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AI boards > Nuggets > Flashcards

Nuggets Flashcards

1
Q

what is CTLA-4/what does it do?

A

-receptor on activated T cells and regulatory T cells
-prevents further activation by competing with activating receptor CD28 (for ligands B7-1/CD80 and B7-2/CD86)

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2
Q

which adhesion molecule mediates FIRM adhesion of leukocytes in blood vessels?

A

integrins
- leukocyte function-association antigen (LFA-1) - macrophage antigen-1 (MAC-1)
these bind to ICAM-1 and 2 on endothelium

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3
Q

in the MHC 1 pathway, what protein mediates transport of peptides from cytosol to the ER?

A

TAP protein

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4
Q

Which T cell ligand-receptor interaction:
1) activates naive T cells
2) activates and maintains effector and memory T cells?

A

1) naive: CD28 + ligands CD80/B7-1, CD86/B7-2
2) effector/mem: ICOS receptor + ICOS ligand

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5
Q

which types of HAE have normal C1 inhibitor?

A

Factor XII mutation
ANGPT1 mutation (angiopoietin-1) not related to bradykinin pathway
plasminogen mutation

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6
Q

what is the p-i concept?

A

-pharmacologic interaction of drugs with immune receptors concept
-drug hypersensitivity mechanism - medications directly interacts with the MHC or TCR molecules to stimulate an immune response
-e.g. abacavir with HLA-B*5701

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7
Q

Which Fc Receptor expressed on the natural killer (NK) cell is responsible for antibody-dependent cell-mediated cytotoxicity (ADCC)?

A

FcyRIIIA

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8
Q

contraindications to bronchial thermoplasty

A

implantable electronic devices
sensitization to meds needed for procedure
previous treatment in same area
active resp infection
active asthma exacerbation or OCS dose change in let 14 days
known coagulopathy

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9
Q

During an immune response, which chemokine promotes eosinophil recruitment and infiltration into tissues?

A

CCL11

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10
Q

what lipid mediators are newly synthesized in mast cells when activated?

A

prostaglandin D2
LTB4, LTC4, LTD4, LTE4

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11
Q

Which mediator is a neutrophil chemokine that promotes MIGRATION?

A

CXCL8 aka IL8

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12
Q

transcription factor associated with Th2 cells?

A

STAT6
GATA3

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13
Q

Which chemokine receptor is associated with eosinophil, basophil and mast recruitment?

A

CCR3

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14
Q

main cause of symptoms of nasal congestion

A

interaction between airflow and airflow RECEPTORS in nasal mucosa

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15
Q

Which cytokine is most responsible for pruritus in atopic dermatitis?

A

IL-31

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16
Q

what do you check nasal discharge for to check if it’s CSF fluid?

A

beta-2 transferrin

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17
Q

vernal keratoconjunctivitis highlights

A

warm, dry climates, warm seasons
young atopic males
thick discharge/pain/photophobia
giant papillae >1mm, honer truants dots (per limbic white dots) - only conjunctivae and cornea affected

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18
Q

which heterozygous C’ deficiency causes mildly low CH50?

A

hetero C2 deficiency

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19
Q

nonbronchodilator effects of beta agonist (7)

A
  1. Decreased microvascular permeability
  2. Inhibit cholinergic neurotransmission
  3. Inhibit mediator release from basophils, mast cells, and lung tissue
  4. Priming glucocorticoid receptor activity
  5. Increase mucociliary clearance
  6. Protect epithelium from bacteria
  7. Inhibit function of bronchial epithelial cells, eosinophils, macrophages and T cells
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20
Q

pretreatment prior to ASA desens in AERD?

A

leukotriene modifying drug

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21
Q

Which of the following measures of lung function is most useful at monitoring adherence and predicting exacerbations?

A

FeNO

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22
Q

most common genetic cause of primary ciliary dyskinesia?

A

DNAH5 mutation

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23
Q

pork cat syndrome

A

pts sensitized to FelD2 react to ingested pork within 30-45 min
GI sxs common, but can be anything up to anaphylaxis

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24
Q

mutation in type 2 HAE?

