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ACAAI Review for the Allergy & Immunology Boards Flash Cards ALL CHAPTERS 409 Questions with Correct Answers Small-molecule antigen requires covalent linkage to a larger carrier to stimulate adapt... ive immune response. The process is achieved in collaboration between haptenspecific B cells and carrier-specific T cells. This is the basis of developing conjugated vaccines. >>>What is the hapten-carrier effect? SEB and SEC from Staphylococcus aureus cause food poisoning. TSST from Staphylococcus aureus and SPE-C from Streptococus pyogenes cause toxic shock syndrome. >>>What are the common superantigens and related diseases? Natural killer T (NKT) cells recognize lipid antigens, and the CD1 molecule is involved. >>>Which type of T cell recognizes lipid antigens and what molecule is involved? Protein antigens are T-cell dependent. Polysaccharide, nucleic acid, and lipids are Tcell independent. >>>Which antigens are T-cell dependent and which are T-cell independent? On the MHC class I molecule, α3 is the binding site for CD8. On the MHC class II molecule, β2 is the binding site for CD4 >>>What is the binding site for CD4 on the MHC class I molecule? What is the binding site for CD8 on the MHC class II molecule? On the MHC Class I molecule, α1 and α2 make up the peptide binding cleft. On the MHC class II molecule, α1 and β1 make up the peptide binding cleft. >>>Which chain makes up the peptide binding cleft in MHC class I molecules? Which chain makes up the peptide binding cleft in MHC class II molecules? An MHC class I molecule presents both intracellular antigens (e.g., viral antigen in cytoplasm) and extracellular antigens (via cross-presentation). The loading site is endoplasmic reticulum (ER). >>>MHC class I molecule presents which type of antigens and where does the antigen-MHC class I loading happen? The MHC class II locus. They are involved in peptide processing for MHC class I however. >>>In which chromosomal region are the TAP proteins located? The MHC class II molecule presents extracellular antigens (e.g., antigens from phagocytosed bacteria). The loading site is phagolysosome. >>>MHC class II molecule presents which type of antigen? Where does the antigen-MHC class II loading happen? IL-10 and TGFβ >>>Which cytokines are important for Tregs?TCR, MHC molecules, CD4, CD8, CD19, B7-1, B7-2, Fc receptors, KIR, and VCAM-1 >>>What molecules belong to the Ig superfamily? Junctional diversity; TdT enzyme is important in this process. >>>Which somatic recombination process introduces the greatest diversity in immune receptors and which enzyme is important in this process? The FcRn receptor binds to IgG and allows it to be endocytosed. It protects it from the lysosome and instead recycles it to the cell surface. This accounts for the long half-life of IgG in humans. This is also the mechanism by which IgG is transported across the placenta and fetal intestine. IgG3 has the shortest half-life because it poorly binds to FcRn. >>>What is the role of the FcRn receptor? X-linked lymphoproliferative syndrome (XLP) >>>Mutation in SAP causes which disease? Cyclosporine binds to cyclophilin, which are proteins also called immunophilins. The drug-protein complex inhibits calcineurin and therefore NFAT translocation to the nucleus. >>>Immunosuppressant cyclosporine binds to which molecule in T-cell signaling pathway? CD21, CD19, and CD81 >>>What CD molecules are in the BCR coreceptor? CXCR4 >>>Which chemokine receptor is associated with WHIM (warts, hypogammaglobulinemia, infections, amd myelokathexis) syndrome? α4β7 >>>Which integrin molecule is important for gut homing by binding to MAdCAM? 4-10 days >>>Upon first exposure to a medication, when might a patient develop symptoms of serum sickness? IL-4 Rα, which is targeted by dupilumab >>>Which component is shared by IL-4 and IL- 13 receptors? IFNγ receptor deficiency results in susceptibility to Mycobacterium tuberculosis and other intracellular bacteria. >>>What is the result of IFNγ receptor deficiency? IL-1β >>>What interleukin is excessively produced in cryopyrinopathies? Lipopolysaccharide responsive beige-like anchor protein (LRBA) deficiency >>>What disease presents with early onset hypogammaglobulinemia with autoimmunity and immune dysregulation? It maintains intracellular stores of CTLA4, which can stop T-cell activation. >>>What is the function of LRBA?Properidin >>>Which complement deficiency is inherited as X-linked? CD 59 and S protein >>>What two molecules inhibit MAC formation? CD 55 and CD 59 >>>Which complement receptor is implicated in PNH (paroxysmal nocturnal hemoglobinuria)? Adipose tissue >>>What is the main source of Factor D? HUVS (hypocomplementemic urticarial vasculitis) >>>Anti-C1q antibody is found in which disease process? Factor XII >>>Contact activation pathway is initiated by which factor? B-2 receptor >>>Bradykinin acts