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AI boards > Key facts > Flashcards

Key facts Flashcards

1
Q

xthrough what does the spleen receive lymphocytes and antigen?

A

splenic artery
*no afferent lymphatic supply

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2
Q

Which Tregs express FOXp3?

A

natural T regs
(NOT induced Treg1 or 3)

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3
Q

what is Bruton’s tyrosine kinases required for? what do its defects lead to?

A

pro to pre-B cell > B cell maturation
XLA

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4
Q

pre B cell receptor chains and main signal

A

u heavy chain with surrogate light-chain (gamma5 + VpreB)
signals B cells to stop heavy chain gene rearrangement

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5
Q

VDJ recombination results in __ diversity
Addition of nucleotides by TdT (terminal
deoxynucleotidyl transferase) and removal of endonuclease results in __ diversity (greatest variability for diversity)

A

combinatorial
junctional

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6
Q

most common form of T-B-NK+ SCID mutation in _, which affects _

A

RAG1 or 2
several proteins involved in VDJ recombination

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7
Q

what chemokines attract neutrophils to tissue

A

IL8 (CXCL8)
LTB4

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8
Q

defects in types of LAD

A

Type 1 LAD: defective CD18 integrin *Defective adhesion
Type 2 LAD: absent sialyl lewisX *defective rolling
Type 3 LAD: defective kindlin-3 which activates integrin *defective adhesion

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9
Q

Which cells secrete cytokines that activate neutrophils>

A

Th17

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10
Q

neutrophil granules in chediak-higashi syndrome vs specific granule deficiency

A

CHS - 1* granules enlarged
specific granule deficiency - 2* granules absent

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11
Q

what is FCepisolonRI expressed on?

A

mast cells
basophils
NOT EOS

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12
Q

what is KIT

A

receptor for cytokine stem cell factor
critical growth factor of mast cells and target of imatinib
encoded by photo-oncogene c-KIT

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13
Q

how does IgE bind to FCepisilonRI
main proteins in mast cell signaling

A

binds via alpha chain
proteins: lyn, fyn, syk

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14
Q

where does Xolair bind?

A

CH3/Fc (heavy chain) domain of free IgE, at FCepisilonRI binding site
Cepisilon3

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15
Q

do basophils produce tryptase, chimase, carboxypeptidase, heparin, PGD2, and LTB4?

A

NO

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16
Q

major basic protein

A

from EOS -> MC and basophils degranulate -> histamine release
can be used to detect recent presence of EOS in tissue

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17
Q

1 cause of marked eosinophilia in US vs worldwide

A

US: drug reactions
world: parasitic infection

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18
Q

CD14

A

highly expressed receptor on monocytes and macrophages
binds LPS and activates immune response
component of TLR4

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19
Q

mutation in what gene cause X linked lymphoproliferative syndrome

A

SH2D1A (encodes SAP protein)
*susceptible to HLH from EBV

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20
Q

langerhans cell histiocytosis

A

rare malignancy of langerhans cells
s/s: eczema, seborrhea, lytic bone lesions, mutlisystem involvement
dx: biopsy with positive staining for CD1a and CD207 (IDs langerhans cells)

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21
Q

do intestinal epithelial cells present Ag from gut lumen via MHCI or II

A

MHCII

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22
Q

IL4 and IL13 suppress __, contributing to pathogenesis of AD?

A

beta defensins
cathelicidin

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23
Q

stem cell factor (SCF)

A

aka kit ligand
signals through c-kit receptor (CD117)

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24
Q

what do platelets from patients with asthma release?

A

CCL5
IL33

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25
Q

intracellular TLR

A

NEST
9
8
7
3

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26
Q

which TLR signaling is MyD88 dependent pathway not involved in?