A

missense SERPING1 gene

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25
Q

what class of drugs increases risk of ACEi induced angioedema?

A

DPPV-4 inhibitors (gliptins/liptins)
immunosuppressive drugs

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26
Q

first line HAE ppx in pregnancy?

A

pdC1-INH (subQ)

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27
Q

first line HAE ppx in peds?

A

pdC1-INH (subQ every 3-4days)
Alternative in 12+yo: lanadelumab (subQ every 2-4 wks)

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28
Q

which HAE abortive therapy can be self-administered at home?

A

icatibant

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29
Q

what HAE medication has some rabbit components?

A

recombinant C1-INH

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30
Q

how long to delay new treatment after ACEi induced angioedema?

A

6 weeks

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31
Q

which erythrocyte bound receptor results in decreased clearance of immune complexes from circulation?

A

CR1/CD35 (complement receptor 1)

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32
Q

which IgG doesn’t participate in complement activation?

A

IgG4

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33
Q

region along the precipitin curve, which results in the most pathogenic Ab/Ag immune complexes

A

zone of moderate antigen excess

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34
Q

what product/molecule is found in conjunctivae and corneal pathology of vernal keratoconjunctivitis?

A

conjunctivae: eosinophil major basic protein
cornea: eosinophil peroxidase, eosinophil cationic protein

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35
Q

MOST common corneal pathology seen in atopic keatoconjunctivitis?

A

punctate epithelial keratopathy

less common: corneal scarring, keratoconus, shield ulcers

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36
Q

which med for ankylosing spondylitis also has specific indication for management of uveitis?

A

infliximab

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37
Q

peripheral ulcerative keratitis sxs/s, dz assn, tx

A

s/s: few days of unilateral progressively worsening ocular pain, light sensitivity, redness, tearing, yellow-white peripheral corneal infiltrates
dz assn: s aureus blepharitis
tx: abx + topical steroids

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38
Q

cytokines involved in pathogenesis of irritant contact dermatitis?

A

**IL1alpha **
IL1beta, TNFalpha

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39
Q

what molecule is increased in tears of pt with giant papillary conjunctivitis?

A

tryptase

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40
Q

how do maternal smoking and gender influence AR risk

A

smoking: increase
male: increase

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41
Q

anhydrides are LOW or HIGH molecular weight and found in __?

A

LOW molecular weight
found in paints, varnishes, plastics

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42
Q

what receptor is activated in irritant rhinitis?

A

TRPA1 (transient receptor potential ankyrin type 1)

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43
Q

where are the highest eos counts in the GI tract (normally)?

A

colon

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44
Q

which first line EOE tx has 75% success rate after 8 weeks?

A

6 food elimination

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45
Q

which cytokine is most important for terminal differentiation of eos?

A

IL5

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46
Q

SPT vs sIgE sens and spec

A

SPT more sensitive
sIgE more specific

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47
Q

when do you SPT in occupational asthma

A

for high molecular weight sensitizers
and certain low molecular weight sensitizers (acid anhydride, sulfonechloramide, vinyl sulfone reactive dyes, per sulfate salts, platinum salts)

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48
Q

lab findings in wiskott Aldrich syndrome

A

low and small plt (normal megakaryocytes)

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49
Q

which C’ deficiency is ass’d with SLE?

A

C2 deficiency

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50
Q

IRAK4 and MyD88 deficiency main characteristics

A
  • Defect in TLR signaling
  • Pyogenic bacterial infections pneumococcal, pseudomonas without fever
  • Improve with age
  • AR inheritance
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51
Q

Types of T-B+NK- SCID

A
  • JACK3 deficiency
  • Xlinked SCID
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52
Q

what markers can be used to determine basophil activation?

A

CD63 and CD203c

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53
Q

what innate immunity associated receptors do basophils express?

