Chekarian Final Review Flashcards by Maddie Palmer

A 6 year old child has a history of recurrant infections with pyogenic bacteria, uncluding staph aureus and strep pneumoniae. the infections are accompanied by a neutrphilic leukocytosis. Microscopic examination of a biopsy specimen obtained from an area of soft tissue necrosis shows a DEFECT IN ROLLING. This childs increased succeptability to infections is most likely caused by a defect in which one of the followign molecules

selectins

integrins

leukotriene B4

complement C3b

NADPH oxidase

SELECTINS- this is c/w LAD 2

integrins would be LAD1

Leukotriene B4 is associated with chemotaxis of neutrophils

complement C3b is opsonization

NADPH oxidase is CGD

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high IgA, normal IgG, low IgM, high IgE

wiskott aldrich

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Chediak-Higashi syndrome

defective lysosomal function in neutrophils, macrophages, and DCs, and defective granule fn in NK cells

mutations in a gene encoding a lysosomal trafficing regulatory protein

DEFECT IN LYSOSOMAL STORAGE PROTEIN:
DYSFUNCTIONAL MICROTUBULES
DYSFUNCTIONAL PHAGOCYTOSIS

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leukocyte adhesion deficiency 2

primary immunodeficiency- inante

absent or deficient expression of leukocyte ligands for endothelial E and P selectins causing failure of leukocyte migration into tissues

mutations in genes encoding a protein required for synthesis of sailyl-Lewis X component of E and P selectin ligands

SELECTIN/ROLLING

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Both the inactivated polio vaccine (IPV) and the attenuated oral polio vaccine (OPV) are effective in preventing poliovirus infefction. WHy is the IPV vaccine recommended for use in children in north america

IPV is effective after a single dose of vaccine

immunity induced with IPV is longer lasting than immunity induced with OPV

IPV induves stronger cytotoxic T lymphocyte responses

worl wide polio eradication is more attainable with universal IPV immunozation

there is no risk of vaccine associated paralytic polio when using IPV

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a 3 year old boy comes to the pediatrician with the 4th infection this year. Past causes include ppneumocustitis and CMV, which you typically wouldnt seein a healthy child. You presict that later in life he may develop liver disease. Testing reveals that he has a mutation in CD40L on T cells. Which of the following would represent his serum levels of immunoglobuliuns?

low IgA, low IgG, low IgM, low IgE

high IgA, normal IgG, low IgM, high IgE

normal IgA IgG IgM, high IgE

low IgA low IgG high IgM low IgE

low IgA normal IgG normal IgM normal IgE

low IgA low IgG high IgM low IgE

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Leukocyte adhesion deficiency 1

primary immunodeficiency- innate immunity

absent or deficient expression of beta2 integrins causing defective leukocyte aadhesion- dependent functions

mutations in gene encoding the beta chain (CD18) of beta 2 integrins

INTEGRIN/ADHESION

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a 7 year old boy presents to famuly medicine clinic with pharyngitis. He has a positive strep test and is treated with amoxicillin. Three weeks later, the patient presents to the ER with a fever, malaise, nausea, and cocacola colored urine. You suspect that he is experiencing a hypersensativity reaction that can cause systemic effects and be associated with vasculitis. What diseqase occurs by a similar mechanism?

chronic granulomatous disease

asthma

graves dz

SLE

multiple sclerosis

SLE

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X linked hyper IgM syndrome

defefcts in adaptive response

Defect in helper T cell development
B cell and macrophge activation

Mutaitons in CD40 ligand

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Complement C2 C4 deficiency

primary ID- innate

deficienct activation of classical pathway of complement leading to failure to clear immine complexes and develip of lupus like disease

mutations in C2 or C4 genes

ALSO TERMINAL COMPLEMENT PRODUCT DEFICIENCIES C1 INH

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What genetic deficiencies in complement is this disease associated with

infection, nisseria meningitidis

Alternative pathway

properin

factor D

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a 4 year old boy with hx otitis media and PNA brought in to the ED because of an acute sinus infection. His IgG and IgA levels are very low but his IgM level is very high. These findings are most commonnly assocaited with a deficiency in which of the following?

CD3

CD4

CD8

CCD40L

P selectin

CD40 L

this is X linked hyper IgM syndrome

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common variable immunodeficiencies

defects in adaptive response

reduced or so production of selective isotypes or subtypes of immunoglobulins; succeptibility to bacterial infections or no clinical problems

mutations in receptor for B cell fowth factors, costimulators

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normal IgA IgG IgM, high IgE

Hyper IgE

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low IgA normal IgG normal IgM normal IgE

selective IgA deficiency

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X linked agammaglobulinemia

ID that affects B and T cell development

decrease in all serum Ig isotypes; reduced number of B cells

block in maturation beyond pre-b cells, because of mutation in B cell tyrosine kinase

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Which of the following vaccines would most likely be effective after a single dose?

inactivated virus

live, attenuated virus

toxoid

conjugate vaccine

virus like particle

Live, attenuated virus

What genetic deficiencies in complement is this disease associated with

recurrant diminished nisseria infection

shared

C5b, C6, C7, C8, or C9

A 4 year old girl presents with a severe staph aureus (bacterial) abcess. Her history is significant for a previous infection with Serratia marcescens. If she has an enzyme deficiency, which of the following is most likely?

adenosine deaminase

C1 inhibitor

myeloperoxidase

NADPH oxidase

NADPH oxidase!