A

TLR3

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27
Q

which TLR can signal through both MyD88 dependent and MyD88 independent pathways

A

TLR4

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28
Q

which diseases are activating mutations in NLRP3 associated with

A

hereditary periodic fever syndromes
- familial cold auto inflammatory syndrome (FCAS)
- muckle-wells syndrome (MWS)
- chronic infantile neurologic cutaneous and articular syndrome (CINCA)

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29
Q

HOW CAN YOU DISTINGUISH plasmacytoid DCs from other types of DCs?

A

lack of CD11c expression (they express CD123)

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30
Q

most variable part of Ig molecule?

A

CDR3

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31
Q

what is the hapten carrier effect

A

allows for small molecules that cannot activate T cells to stimulate immune responses
basis for many drug allergies

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32
Q

where do superantigens bind?

A

Vbeta region of TCRs (outside of peptide binding groove on MHC molecule)

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33
Q

what is MHC restriction?

A

when T cells can recognize and respond to a peptide only if bound to a specific MHC molecule

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34
Q

NK T cells recognize lipids and glycolipids displayed by class I MCH-like molecule __

A

CD1

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35
Q

which region of the TCR imparts the most significant sequence variability?

A

alpha-beta CDR3 region

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36
Q

what process creates TCR vs BCR/Ig diversity?

A

TCR: VDJ recombination
BCR/Ig: class switching, somatic mutation

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37
Q

shared mechanism of cyclosporin and tacrolimus?

A

inhibit calcineurin
-> block translocation of NFAT into the nucleus
-> prevent transcription of IL2 and other cytokines

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38
Q

disease from AIRE mutations

A

autoimmune polyglandular syndrome (APS-1) aka APECED
- lymphocytes are not deleted or tolerized to endocrine self antigens during selection in thymus -> endocrine organ attacked by self reactive T cells and autoAb

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39
Q

order of BCR editing

A

kappa light chain rearranged
then if receptor editing needed, lambda light chain used

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40
Q

when does lymphocyte become anergic?

A

T lymph recognizes Ag repeatedly without costimulation

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41
Q

FOXP3 mutation disease

A

IPEX = watery diarrhea + eczema + endocrinopathy
Immune dysregulation
Polyendocrinopathy
Enteropathy
X-linked

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42
Q

ALPS mutations

A

in Fas or caspase 10
ALPS = autoimmune lymphoproliferative syndrome
lymphs accumulate in peripheral lymph organs
lack of tolerance causes autoimmune features, esp cytopenias

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43
Q

how do CTLs and helper T cells cause cytokine mediated damage to grafts?

A

direct and indirect alloantigen recognition
*only CTLs generated by direct allorecognition can kill graft cells

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44
Q

which transplanted tissues don’t require immunosuppression?

A

cornea
bone
joint tissue

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45
Q

what is a syngeneic transplant

A

genetically identical, from twin

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46
Q

what is a xenogeneic transplant

A

across species

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47
Q

what is considered a match for umbilical cord blood and for adult donors?

A

UCB: 6 out of 6 at A, B, and DRB1 alleles
adult donor: at least 6 out of 8 at A, B, C, and DRB1 alleles

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48
Q

how are leukemia cells killed in graft vs leukemia?

A
  • graft T lymphs contribute to eradication of tumor
  • recipient NK cells express HLA I that is different than donor HLA I -> results in donor NK mediated cellular killing of leukemic cells (killer inhibitory receptors KIRs aka inhibitory killer Ig-like receptor on donor NK cells are inhibited by cells that display HLA I markers that they recognize)
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49
Q

what is the major factor in long term survival in SCT?

A

chronic GVH reaction

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50
Q

where are HLA-G and F expressed and purpose

A

extra villous fetal trophoblast
protect fetus from maternal immune rejection

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51
Q

what explains the long half life of IgG?

A

FcRn protects from catabolism

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52
Q

IL-1-5 main actions

A

Hot T Bone stEAk
IL1: hot, fever
IL2: T lymph stim
IL3: Bone marrow stim
IL4: IgE stim
IL5: IgA stim

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53
Q

IL1beta maturation mediated by_
ass’n mutation

A

caspase-1, requires activation by multiple proteins including NALP (NLRP)
GOF mutations in NALP causes uncontrolled IL1 production and autoinflammatory syndromes
- can be treated with IL1 receptor antagonists (anakinra, canakinumab, rilonacept)

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54
Q

IL-12Rbeta1 mutation cause susceptibility to _?

