chediak-higashi disease |
| giant cytoplasmic granules in PMNs caused by defective phagocyte lysosome | Chediak-Higashi disease |
| triad: partial albinism, recurrent respiratory tract and skin infections), and neuro disorders | Chediak-Higashi disease |
| coarse facial features, doughy skin, and 2 rows of teeth (retain primary teeth) | Job syndrome |
| lack of NADPH oxidase activity | Chronic granulomatous disease |
| susceptible to catalase + organisms like staph aureus, e coli, klebsiella, aspergillus, and candida | Chronic Granulomatous Disease |
| Negative nitroblue tetrazolium test | Chronic Granulomatous Disease |
| Tx options for Chronic Granulomatous Disease | Prophylactic TMP-SMX; also INF-gamma |
| Normal number of B cells, but decreased plasma cells and immunoglobulin b/c there is a defect in B cell maturation | Common variable immunodeficiency (CVID) |
| Increased risk of autoimmune disease, lymphoma, and sinopulmonary infections that are typically acquired in the 20s and 30s | Common variable immunodeficiency (CVID) |
Created by:
sarah3148
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