on what receptor on endothelial cells? IgG and IgM >>>What are the primary immunoglobulins involved with type II hypersensitivity? Classical pathway >>>IgG and IgM activate which complement pathway? Both are type III hypersensitivity reactions; however, serum sickness is systemic and arthus reaction is local >>>How are serum sickness and arthus reaction similar and different? Innate immunity and adaptive immunity >>>Complement activation is involved with what types of immunity? PAMPS >>>How do receptors of the innate immune system recognize microorganisms? NODs >>>What receptor is involved with the inflammasome? TLR 3 >>>Which TLR does not signal through MyD88? TLR 4 >>>Which TLR can signal through both MyD88 dependent and independent pathways? TLR 3, 7, 8, and 9 >>>Which TLRs are present in the intracellular compartment and implicated in HSV1 encephalitis? PAMPs are conserved microbial sequences from microorganisms whereas DAMPs are endogenous molecules derived from damaged or dying cells. >>>How do PAMPS differ from DAMPs? TLR 4 >>>Which TLR binds lipopolysaccharide (LPS) on gram-negative bacteria?IL-6, TGFβ, IL-1, IL-21, and IL-23 >>>Which cytokines induce development of Th17 cells? TdT >>>Which enzyme is responsible for adding nucleotides during junctional diversity? Pre B cell >>>At which stage does signaling through the pre-B-cell receptor occur? The medulla, which is also where the AIRE protein functions >>>Where does negative selection occur for T cells? Fas (CD95), FasL, caspase 10 >>>What known mutations can lead to ALPS? CD14; macrophages also express CD33 >>>What CD molecule can be used to distinguish monocytes and macrophages from other cells? Plasmacytoid (pDCs) >>>Which subset of dendritic cells produces type I IFN during viral infections? Chemokine CXCL8 (IL-8) >>>What is the most important chemoattractant that guides neutrophil to inflammation? ITP >>>In which platelet disorder are platelets abnormally large? Hint: Antibodies to platelet's GP2b3a are also seen. Keratinocytes produce CCL17 (TARC) and CCL22 (MDC); skin-homing T cells express cutaneous lymphocyte antigen (CLA) and CCR4 and CCR10 >>>Which chemokines produced by keratinocytes are responsible for homing T cells to the skin? What are the corresponding receptors on the T cells? L-selectin is found on leukocytes (E-selectin and P-selectin are found on endothelium; P-selectin is also found on platelets) >>>Which selectin is not found on endothelium? CCL19 and CCL21 on high endothelial venules, and CCR7 on the naive T cell. >>>What surface markers on high endothelial venules and naive T cells are responsible for homing to the lymph nodes? ICAM-1, ICAM-2, and VCAM-1 >>>What integrin ligands are found on the surface of activated endothelium? LAD I: CD18; LAD II: CD15s >>>Where is the molecular defect in LAD I versus LAD II? TNFα >>>Which mast cell mediator is both preformed and produced in the late phase? Charcot-Leyden crystals >>>What is contained in eosinophil primary granules?Hyper-IgE syndrome, Wiskott-Aldrich syndrome, Omenn syndrome, IPEX, atypical complete DiGeorge syndrome >>>What primary immunodeficiencies are associated with high IgE levels Urine N-methylhistamine or prostaglandin D2 (11-β-prostaglandin F2 is a metabolite of prostaglandin D2) >>>What mediators can be elevated in a 24-hour urine collection after mast cell activation? IL-5, CCL11, TSLP, IL-25, IL-33 >>>What cytokines promote eosinophilia? IL-3 >>>What is the major cytokine that drives basophil development? SIGLEC-8 >>>What immunoglobulin-like lectin is found on human eosinophils? Eotaxin-3 >>>Dysregulation of which eotaxin has been associated with eosinophilic esophagitis? Interleukin 7 (IL-7) >>>Which cytokine produced by bone marrow and thymic stromal cells is a growth factor required for development of B cells and T cells? Negative selection >>>What process results in the apoptosis of autoreactive or nonfunctional lymphocytes? DiGeorge syndrome, SCID, CHARGE, infant of a diabetic mother >>>What diseases are associated with congenital thymic aplasia or hypoplasia in humans? Good's syndrome >>>Hypogammaglobulinemia and thymoma are associated with what condition in adults? Chronic Helicobacter pylori infection is associated in 72-98% of cases >>>What pathogen is frequently associated with gastric MALT lymphoma? Concha bullosa >>>What anatomic variant is pictured in Figure 3-1 (denoted by the asterisk)? (Click on Show Figures for Chapter 3 below this window.) Nasal valve; it is the narrowest point of the whole airway >>>What is the name of the junction between the nasal vestibule and main nasal cavity just anterior to the tip of the inferior turbinate and why is it important? Encapsulated bacteria, including Neisseria meningitidis, Haemophilus influenzae, Streptococcus pneumoniae >>>Physical or functional absence of a spleen is associated with increased susceptibility infections with what