A

infections with intracellular bacteria
*salmonella, atypical mycobacteria

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55
Q

what receptor is shared by IL-4 and IL-13

A

IL4Ralpha (dupixent target)

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56
Q

which receptors share a common beta chain (CD131)

A

IL3
IL5
GM-CSF

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57
Q

what is hyperproduced in castleman’s disease and what does it cause?

A

IL6
causes angiofollicular LN hyperplasia -> pts p/w fever, microcytic anemia, LAD, normal BM iron, hypoalbumin, and elevated CRP

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58
Q

which receptors all contain the common gamma chain (affected in SCID)?

A

IL2
IL4
IL7
IL9
IL15
IL21

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59
Q

IRAK-4 deficiency leads to susceptibility to __

A

pyogenic infections
*strep pneumo, staph aureus, pseudomonas aeruginosa

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60
Q

which chemokine expression is increased in skin of eczema patients

A

CCL27

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61
Q

what activates CCR3 receptor and what does it cause?

A

“Three-osinophil”
CCR3 receptor activation by eotaxin leads to eos recruitment to tissues

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62
Q

defect in LAD2

A

PMNs can’t express carbohydrate ligands (sialyl lewisX) for E and P selectin

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63
Q

what does fourth beta integrin family alpha4beta7 do?

A

mucosal addressin that binds to mucosal addressin cell adhesion molecule (MAdCAM), important for gut homing

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64
Q

what stabilizes Factor B in the alternative complement cascade?

A

Properdin
*only known positive regulator of complement

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65
Q

Binding affinity of C1q to IgG subtypes and IgM (classical complement cascade)

A

IgM > IgG3, IgG1, IgG2
IgG4 cannot bind C1

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66
Q

rate potency of anaphylotoxins

A

C5a >C3a>C4a

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67
Q

what complement receptors are markers for dendritic cells?

A

CR4, CD11c

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68
Q

What does CR2 (CD21) bind and do?

A

binds C3d
provides 2nd signal for B cell activation by antigen, helps trap Ab-Ag complexes in germinal center

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69
Q

what is DAF? (in complement system)

A

decay activating factor
dissociates C3 convertase

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69
Q

which complement receptors are used for cell entry or phagocytosis by microorganisms?

A

CR1 by HIV
CR2 by EBV
CR3 by mycobacterium tuberculosis

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70
Q

complement deficiencies in SLE

A

C1q»C1r/C1s, C4, C2

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71
Q

C1 inhibitor key points

A
  • sole plasma inhibitor of factor XIIa and factor XIIf, a cleavage product derived from factor XIIa by digestion with kallikrein or plasmin
    one
  • one of the major inhibitors of kallikrein, factor XIa, C1r, and C1s
  • consumed by plasmin
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72
Q

what does a negative DTH test indicate?

A

lack of exposure to antigen
OR
anergy as a result of 1* or 2* cellular immunodeficiency

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73
Q

effect of histone acetylation/deacetylation
*COPD link?

A

histone acetylation –> opens chromatin/allows transcription
histone deacetylation –> repressed gene expression *reduced in COPD

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74
Q

in what type of study is OR most often used

A

case-control study

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75
Q

in what type of study is RR most often used

A

cohort

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76
Q

what 2 signals induce IgE production:

A
  1. Th2 lymph secreted IL4/IL13
  2. CD40-CD40L B and T cell interaction
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77
Q

___ is a sensitive markers of type 2 inflammation that is increased in AR and decreased in sinusitis

A

exhaled nitric oxide

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78
Q

what type of MOA do H1 antihistamines have?

A

inverse agonists
downregulate H1 receptor constitutive activity

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79
Q

what patients have more severe alcohol induced rhinitis

A

patients with AERD or CRSwNP (w/ or w/o asthma)

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80
Q

what type of sinusitis is a surgical emergency?