A

TLR
leukocyte Ig-like receptors (LIR)

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54
Q

mediators that mast cells produce but basophils do not (or very little) (6)

A

tryptase
chymase
carboxypeptidase
heparin
PGD2
LTB4

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55
Q

transcription factors for eos

A

GATA-1
PU.1
C/EBP members

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56
Q

which cytokines promote maturation of eos from myeloid precursors? (3)

A

GMCSF
IL3
IL5

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57
Q

which cell is the body’s major producer of type I IFNs

A

plasmacytoid dendritic cells
important for innate immune response to viruses

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58
Q

what cytokine targets immature lymphoid progenitors to induce T lymphs?

A

IL7

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59
Q

which chemokine receptor is expressed by activated DCs?

A

CCR7 (also expressed on naive T cells)

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60
Q

impact of puberty, menses, pregnancy on nasal airflow?

A

vasodilation + glandular hypersecretion -> decreased flow

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61
Q

molecules expressed on T cells infiltrating skin (e.g. atopic derm) (4)

A

CLA
CCR4
CCR10
CRTH2

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62
Q

what is methylisothaizoline in and where is it’s classic contact derm rash?

A

sunscreen, cosmetics, wipes, hair products, liquid soap, laundry products
face, hands, buttocks

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63
Q

what is reactive airways dysfunction syndrome?

A

type of occupational asthma
aka irritant induced asthma

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64
Q

labs findings in HP

A

BAL: CD4:CD8 <1
lymphocytosis >20%
elevated PMNs, eos, mast cells, Igs, hyaluronic acid

biopsy: noncaseating granulomas, mononuclear cell infiltrates

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65
Q

meds for idiopathic pulm fibrosis

A

nintedanib
pirfenidone

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66
Q

basics of exercise challenge test

A

2-3min warm up to reach target HR of 80-90% max HR
last 6 min in kids, 8 min in adults

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67
Q

sxs and gene of mevalonate kinase deficiency aka hyperIgD syndrome

A

MVK
periodic fevers, LAD, rash, oral ulcers, rash, GI sxs, splenomegaly autoinflammatory dz
triggers: illness, vaccinations

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68
Q

genes for T-B-NK- SCID (3)

A

ADA
RAC2
AK2

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69
Q

NEMO deficiency gene and s/s

A

IKBKG (NF kappa B essential modulator)
ectodermal dysplasia, infections - mycoplasma, pneumococcal, staph

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70
Q

WHIM syndrome s/s and gene

A

warts, hypogamm (can happen later), infections, myelokathexis (causes neutropenia bc can’t exit BM), T and B lymphopenia
GOF variant in CXCR4

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71
Q

which C’ deficiencies have normal CH50 but absent AH50

A

factor B, factor D, properdin

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72
Q

what is autophagy?

A

autophagosomes engulf cytosolic proteins (intracellular) and fuse with lysosome to enter the MHC II pathway to be presented to CD4+ T helper cells

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73
Q

what receptor expression is increased with IVIG and sends inhibitory signals to B cells?

A

FcyRIIB

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74
Q

what does activation of IL2 receptor gamma lead to? (immediately)

A

STAT5 phosphorylation

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75
Q

what is Siglec-8 and where is it expressed

A

inhibitory receptor
eos, mast cells > basophils

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76
Q

LAD type II s/s and gene

A

poor but not absent pus formation, Recurrent skin infections, microcephaly, developmental delay, short stature, periodontal infections and Bombay blood group (absence of H antigen, hh phenotype) delayed umbilical cord separation is not observed.

Mutations in SLC35C1 (encodes GDP-fucose transporter 1 (FUCT1) and leads to the absence of fucosylated carbohydrate ligands and absence of Sialyl-LewisX (sLeX) expression (CD15a))

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77
Q

cell surface markers on naive T cells

A

CD45A
L selection (CD62L)
CCR7

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78
Q

type I interferon function

A

(IFN alpha and beta)
key cytokine in antiviral response
induce gene expression that viral transcription, degrade viral RNA via RNases, and inhibit viral protein synthesis
sequester lymphs in lymphoid tissues

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79
Q

surface markers of effector T cell

A

CD25
CD45RO

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80
Q

what type of B cell has high rate of somatic hypermutation?