c/w chonic granulomatous disease

adenosine deaminase would be associated with SCID (which would also be more succeptable to viral and fungal pathogens)

What genetic deficiencies in complement is this disease associated with

SLE

classical pathway

C1, C4, C2

Chronic granulomatous disease

primary immunodeficiency- innate immunity

pefective production of ROS by phagocytes

mutations in genes encoding components of the phagocyte oxidase enzyme, most often cytochrome b558

ROS/PHAGOCYTOSIS

which of the following antigens would NOT be expected to elicit memory B cells

a conjugate vaccine (bacterial polysacchardise linked to a protein sych as tetanus toxoid)

a live virus vaccine

an inactivated virus vaccine

purified bacterial polysaccharide

purified tetanus toxoid

What genetic deficiencies in complement is this disease associated with

recurrant childhood infection, nisseria meninditidis , other encapsulated bacteria, autoimmune disease

shared

Digeorge syndrome

decreased T cells; normal B cells; normal or decreased serum Ig

HYPOCALCEMIA

anomaluos development of 3rd and 4th brachial pouches, leading to thymic hypolpasia

What genetic deficiencies in complement is this disease associated with paroxysmal nocturnal hemoglobinuria

membrane regulations CD55(DAF), CD59

Complemenet C 3 deficiency

primary ID- innate defect in complement cascade activation mutations in C3 gene ALSO TERMINAL COMPLEMENT PRODUCT DEFICIENCIES C1 INH

low IgA, low IgG, low IgM, low IgE

Btk, X linked brutons kinase deficiency

Which type of vaccine is the best

VLPs

Wiskott-Aldrich syndrome

SCID cytoskeletal deficiency leading to poor uptake by DCs and other APCs

When the exotoxine polypeptides of bacteria are treated with formaldehyde, acid, or heat, toxoids are produces. These toxins are then used to vaccinate humands against diease since they are antigenic and no longer toxic. Toxoids make effective vaccines because they induce which of the following immune responses toxin neutralizing antibodies macrophage activation activation of complement toxin-specific cytotoxic T lymphocytes

toxin neutralizing antibodies

What genetic deficiencies in complement is this disease associated with atypical hemolytic uremic syndrome, age related macular degeneration

membrane regulatoin CD46 (MCP), factor H

in 1998 a new childhoo vaccine was developed to protect against epidemic meningitis by mixing haemophilus influenzae type B polysaccharide with whole killed bordetella pertussis bacteria. The function of the whole, killed bacteria in this vaccine is as a(n)? carrier hapten mitogen adjuvant immunogen

ADJUVANT here this is using the vacteria to elicit a stronger immune resonse carrier and hapten refer to a conjugate vaccine which is a polysarccharide attached to protein rather than mixed with a whole bacteria

What genetic deficiencies in complement is this disease associated with recurrant childhood infection, phagocytic bacteria

Lectin pathway MBL, MASP

What genetic deficiencies in complement is this disease associated with recurrant angioedema

CP regulation C1-INH

Autosimal recessive SCID due to ADA, PNP deficiency

ID that affects B and T cell development progressive decrease in T and B cells (mostly T) reduced serum Ig in ADA deficiency, normal B cells, amd serum Ig in PNP deficiency ADA or PNP deficiency leads to accumulation of of toxic metabolites in lymphocytes

A 5 year old boy has had three episodes of invasive disease due to nisseria meningitidis, a bacteria that is normally cleared by opsonization and phagocytosis. Histotal leukocyte count and serum IgG, IgM, and IgA concentration are within the reference ranges. Which of the following immune deficiency diseases is most likely responsible for his increased succeptibility to infection? AIDS common variable immunodeficiency deficiency of terminal complement components myeloperoxidase deficienfcy Wiskitt-Aldrich syndrome

deficiency of terminal complement components cannot super rule out AIDS bu there should be a decrease in CD4 levels for wiskott-aldruch there would be low T cell count for variable immunodeficiency there would be low Ig levels

What genetic deficiencies in complement is this disease associated with infection, membranoproliferative glomerulonephritis

AP regulation Factor H, Factor I acquired C3 deficiency

X linked SCID

ID that affects B and T cell development markedly decreased T cells; normal or increased B cells; reduced serum Ig cytokine receptor defect, common gamam chain gene mutations, defective T cell maturation due to lack of IL7 signals

A 30 year old woman gives birth at term to a normal appearing girl. One hour after birth, the neonate exhibits tetany (involuntary muscle contraction). On PE, she is at the 55th percentile for height and weight. Lab studies show calcium of 6.3 mg/dL (normaal 8.5-10.2) and phosphorous of 3.5 mg/dL (normal 2.4-4.1). Over the next year, the infant has bouts of PNA caused by pneumocystis carinii and aspergillus fumigatus and URIs with parainfluenza virus and herpes simplex virus. Which of the following mechanisms is most likely to be responsible for the development of the clinical features seen in the infant? malformation of the 3rd and 4th pharyngeal pouches failure of maturation of B cells into plasma cells genetically based failure to produce complement C3 acquisitoin of maternal HIV infection at delivery faulure of differentiation of preB cells into B cells

malformation of the third and 4th paryngeal pouches

RAG-1 gene deficiency is a cause of which of the following immunodefeiciency diseases? Chronic granulomatous disease leukocyte adhesion deficiency severe combined immunodeficiency X linked agammaglobinemia X linked hyper IgM syndrome

Severe combined immunodefeciency (SCID) No RAG- no VDJ recombination No B cells No T cells