A

isolated sphenoid sinusitis
refer to ENT immediately

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81
Q

what type of patients does AFRS affect

A

young, immunocompetent
*it’s noninvasive

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82
Q

what are signs of moderate to severe AFRS

A

bony expansion or erosion leading to double vision, proptosis, periorbital edema, focal neuro signs, severe HA, meningeal signs
*urgent referral to ENT

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83
Q

what staining is required to identify fungal hyphae in mucin?

A

PAS or GMS staining
*they are usually sparse

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84
Q

giant papillary conjunctivitis is associated with ___ and has ___

A

ass’d with contact lens use
has tarsal papillary hypertrophy (typically smaller than VKC - <0.3mm - but can increase to 1-2mm with dz progression)

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85
Q

where do the various sinuses drain?

A

“Sinuses listen to radio channels - FM, AM, PS, SS”

Frontal and Maxillary and anterior ethmoid drain to middle meatus

posterior ethmoids and sphenoid drain into sphenoethmoidal recess above superior turbinate

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86
Q

useful PCD screening test

A

very low or absent nasal nitric oxide

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87
Q

PCD patients have MORE or LESS airway infections than CF patients

A

LESS
*suggest that altered mucus plays a larger role in dz progression than ciliary mvmt in chronic bacterial infections

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88
Q

what % of patients with PCD have situs inversus

A

50%

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89
Q

what % of adults does chronic cough affect?
peak onset age?

A

10-12% adults F>M
peak onset in 6th decade

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90
Q

most likely causes (4) of chronic cough in non-smoking immunocompetent adult with normal CXR and not taking ACEi

A

upper airway cough syndrome
asthma
non-asthmatic eos bronchitis
GERD

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91
Q

clinical markers of irritant contact derm

A

sharp borders
acute onset (w/in min to hr)
rapid peak and variable time course to resolution

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92
Q

what type of hypersensitivity rxn is allergic contact dermatitis

A

type VIa/c
typical onset 24-72 hours after exposure often with exposure to low molecular weight hapten molecule (after prior sens)

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93
Q

what bug is a common cofactor in seborrheic dermatitis

A

malassezia

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94
Q

what cytokine is most strongly associated with pruritus in AD

A

IL31

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95
Q

most frequently associated environmental allergen for triggering atopic dermatitis flares

A

DM

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96
Q

association of anterior vs posterior subcapsular cataracts

A

anterior = Atopkic keratoconjunctivitis
posterior = Prednisone

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97
Q

what contact dermatitis materials to think of with day 6 or 7 delayed peak reaction

A

corticosteroids
neomycin
nickel
gold

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98
Q

cholinergic urticaria can present similarly to __

A

exercise induced anaphylaxis
can even have hypotension
pts with EIA won’t react with passive heating

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99
Q

cold urticaria syndromes with negative ice cube test (6)

A

PLAID
delayed cold urticaria
cold induced cholinergic syndrome
systemic cold urticaria
familial cold autoinflam syndromes
cold dependent dermatographism

100
Q

most common cause of acute angioedema presenting to the ED

A

ACEi

101
Q

most common genetic abnormality resulting in HAE

A

SERPING1 on chromo 11

102
Q

estrogens in HAE?

A

should be avoided or used with extreme caution

103
Q

what acute HAE treatment is approved for all peds patients and pregnancy/breastfeeding

A

plasma derived C1 INH

104
Q

icatibant MOA and ages

A

for acute HAE attacks
bradykinin B2 antagonist
approved for 18+ but can be used down to 2yo

105
Q

oral HAE ppx options

A

berotralstat (kallikrein inhibition) *only 12+
androgens *adults

106
Q

langerhans cell histiocytosis presentation

A

caused by clonal proliferation of langerhans cells
nonspecific inflame response and multifocal organ involvement *bone, skin, BM, LN, endocrine, lungs