A

follicular B2 cells

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81
Q

staining for what can ID macrophages (e.g. Mac activation in HLH in BM)

A

CD163

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82
Q

NNT = ?

A

NNT = 1/ARR
ARR = control event rate - treatment event rate

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83
Q

which antihistamines don’t require adjustment for renal impairment

A

first gen
loratidine

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84
Q

most common lab technique to measure Igs

A

nephelometry

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85
Q

which proliferation assay gives % of proliferating cells with fluorescent dye dilution

A

carboxyfluorescein succinimidyl ester (CFSE) assay

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86
Q

what med affects results of BAT?

A

oral steroids

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87
Q

what is the major allergen of honeybee

A

phospholipase A2

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88
Q

which segment of the Ig is most specific binding site for 3D epitopes

A

complementary determining region = hypervariable region

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89
Q

how many globular domains in each types of Ig

A

4 in IgG, IgD, IgA
5 in IgM, IgE

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90
Q

What structural component of an immunoglobulin distinguishes isotypes from one another?

A

Fc portion (changes what they bind to and their effector functions)

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91
Q

proposed mechanisms of DRESS

A

drug detox enzyme abnormalities with accumulation of reactive frug metabolites
sequential reactivation of herpesviruses
HLA allele genetic predispositions

92
Q

BAL and biopsy in sarcoidosis

A

BAL showed increased lymph and Mac, increased CD4/CD8 ratio
biopsy showed noncaseating granuloma

93
Q

XMEN (x linked immunodeficiency magnesium defects-EBV infection-neoplasia) s/s and genetics

A

mutation in Mag transporter protein MAGT1
uncontrolled EBV viremia, lymphoma, cutaneous HPV infections

94
Q

what is affected in X linked T-B+NK- SCID

A

common gamma chain (CD132) of IL2 receptor gene IL2RG

95
Q

deletion of __ in the T lineage results in loss of Treg IPEX syndrome

A

dicer (endoribonuclease)

96
Q

Omenn syndrome mutations and affected process/enzyme

A

RAG1/2 - affects VDJ recombinase
ARTEMIS - affected hairpin opening, end processing and joining steps of VDJ recombination

97
Q

CD4:CD8 ratio normal and during acute infection

A

normal - 2:1
acute infection - 1:25

98
Q

most important cytokine for maintenance of memory T cells

A

IL7
IL15

99
Q

What is the underlying mechanism of cytotoxic T cell (CTL) exhaustion in chronic viral infections/tumors

A

expression of inhibitory receptors (PD1, CTLA4)

100
Q

Which adhesion molecules are associated with CTL-mediated target cell killing

A

integrin LFA-1 on CTL binds to ligand ICAM-1 on target cell

101
Q

T-independent cell-mediated immunity process is facilitated by __ activation

A

TLR activation

102
Q

what is the ligand for CCR9 and where is it found

A

ligand = CCL25
found in gut epithelium, facilitates gut homing of activated lymphocytes

103
Q

which HLA genotypes ahve highest risk of celiac disease

A

HLADQ2 and DQ8

104
Q

what induces CCR10 expression in the skin
AND
ligand for CCR10

A

Vit D binding to vit D receptor in effector T cells
CCR10 goes on to recognize its ligand CCL27 made by keratinocytes

105
Q

immune regulator proteins of airway

A

surfactant proteins A and D

106
Q

typical order of development of AERD

A
  1. CRSwNP
  2. asthma
  3. ASA hypersens
107
Q

target dose of ASA desens for different indications
- CV dz prevention
- maintain cross-desens to all NSAIDs
- AERD

A
  • CV dz prevention: 81mg
  • maintain cross-desens to all NSAIDs: 325 mg
  • AERD: 650mg BID
108
Q

How long does it typically take for cow’s milk and soy protein-induced proctocolitis to resolve after allergen avoidance?