pathogens? Swelling of the inferior turbinate >>>How is nasal airway resistance regulated?Anticholinergic medications (e.g., ipratropium bromide nasal spray) >>>What family of medications would be best suited to treating clear watery nasal secretions? Saccharin test. This is a timed test where a small amount of saccharin is placed on the anterior end of the inferior turbinate. It is complete when the patient tastes the sweet flavor in the mouth. Normal result is 7-11 minutes. >>>How can mucociliary clearance be clinically evaluated? Kartagener's syndrome; 50% of individuals with PCD have Kartagener's syndrome >>>What syndrome is characterized by ciliary dyskinesia, situs inversus, bronchiectasis, and chronic sinusitis? (A) maxillary sinus; (B) ethmoid sinus; (C) concha bullosa; (D) inferior turbinate >>>Identify structures A, B, C, and D in Figure 3-2. (Click on Show Figures for Chapter 3 below this window.) (E) sphenoid sinus; (F) posterior ethmoid sinus >>>Identify structures E and F in Figure 3-3. (Click on Show Figures for Chapter 3 below this window.) Maxillary and ethmoid sinuses are present at birth. The frontal sinus is last to develop, when a child is around 6 years old. >>>Which sinuses are present at birth? Which is the last to develop? Haller cells >>>What is the name of the ethmoid air cells found in the infraorbital area? Onodi cells >>>What are displaced sphenoethmoid air cells called? Agger nasi cells >>>What is the name of the anterior ethmoid air cells found above the middle turbinate? These are Curschmann's spirals, which are found in asthma. They are twists of condensed mucus. >>>What constitutes the structures in Fig 3-4 and name the condition they are associated with. (Click on Show Figures for Chapter 3 below this window.) These are the creola bodies, which are found in asthma. They are clusters of surface epithelial cells. >>>What constitutes the structures in Fig 3-5 and name the condition they are associated with. (Click on Show Figures for Chapter 3 below this window.) These are the Charcot-Leyden crystals, which are found in asthma. They are eosinophil cell and granule membrane lysophospholipase >>>What constitutes the structures in Fig 3-6 and name the condition they are associated with. (Click on Show Figures for Chapter 3 below this window.)Airway wall thickening with increased collagen deposition and airway smooth muscle, goblet cell hyperplasia, subepithelial fibrosis, and angiogenesis >>>What are the pathologic features of airway remodeling? Connective tissue mast cells (MCTC). Bonus: C5a anaphylatoxin >>>What chymaseand tryptase-containing cells are typically found in the skin, conjunctiva, heart, and intestinal submucosa and have the CD88 receptor? Bonus: What does the CD88 receptor bind to? Cutaneous lymphocyte antigen (CLA)-1 >>>What carbohydrate epitope helps dermal T lymphocytes home to the skin? Filaggrin (FLG) >>>Mutations in which gene are associated with ichthyosis vulgaris and atopic eczema? Pemphigus vulgaris. Bonus: Epidermal IgG and C3 cell surface staining of the suprabasal layers >>>What condition is associated with flaccid bullae affecting the scalp, chest, intertriginous areas, and oral mucosa. Bonus: What will tissue immunofluorescence demonstrate? Bullous pemphigoid >>>A 75-year-old woman complains of a very pruritic rash characterized by tense blisters. Tissue immunofluorescence demonstrates linear basement membrane zone IgG and C3. What condition does she have? Dermatitis herpetiformis and celiac disease >>>A 4-year-old boy presents with an itchy rash characterized by small blisters on his elbows and knees. His mother mentions that he also has chronic diarrhea. What conditions does he likely have? Greater than 15 eosinophils per high powered field while on a proton pump inhibitor (PPI) for 8 weeks. >>>What is a diagnostic biopsy characteristic for eosinophilic esophagitis (EoE)? Cross-sectional and case control >>>Which types of studies are subject to recall bias? Cross-sectional >>>What type of study samples subjects at one point in time for presence or absence of exposure and disease? Student t test >>>What parametric test compares means between two groups? Alpha error >>>What type of error occurs when the null hypothesis is falsely rejected? (Risk of disease or death among people who are exposed)/(Risk of disease or death among people who are not exposed) >>>Define relative risk (risk ratio) A measure of disease present as one point in time >>>Define prevalence.True positive / (True positive + False negative) >>>Define sensitivity. Confounding variable >>>X is assumed to cause Y, when in truth Z is common also and causes X and Y. What is Z? Ib >>>What category of evidence uses evidence from at least one randomized controlled trial? Maximizing the benefits for the research project while minimizing the risks to the research subjects >>>Define beneficence. Early-phase mechanisms are allergen-induced