107
Q

what is filaggrin
what have mutations in filaggrin been linked to

A

matrix protein promoting aggregation and disulfide boning of keratin filaments
AD, asthma, FA, ichthyosis vulguris

108
Q

where are connective tissue mast cells vs mucosal mast cells

A

CT MC: have tryptase + chymase (MCtc)
- in skin, conjunctiva, heart, intestinal submucosa
- have CD88 receptor for C5a anaphylatoxin
- responds to vanc, opioids

mucosal MC: have tryptase (MCt)
- in alveolar wall, resp epithelium, and small intestinal mucosa

109
Q

biopsies for suspected immunobullous disease

A

2!
One for intact vesicle/bullae and one for perilesional skin (for immunofluorescence since immunoreactants may not be present in lesions tissue)

110
Q

Ab to desmoglein and epidermal immunoflouorence =?

A

pemphigus disease
(pemphigus vulgaris, pemphigus follaceous, paraneoplastic pemphigus, IgA pemphigus)

111
Q

accumulation of __ in the airway is a hallmark of fatal asthma

A

neutrophils

112
Q

what are the biomarkers for Type 2 Th2 high asthma

A

elevated total IgE
+sIgE
elevated FeNO

113
Q

what are mucus plugs composed of?

A

serum proteins
inflammatory cells
mucus
cellular debris

114
Q

pathognomonic findings that should make you consider CF (4)

A

hypoNa, hypoCl, meta alka
rectal prolapse
nasal polyposis
digital clubbing

115
Q

when to consider ABPA in CF pt

A

continues to clinically worsen despite 1-2 weeks of antibiotics

116
Q

what is echogenic bowel on prenatal US suggestive of

A

CF

117
Q

sputum cell count test can be used to diagnose? (2)

A

eosinophilic bronchitis or occupational asthma induced by high molecular weight agent

118
Q

what percentage of adult onset asthma cases are occupationally related?

A

2-25%

119
Q

occupational rhinitis typically precedes occupational asthma for __ molecular weight agents

A

HIGH

120
Q

years for animal lab workers to sensitize for occupational asthma/sxs

A

2 years
*flour workers take much longer

121
Q

plicate acid (anhydride) activates ___

A

complement

122
Q

what industry is OA from isocyanates seen

A

roofers, insulators, painters including auto body
*can induce both immunologic and non-immunologic mechanisms

123
Q

is ABPA due to fungal colonization or local invasion?

A

fungal colonization

124
Q

does absence of IgE sensitization to aspergillum exclude ABPA?

A

yes

125
Q

treatment goal of ABPA

A

prevent development or progression of bronchiectasis and worsening of pulm function

126
Q

what lab can help predict ABPA recurrence?

A

rising IgE levels *esp doubling of baseline IgE level

127
Q

what can cause intermittent symptoms in hypersensitivity pneumonitis

A

episodic exposure to inciting agent

128
Q

how does cigarette smoking impact risk of HP

A

decreases risk

129
Q

helpful clues to recognize HP (6)

A
  1. hx of recurrent atypical PNA
  2. sxs develop after moving to new job or home
  3. sxs improve when away from work/home
  4. unusual exposures or hobbies
  5. lab workers
  6. hay handling
130
Q

how much more/less common is organic dust toxic syndrome than farmers lung HP?

A

ODTS is 50x more common than farmers lung

131
Q

what is the most common extra-pulm feature of EGPA

A

mononeuritis multiplex = periph neuropathy due to vasculitis involving the vasa nervorum

132
Q

basics of:
eos granulomatosis with polyangitis VS
granulomatosis with polyangitis (Wegners) VS
microscopic polyangitis

A

EGPA: sinus dz + asthma + eos >1500 + neuropathy +/- MPO or p ANCA
GPA: sinus + lung + kidney dz +/- PR3 or c ANCA
MPA: lun g+ kidney dz +/- MPO or p ANCA no sinus or granulomatous inflam

133
Q

sarcoidosis vs HP BAL CD4:CD8 analysis

A

sarcoidosis (one word) = higher CD4 (lower number)
HP (2 words): higher CD8 (higher number)

134
Q

leading cause of mortality in EGPA

A

cardiac involvement

135
Q

alpha1 antitrypsin should be obtained for which patients?