A

6mth-2yr

109
Q

APS-1 (autoimmune polyendocrine syndrome type 1) basics

A

autoimmunity of endocrine organs + thrush
mutation in AIRE gene = failure of central tolerance
autoAb to IL-17 = susceptible to mucocut candidiasis

110
Q

CTLA-4 haploinsufficiency vs LRBA deficiency

A

CTLA-4 haploinsufficiency
- recurrent inf, hypogamm, lymphocytic infiltration into organs including lungs

LRBA deficiency
- AR
- LRBA is involved in endocytosis/recycling of CTLA-4
- mutations cause similar lymphoproliferative syndrome as CTLA-4

111
Q

IL10 deficiency

A

severe colitis before 1yo
decreased IL10 –> increased mac and DC activation –> uncontrolled lymph and mac activation –> increase MHC class II, IL12, and IFN gamma

112
Q

what does IL10 do

A

inhibits production of IL12 and class II MHC molecules by DC and macrophages
inhibits IFN gamma production

113
Q

what is CD95

A

death receptor FAS

114
Q

what is epitope spreading

A

immune response is initiated against a previously hidden self-antigen. The injury to tissues results in further release of other antigens with activation of lymphocytes specific to the other antigens. This leads to disease exacerbation

115
Q

which HLA is ass’d with ankylosing spongylitis>

A

HLA B 2705

116
Q

what mutations are ass’d with SLE

A

FcyRIIB
HLA DRB1-1501 and 0301

117
Q

functions of IL2 (5)

A

potent activator of the proliferation and function of T lymphocytes and natural killer cells
- T cell growth factor
-can augment natural killer (NK) cell cytolytic activity
-contributes to the development of (Treg) cells
-promotes immunoglobulin production by B cells
-regulates the expansion and apoptosis of activated T cells

118
Q

Which cytokine modulates eosinophilic production, maturation, activation, and survival in blood

A

IL5

119
Q

IgE binds to which subunit of FcepislonRI?

A

alpha

120
Q

The Early Asthmatic Reaction is a decline in respiratory function within how many minutes ofbronchial allergen challenge?

A

10-20min

121
Q

The percentage of which type of mast cell is increased in the skin of patients with atopic dermatitis?

A

MCt
normal skin is 90% MCtc

122
Q

AERD urinary measurement?

A

increased urinary LTE4 (reflects increased production of cysLTs)

123
Q

Which cytokine plays a significant role in the survival and activation of mature basophils?

A

IL3
basophils have high expression of IL3R (CD123)

124
Q

If there is an exposure to an allergen, the bone marrow activates and increasesthe production of the following cells:

A

eos
basophils

125
Q

Which cytokines inhibits the priming effects that IL-3 has on basophil function?

A

IFN alpha
IFN beta

126
Q

How do basophils provide signals for B cells to produce IgE?

A

Expressing CD40L and secreting IL-4 and IL-13

127
Q

Committed eosinophil precursors in the bone marrow can be identified by their expression of?

A

IL5R (CD125)
CCR3

128
Q

which eos granule [ ] in BAL fluid is correlated to bronchial hyperactivity in asthmatics?

A

major basic protein

129
Q

predominant metabolic of 5 lioxygenase pathway in eos?

A

LTC4 (then metabolized to LTD4 and LTE4)

130
Q

eos derived substances and their effects in pathogenesis of asthma
MBP/eos cat protein, EDN, EPX
MMP9
LTC4
TGFbeta

A

MBP/eos cat protein, EDN, EPX - epithelial injury
MMP9 - tissue damage and remodeling
LTC4 - smooth muscle contraction
TGFbeta - tissue remodeling and fibrosis

131
Q

cytokines released by Th17

A

IL17
IL22

132
Q

What is the primary determinant of the site of lung deposition, distribution of the drug within the lung, and resulting deposition efficiency?

A

size of aerosol particle

133
Q

most ultrasonic nebulizers operate at what frequency

A

> 1MHz

134
Q

Which HFA inhaled corticosteroid has a built-in spacer

A

flunisolide

135
Q

For patients with a history of severe anaphylaxis, what should be the initial dose for drug desensitization?

