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37 Cards in this Set
- Front
- Back
Baby has recurrent fungal ,viral and bacterial infxns. A low IgGm and a thymic shadow. Whats he got?
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NOT DIGEORGE! Severe combined immunodeficiency. Both T and B cell immunity is fucked. Digeorge has normal humoral immunity
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What is the most common form of childhood systemic Vasculitis?
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HenochSchonlein purpura- IgA complexes
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Whats the triad for Henoch Scholein purpura?
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Skin, Joints, GI
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A kid comes in with colicy abdominal pain, joint pain, and palpable purpura. What renal problem is this disease associated with?
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IgA nephropathy- This disease is Henoch-Schonlein purpura ( due to IgA complexes)
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What disease affects the skin, joints, and Gi and has Iga complexes?
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HenochSchonlein purpura- Type III hypersensitivity
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True/False. Henoch schonlein Purpura have multiple lesions of different ages.
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False, they are all of the same age
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What is the first cell to show up during a Type IV hypersensitivity to poison Ivy?
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Apprently not macrophages, but rather T LYMPHOCYTEs
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True/False. The Polio vaccine (attenuated) is intramuscular while the killed Salk vaccine is given orally.
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False, the attenuated version is give orally, and stimulates IgA synthesis so the polio virus is stopped at its site of entry- mucosa (the heat killed one is give intra-muscularly)
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A patient has diplopia and difficulty chewing that improves on edrophonium infusion. What type of hypersensitvity?
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Type II
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Someone has a renal transplant. A biopsy shows vascular fibrinoid necrosis and neutrophils. How long after transplant is this?
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Immediate- Hyperacute sensitivity (preformed antibodies fix complement)
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2 yrs after a renal transplant someone shows up with renal failure. Biopsy shows dense lymphocyte infiltration. What happened?
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They probably stopped their immunosupressants (acute rejection can occur if meds are stopped
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What bacterial infection does silicosis sometimes predispose to?
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TB by impairing Macrophage phagolysosome disruption
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A patient working in a foundry and mine, is found to have tuberculosis, and restrictive lung disease. What is the state of his hilar lymph nodes on CT?
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This is silicosis- predisposes to Tb (disrupts phagolysosomes). The hilar lymph node would have egg-shell calcification
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A 4 yr old boy has difficulty walking, and has reccurent respiratory infections. What other enzymes are messed up if he also has IgA deficiency?
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Ataxia- Telengiectase- DnA Repair enzymes - He will also have spider angiomas
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What are the three polysach vaccines given to kids that are conjugated with protein?
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Strep, H. Flu, and Neiseria meningitidis (conj to protein to improve T cell response)
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What is the role of Nef protein from HIV on immune cells?
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To reduce MHC-1 on the surface of infected cells
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True/False. Secretory IgA acts as an opsonin.
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False, it binds to pilli and other membrane proteins that mediate bacterial adherence. IgA protease lets N.meningitidis, gonnorhea, strep pneumo, h. flu
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True/False. Although N.meningtidis makes Iga protease, n. gonorrhea does not.
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False, they both make iga protease
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Which T helper subset secretes IL-13?
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Th2 - this is thought to be a mediator in asthma (it is thought that together with Il-4, you get wayyy too much B cell IGE production)
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True/false. Even though IgGs cross the placenta, RhoGam is an IgG.
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yes
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Whats the order fo the immunoglobin heavy chains on the gene?
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MD GEA
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If some has really high IgM and low all the other abs, whats the likely defect?
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Lack of Cd40 Ligand on helper T cells- inability to class switch==HYPER IGM syndrome
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A patient has recurrent pusforming infxns and lymph hyperplasia. If he has very low IgG and A, what is the likely syndrome?
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CD40 ligand is missing on T cells so B cells cant class switch= Hyper IGM
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What cytokines released during tissue injury are anti-inflammtory?
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il-10 and Tgf beta
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What two syndromes manifest as IgA deifciencies?
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Selective Ig Deficiency (isotype switching) and Ataxia-telengectesia (Dna repair enzymes)
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How many proteins does a Secretory IgA have?
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ten- 4 Ig heavy, 4 Ig light, J protein , and secretory peptide
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A very pale child has recurrent skin infxns and respiratory infxns. He has giant cytoplasmic granules in nuetrophils and macrophages. What finding on neuro exam is also likely?
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Horizontal nystagmus- Chidiak higash (failure of phagolysosomal fusion=albino, peripheral neuropathy, and recurrent infxns)
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What b and T cell immunodeficiency is X linked?
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wiXcott Aldrich syndrome= Wiskot aldrich wears a TIE= Thrombocyto, Immunodef, and Exzema = Increased Ige and A, lower IGM
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What is the lab profile like in wiXcot aldrich syndrome?
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Increased IgE and A, low IgM
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What immunodeficiency presents with Eczema?
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wiXcott Aldrich syndrome= Wiskot aldrich wears a TIE= Thrombocyto, Immunodef, and Exzema = Increased Ige and A, lower IGM
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Where on IgG does comlpement bind?
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Near the hinge region (Ch2)
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Skin infections and gingivitis in a baby. The god Damn umbillical cord just wont fall off. Whats he got?
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Leukocyte adhesion deficiency- LFA1 intregrin has a defect (CD18) on phagocytes
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A baby keeps getting skin infections, but there is no pus. Labs show neutrophilia. What is absent?
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CD18 for LFA-1 integrin marker = the problem is Leukocyte Adhesion Deficiency Type 1 - umbillical cord doesn’t fall off either
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For a woman with non-caseating granulomas in the lung, and bilateral lymphadenopathy, what is the broncoalveolar lavage likely to show?
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Increased CD4/CD8 T cells ==More cd4s
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What immunodeficiency deals with a lack of a tyrosine kinase ad has reccurent bacterial infxns after 6mos?
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Brutons X linked Agammaglobulinemia
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What immunodeficiency has normal pro-B cells , but less maturation, # of b cells, and immunoglobiun?
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Brutons X linked Agammaglobulinemia
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A boy comes in with recurrent respiratory infxns, low immunoglobulin of all kinds, and has a maternal uncle with the defect. Whats his lymph node look like?
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lack of germinal centers
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