A

all patients with sxs of COPD and persistent airflow obstruction on spirometry, esp if <45yo nonsmoker

136
Q

CXR findings in COPD

A

lung hyperinflation and hyper lucency
rapid tapering of vascular markings
*doesn’t help dx but can exclude other dx

137
Q

loss of lung function in smokers vs nonsmokers

A

smokers decline in FEV1 60ml/year
normal loss 30mL/year

138
Q

which treatments prolong life in COPD (3)

A

smoking cessation
supplemental O2 used 24hr/day
lung volume reduction surgery

139
Q

what airway epithelial cells are the major producers of lung surfactant

A

type II pneumocytes

140
Q

are neutrophils and eos permanent residents of the lung

A

NO - recruited there during inflammation

141
Q

definition of significant bronchodilator response in adults and children

A

adult: FEV1 or FVC increase >10% relative to predictive value
children: FEV1 increase >12% from baseline

142
Q

can you exclude seasonal pollen induced asthma with methacholine challenge or histamine challenge in winter

A

no

143
Q

selective vs nonselective bronchoprovocation testing

A

selective aka allergic agents - looks at specific triggers in susceptible asthmatics
nonselective - potential to induce bronchoconstriction in all asthmatics

144
Q

direct vs indirect bronchoprovocation testing

A

direct: acts on smooth airway muscle receptors (muscarinic and histamine)
indirect: mediator release is needed for actions

145
Q

sens/spec of methacholine and mannitol testing for asthma dx

A

methachOline - high sens, useful to rule Out asthma
mannItol - high spec, useful to rule in asthma *esp exercise induced

146
Q

factors that increase (7) vs decrease (6) FeNO

A

increase:
1. asthma
2. atopy
3. URIs
4. >12yo
5. nitrogen rich foods
6. COPD exacerbation
7. non asthmatic eosinophilic bronchitis

decrease:
1. bronchiectasis
2. tobacco smoke
3. drugs - steroids, antileukotrienes, oxymetazoline, NOS inhibitors
4. exercise
5. moderate altitude
6. hypothermia

147
Q

highest risk factor for developing IgE mediated food allergy

A

AD
*filaggrin LOF variant also confer risk independent of AD

148
Q

what percentage of egg and milk allergic kids can tolerate baked egg and milk

A

60-70%

149
Q

latex fruit allergy syndrome

A

~30-50% of latex allergic patients haver hypersensitivity to some plant derived foods, esp fresh fruits: Avocado, Banana, Chestnut, kiwi, white potato, bell pepper, tomato

150
Q

linear vs conformational epitopes in food allergy

A

linear = more prolonger allergy, allergen is stable and persistent
conformational = mild and transient allergy

151
Q

what % of drug reactions does drug allergy account for?
type A or B reaction?

A

10%
Type B reaction

152
Q

risk factors for drug allergy

A

latex or radiocontrast reactions
*not atopy

153
Q

what is the hapten hypothesis?

A

small drugs which are not by themselves immunogenic become immunogenic or allergenic after bindings to a carrier protein to form a hapten-carrier complex

154
Q

what is the most common form of delayed drug reaction

A

maculopapular (morbilliform) drug eruption

155
Q

what to screen for prior to starting abacavir

A

HLA B*57:01

156
Q

decrease in PCN-specific IgE Ab over time

A

~10% per year
- 50% with immediate rxn negative at 5 years, 80% at 10yrs
*similar for cephalosporins

157
Q

common drug that causes IgA bullous dermatitis

A

vAncomycin

158
Q

during ASA desens for AERD, do reactions typically occur at lower or higher doses

A

lower (~40mg)