A

between 1/10,000 and 1/1,000,000

136
Q

In the airway epithelial cells, glucocorticoids suppress the expression and release of a number of inflammatory cytokines, chemokines, and growth factors likely by affecting which transcription factor?

A

NFkB

137
Q

Inhibitors targeted at which enzyme have been able to restore glucocorticoid sensitivity inpatients with steroid refractory asthma?

A

P38 MAPK (MAPK14)

138
Q

Which interleukin, associated with neutrophilic asthma, is elevated in sputum and serum samples from patients with severe asthma and has been shown experimentally to inhibit corticosteroid responsiveness in human bronchial epithelial cells?

A

IL17

139
Q

The primary toxicity associated with zafirlukast and zileuton affect which organ?

A

liver - elevated LFTs

140
Q

Which protein helps activated naïve T-cells remain in the lymph node?

A

CD69 - protein that binds S1PR1 and reduces its cell surface expression –> prevents naive T cell from migrating out of LN into blood (where [S1P] is higher)

141
Q

What do bone marrow-homing IgG-secreting plasma cells express? (2)

A

VLA4 and CXCR4

142
Q

Genetic deficiency in UNC-93B results in susceptibility to:

A

HSV infections or other DNA/RNA viruses

protein on ER required for endosomal localization and function of TLR 3, 7, 8, 9

143
Q

Type 1 interferons (IFN-α and IFN-β) actions? (5)

A

protect against viral infections by:
1) requesting lymphs in LN
2) up regulating class I MHC
3) increasing cytotoxicity of NK cells
4) promoting differentiation of naive T cells to Th1 cells

144
Q

avidity vs affinity

A

Avidity is a semiquantitative term used to describe the overall binding of antibodies to antigen at all antigen binding sites.
Affinity refers to the strength of a single antigen-antibody interaction.

145
Q

what Ab do immature B cells produce

A

membrane IgM

146
Q

what type of residue do m membrane forms of Ig heavy chains have

A

hydrophilic amino acid residues but hydrophobic transmembrane portion
cytoplasmic domains differ between isotypes

147
Q

where is the hinge region of the Ab located and what it is purpose

A

between CH1 and CH2
flexibility permits independent movement of antigen binding sites

148
Q

protein digestion in MCH I vs II pathway

A

MHC I: occurs in proteasome
MHC II: occurs in phagolysosome

149
Q

Which type of cellular receptor is an integral membrane protein with intrinsic catalytic enzyme domain(s) inthe cytoplasmic tail?

A

receptor tyrosine kinase

150
Q

What are the main ligands for the IL-1 receptor family?

A

IL1
IL18
both are involved in the activity of inflammasome

151
Q

JAK2 GOF disease

A

myelodysplastic syndromes

152
Q

which Ab are transferred to infants via breastmilk

A

IgG and IgA

153
Q

Which cytokine increases eosinophil apoptosis

A

IL4 (in contrast to most other Th2 cytokines)

154
Q

Activation of which receptors leads to eosinophil apoptosis?

A

FAS ligand receptor

155
Q

The 5-lipoxygenase pathway is most abundant in which cell type:

A

myeloid

156
Q

In granulomatosis with polyangiitis, which types of cytokines are increased in response to environmental stimuli and contribute to granuloma formation?

A

Th1/Th17 cytokines (IL17, TNF, IFN gamma)

157
Q

most common extrapulm manifestations of EGPA

A

mononeuritis multiplex

158
Q

what contributes to fibrosis in sarcoidosis?

A

down regulation of Th1 response (increased in initial phase of sarcoidosis)
release of TGFB and CCL10

159
Q

Mutations in the gene that encodes which chemokine receptor protects human cells from HIV infection?

A

CCR5

160
Q

What medications have been implicated in causing eosinophilia and pneumonitis

A

nitrofurantoin
semi-synthetic PCN
NSAIDs

161
Q

What is the defect in MHC class II deficiency?