mast cell secretion of histamine, prostaglandins, and leukotrienes; late-phase mechanisms are eosinophil chemotaxis, caused by chemical mediators produced in the early reaction >>>What are the primary mechanisms of early- and late-phase allergic rhinitis? They block both the early-phase (rhinorrhea, sneezing) and late-phase (congestion) responses >>>Intranasal corticosteroids block which phase(s) of the allergic response? Preexisting allergic rhinitis; about one-third of women with AR experience symptom exacerbation during pregnancy >>>What is the most common cause of rhinitis during pregnancy? Turbinate edema resulting from the release of thyroid-stimulating hormone >>>Why can nasal congestion or rhinorrhea develop in hypothyroidism? Check β2-transferrin in nasal secretions for suspected CSF rhinorrhea >>>Acute onset of unilateral, clear rhinorrhea that is worse when the patient leans forward should prompt evaluation with what test? Fluid extravasation leads to mucosal edema and venous constriction >>>What is the pathophysiologic mechanism for pale, boggy nasal mucosa in patients with AR? Expansile growth of fungal hyphae and generation of allergic mucin >>>How does AFRS lead to bone destruction and extension outside the sinuses? Increases of IgE >10% in a patient's postsurgical IgE correlate with increased risk of recurrence >>>What lab values can be monitored to assess interval progression of AFRS s/p FESS? No, studies have found poor correlation >>>Does serum-specific IgE to fungal element correspond to species of fungus found in allergic mucin of patients with AFRS? Total serum IgE level is not one of the Bent and Kuhn diagnostic criteria for AFRS >>>What value of total serum IgE is necessary to diagnose AFRS?Identification of fungal sensitization and preparation for post-FESS allergen immunotherapy to fungal and nonfungal allergens >>>In a patient with recurrent sinusitis and CT findings of pansinusitis and inspissated mucin, what role(s) does skinprick testing to aeroallergens play? Ciliary flushing is an injection of the deep episcleral vessels, causing redness around the cornea. It is seen in corneal inflammation, iridocyclitis, and acute glaucoma. >>>What is ciliary flushing, and in which conditions is it found? Histamine H1 receptor antagonist + mast cell stabilizer >>>What is the treatment of choice for mild to moderate allergic conjunctivitis? Vernal keratoconjunctivitis >>>A 10-year-old boy is brought into your clinic because of redness in his eyes. His medical history is notable for seasonal allergic rhinitis and asthma. His main complaints include significant ocular pruritus, sensitivity to light, and feeling like something is stuck in his eye. On exam his sclera are injected and his R eyelid is lower than his Left. What is the diagnosis? Atopic keratoconjunctivitis >>>A 45-year-old woman presents with ocular pruritis. Other symptoms include redness in her eyes, difficulty with light, and sometimes pain. When going through her history she reports that she always feels dry and has problems with dry skin. Her eyes feel like leather, and you notice that she has lost her eyelashes. She denies any vision problems requiring glasses or contacts. What is the diagnosis? (a) Anterior vestibulum of nose >>>In which of the following is filaggrin expressed (a) anterior vestibulum of nose; (b) transitional nasal epithelium; (c) respiratory nasal epithelium; (d) human bronchial epithelium? IL-31 >>>Which cytokine is primarily associated with pruritus in atopic dermatitis? Eczema vaccinatum >>>What can a person with eczema develop after smallpox vaccination? Dust mite >>>Evidence supports a role of which aeroallergen in AD? Smallpox >>>Which vaccine is contraindicated in severe AD? Inhibit cathelicidin expression >>>What do IL-4, IL-13 do to cathelicidin in the skin? Rhinovirus >>>What is the most frequent infectious cause of asthma exacerbations? 50% >>>What percentage of children will have episodes of wheezing in the first 3 years of life due to viral respiratory tract infections? Low socioeconomic status and African American race >>>What two factors increase risk of asthma mortality?Alternaria >>>Sensitization to what aeroallergen in children under 6 years old is associated with development of persistent asthma by age 11 years? >12% >>>What percent change in FEV1 on spirometry after bronchodilator administration is diagnostic for asthma? Exhaled nitric oxide (eNO) >>>What biomarker can be readily measured and is increased in eosinophilic asthma? (a) asthma in a parent >>>Which of the following is the biggest risk factor that a 3-yearold child will develop persistent asthma by the age of 6 years: (a) asthma in a parent; (b) asthma in a sibling; {c) wheezing apart from colds; {d) allergic rhinitis in a sibling? Budesonide >>>What is the preferred inhaled corticosteroi [Show More]

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