159
Q

what is anaphylaxis to cetuximab due to

A

IgE against carbohydrate galactose alpha-1-3-galactose

160
Q

levels of __ correlate with severity of anaphylaxis

A

PAF - platelet activating factor
PAF acetylhydrolase levels correlate inversely with PAF levels/anaphylaxis severity

161
Q

receptor on mast cells that can be activated by small molecular weight drugs resulting in degranulation

A

MRGPRX2 receptor

162
Q

most common cause of fatality in anaphylaxis

A

circulatory collapse or resp failure

163
Q

definition of significant increase in tryptase

A

(baseline tryptase x1.2) + 2

164
Q

what is over sulfated chondroitin sulfate

A

contaminant in heparin caused by activation of the contact system with elevated C5a –> hypotension, abd pain, variable angioedema
*typically without urticaria or pruritus

165
Q

most common cofactors in exercise induced anaphylaxis

A

NSAIDS
wheat

166
Q

most common food implicated in food dependent exercised induced anaphylaxis

A

wheat (omega 5 gliadin)

167
Q

most commonly identified latex allergens

A

Hev b1 and 3 - patients with multiple surgeries, spina bifida
Hev b5, 6.01, 6.02 - healthcare workers

168
Q

which Hymenoptera are most cross reactive

A

hornets and yellow jackets - both vespers
vespid

less so:
-honeybee and Yellowjacket due to cross reacting carbohydrate determinants
-bumblebee and honeybee variable

169
Q

what causes sterile pustule from fire ant venom

A

venom is 95% piperidine alkaloids

170
Q

when are whole body extracts routinely used for venoms

A

fire ant only

171
Q

what % of asymptomatic healthy adults are sensitized to Hymenoptera skin tests

A

20% - only 5-15% of these individuals will have systemic reaction to subsequent sting

172
Q

Hymenoptera intradermal testing [ ] and starting dose of VIT

A

intradermal testing [ ]: 1mcg/ml
starting dose of VIT: 1mcg

173
Q

what insect is most common cause of systemic reaction from BITING insect

A

kissing bug (Triatoma)
nocturnal painless bites in Western and southern US/Mexico

174
Q

what cytokine is required for mast cell survival

A

SCF
is ligand for KIT (CD117)

175
Q

most common form of mastocytosis in children

A

cutaneous
*often spontaneously resolves by puberty, persistence after puberty is indication for BM biopsy

176
Q

how many organ systems need to have objective findings for MCAS diagnosis

A

2 organ systems

177
Q

principle methods of volumetric air sampling

A

impaction - slit, rotating arm, or sieve samplers
impingement - air drawn into liquid, particles suspended in fluid
filtration - particles sucked through filter

178
Q

major northern vs southern grasses

A

northern: Timothy, rye, bluegrass, sweet vernal
southern: Bahia, bermuda, johnson

179
Q

grass cross reactivity

A

bermuda - other members of subfamily chloridoideae
Bahia and Johnson have limited cross reactivity
Timothy + sweet vernal - subfamily poodieae

180
Q

foods that cross react with birch

A

ABC PPPP
Apple, apricot
BIRCH
celery, cherry, carrot
pear, peach, plum, potato

181
Q

which mold sensitization is a risk factor for fatal asthma

A

alternaria

182
Q

major outdoor vs indoor molds

A

outdoor: ACE = alternaria, cladosporium, epicoccum
indoor: aspergillus, penicillium

183
Q

what is pancake syndrome

A

storage mites cause oral mite anaphylaxis after consuming wheat flour contaminated with storage mites

184
Q

homology between Der p1-Der f1 and Derp2-Der f2

A

> 80% homology

185
Q

how long after cat removal are cat allergen levels significantly removed

A

4-6 months

186
Q

what dog allergen is associated with tolerating female dogs or castrated male dogs without sxs

A

monosensitization to can f5

187
Q

which genotypes have increased susceptibility to pro-oxidant effects of pollutants contributing to airway inflammation

A

null genotypes in antioxidant enzymes (GSTM1, GSTP1)