A

Class II transactivator (transcriptional activator of MHC class II genes)
deficiency of RFX complex (binds to promotor of MHC class II genes)

162
Q

What contributes to the heterogeneity of MHC? (3)

A

1) replacement substations are more common than silent mutation
2) MHC molecules are codominants and alleles are expressed equally int he cells
3) products of individual MHC alleles/isoforms can differ from one another by up to 20 AAs, making each variant protein distinct

163
Q

transcription factor that is very important for dendritic cell development and for cross-presentation?

A

CXCR1 - uniquely expressed by DC subset that acts in cross presentation

164
Q

super antigens lead to massive production of cytokines by what cells

A

CD4 cells

165
Q

Differentiation of the multipotent progenitor cell into the common lymphoid progenitor requires signaling through which receptor?

A

FLT3 receptor bindings to its FLT3 ligand

166
Q

During which stage of B cell development, does the heavy chain rearrangement begin?

A

early pro-B cell stage

167
Q

The process of isotypic exclusion is:

A

Expression of only 1 of 2 light chains in a B cell, such that kappa chains are rearranged first and if unsuccessful, are followed by lambda chains.

168
Q

Structure of the pre-TCR is

A

BETA chain then pairs with pTalpha chain

169
Q

alpha chain locus is rearranged in what stage of T cell development

A

double positive stage

170
Q

the pre TCR signals constitutively via what protein

A

Lck

171
Q

what shuts off V alpha chain rearrangement in cells

A

recognition of self MHC: self peptide complex which positively selects the receptor

172
Q

What cytokine in required for Treg development?

A

IL2

173
Q

Which of B cell maturation steps is sometimes impaired in ataxia telangiectasia due to a mutation in the ATM gene?

A

class switching

174
Q

B cell coreceptor complex contains which cell surface proteins

A

CD19
CD21 (aka CR2)
CD81

175
Q

on what chromo is the locus encoding the k light chain gene

A

chromo 2

176
Q

what is VDJ recombination guided by

A

recombination signal sequences
(noncoding DNA sequences, found adjacent to point at which recombination takes place)

177
Q

what segments do Ig heavy vs light chain and TCR alpha vs beta have

A

Ig heavy chain and TCRbeta have VDJ
Ig light chain and TCRalpha have VJ

TCR have more J gene segments and there is greater diversification of the junction between gene segments during the process of gene rearrangement

178
Q

which NOD may be important in Crohn’s diseasde

A

NOD2

179
Q

chediak higashi gene and highlights

A

CHS1 mutation
defect in vesicular transport proteins needed for formation or exocytosis of melanosomes
sxs: partial loss of skin/hair pigmentation, HLH, inability to clear EBV

180
Q

are lesions borders in contact dermatitis poorly or well defined?

A

poorly defined because there can be secondary spread

181
Q

most frequent cause of occupational asthma in western countries

A

baking products

182
Q

what agent is used extensively in spray paints

A

hexamethylene diisocyanate
*carries better prognosis than other diisocyanates

183
Q

how do corticosteroids affect neutrophils and eos

A

inhibit apoptosis of neutrophils
promote apoptosis of eosinophils

184
Q

chemoattractant that attracts eosinophils to the airway mucosa in asthma?

A

CXCL5

185
Q

Which cytokines are produced most abundantly by CD4+ T lymphocytes in allergic asthmatic airway inflammation?

A

GM-CSF
IL4, 5, 9, 13

186
Q

which virus is ass’d with asthma exacerbations

A

rhinovirus C

187
Q

how long does cough need to be in children to be chronic

A

> 4 weeks

188
Q

most common cause of upper airway cough syndrome

A

bacterial sinusitis

189
Q

most common causes of chronic nonspecific cough in children (2)

A

asthma
protracted bacterial bronchitis

190
Q

how long does chronic cough from GERD take to improve after tx is started

A

up to 3 months for improvement (6 months to resolve)

191
Q

dominant allergen in egg white

A

ovomucoid (Gal d1)

192
Q

What percentage of children with milk allergy typically tolerate extensively heated milk in baked products?

