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37 Cards in this Set

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