A

75%

193
Q

heiner syndrome

A

food induced pulmonary hemosiderosis
recurrent PNA ass’d with pulm infiltrates and hemorrhea, hemosiderosis, GI blood loss, IDA, and FTT

most often ass’d with non-IgE mediated hypersensitivity to cow’s milk

194
Q

IL-33 may be important in anaphylaxis due to:

A

its direct induction of IgE sensitized mast cell degranulation and cytokine production

195
Q

main pathophysiologic feature of anaphylactic shock is:

A

fluid extravasation causing hypovolemia and hemoconcentration

196
Q

The reason that initial treatment for anaphylactic shock is unsuccessful is most often due to which of the following?

A

insufficient fluid resuscitation

197
Q

which medication is ass’d with enhanced susceptibility to episodes of scombroidosis?

A

isoniazid

198
Q

During an additive challenge for asthma, what percent decrease from baseline FEV1 is considered a positive response?

A

> 20%

199
Q

Which sulfite-containing medication is considered safe for patients with sulfite-induced asthma?

A

epinephrine

200
Q

what dz has MSG been ass’d with

A

persistent rhinitis

201
Q

In what gene does polymorphism modify the effect of farm environmental endotoxin exposure on allergic sensitization in children?

A

CD14

202
Q

what eye conditions can typical cyclosporine be used for

A

dry eye syndrome
AKC
VKC

203
Q

what allergy med interacts with theophylline

A

zileuton can cause increase in theophylline

204
Q

what characteristics in asthmatic children predicts ICS response (2)

A

AEC >300
aeroallergen sensitization

205
Q

what treatment does GLILD seem to respond to

A

systemic steroids

206
Q

diagnostic criteria of ABPS (3)

A

1) asthma or CF
2) sIgE to A fumigatus
3) total IgE >1000

207
Q

which meds blunt ASA reactions in AERD (3)

A

zileuton
xolair
montelukast

*mepo does not although it’s approved to treat asthma and NP in pts with AERD

208
Q

drug inducing most reactions

A

PCN

209
Q

most common intraop anaphylaxis agent in US

A

antibiotics

210
Q

MOA of benralizumab

A

targets alpha subunit of IL5R –> eos apoptosis by ADCC

Fc portion binds FcyRIII on NK cells

211
Q

which biologics for asthma haven’t shown a decrease in PO steroid in steroid dependent asthma

A

tezepelumab
xolair

212
Q

treatment of EGPA ass’d myocarditis

A

steroids + cyclophosphamide

213
Q

features of hypocomplementemic urticarial vasculitis syndrome

A

> 6months urticaria and low C1q +/- low C3/C4
angioedema, COPD, uveitisi/episcelritis, arthritis, glomerulonephritis

214
Q

DOCK8 and STAT3 LOF Job syndromes differentiators

A

DOCK8 - severe warts
Jobs - scoliosis, pneumatoceles

215
Q

cause of autosominal dominant IFNgR1 deficiency

A

mutation that removes signaling and recycling domains of IFNgR1 -> remnant protein dominantly interferes with signaling and accumulates on cell surface

216
Q

5 major organisms causing infections in CGD patients in N America

A

s aureus
serratia marsescens
burkholdia cepacia
nocardia
aspergillus

217
Q

hallmark NK cell deficiency infection

A

herpesvirus

218
Q

what innate signaling mechanism promote a Th17 response in candida infections

A

CLR signaling (innate immune receptor)

219
Q

chance of siblings being an HLA match

A

25%

220
Q

ILC3 are mimics of what T helper subset

A

Th17 - both depend on transcription factors RORgt and produce IL17 and IL22

221
Q

feature of eos activation

A

increased lipid bodies containing eicosanoid products

222
Q

what is TACE

A

TNF alpha converting enzyme aka ADAM 17 endopeptidase

secreted by TNF producing cells, cleaves membrane extension anchoring TNF to release it form the cell

223
Q

[ ] of free serum IgE in healthy humans

A

50-200 ng/mL (1-10 for other IGs)

224
Q

units for non standardized allergen extracts

A

PNU/mL or w/v

225
Q

what intervention is most strongly ass’d with decreased resp disease from air pollution

A

public policy measures

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