188
Q

effect of global climate change on pollen seasons

A

earlier, prolonged, and increased pollen seasons
particulate matter may also enhance delivery of pollen to the airway

189
Q

standardized allergen extracts

A

hymenoptera - mcg protein (hyaluronidase and phospholipase)
DM - AU
cat hair/pelt - BAU
grass - BAU
short ragweed - Amb a1, AU or wt/vol

190
Q

allergen extracts you cannot mix

A

cockroach or mold with pollen
venom and aeroallergens

191
Q

what type of hypersensitivity reaction is serum sickness

A

type III
Immune complexes

192
Q

rapidly progressive glomerulonephritis

A

crescent glomerulonephritis and rapidly deteriorating renal function
seen in anti GBM dz, pauci immune glomerulonephritis, and immune complex mediated disease

193
Q

drug induced lupus

A

occurs months after medication exposure
sxs similar to SLE
anti-histone Ab
meds: procainamide, hydralazine, penicillin, minocycline, diltiazem, isoniazid, anti-TNF

194
Q

drug induced subacute cutaneous lupus

A

photo distributed rash
+anti-Ro/SSA

195
Q

what can prodromal phase of bullous pemphigoid be

A

urticarial without bullae

196
Q

eye drop that can help distinguish episcleritis from scleritis

A

1 drop of 10% phenylephrine will block episcleral redness within 20min, but scleritis will persist

197
Q

what is proteinase 3

A

serine protease enzyme expressed mainly in neutrophil granulocytes
epitope of c ANCA Ab
ass’d with granulomatosis with polyangiitis (wegners)

198
Q

causes of caseating vs noncaseating granulomas

A

caseating - generally infectious
noncaseating granulomas - generally noninfectious

199
Q

what is castleman’s disease

A

aka angiofollicular LN hyperplasia
heterogeneous group of lymphoproliferative disorders that share common path

ass’d with nonHodgkin and Hodgkin lymphoma and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes)

elevated IL6 activity -> cytokine storm with hypogamm and amyloidosis

200
Q

HSCT survival rate for SCID prior to 2mo

A

95%
declines rapidly after 6mo

201
Q

what is the only complement deficiency with X-linked inheritance

A

properdin deficiency

202
Q

what percentage of the population have MBL deficiency?

A

5%

203
Q

what complement deficiency is associated with the greatest risk of SLE

A

C1q

204
Q

what gene defects related to TLR signaling cause severe influenza infection

A

IRF7 and IRF9

205
Q

defects in which pathway are associated with Mendelian susceptibility to mycobacterial disease

A

IL12/IFN gamma

206
Q

what gene mutation confers resistance to HIV infection acquirement

A

double allelic CCR5 32 mutation (CCR5 is a coreceptor that allows HIV entry into cells)
single allelic mutation ass’d with slower progression

207
Q

what is the hypergamm in HIV infection from

A

Ab response to HIV
polyclonal activation of B cells

208
Q

how does ritonavir affect inhaled or intranasal steroids

A

strong CYP3A4 and CYP2DG inhibitor -> can increase inhaled or intranasal steroids to systemic levels leading to Cushings or diabetes

209
Q

sxs of multiple myeloma

A

CRAB
Calcium high
Renal failure
Anemia
Bone abnormalities

210
Q

griscelli vs chediak-higashi hair shafts

A

griscelli - larger irregular clumps of melanin granules mainly near medulla, hair shaft appears uniformly white under polarized light microscopy

chediak-higashi - small aggregates of clumped pigmentation that are evenly distributed, hair shaft appears bright and polychromatic under polarized light microscopy

211
Q

most over expressed gene in EOE

A

eotaxin3 aka CCL26
receptor = CCR3
eos chemotactic
increased by IL13

212
Q

most common food trigger in EOE

A

milk

213
Q

when treating patients for hypereos syndrome with steroids, remember:

A

to treat strongyloides empirically with ivermectin for patients with possible exposure to avoid disseminated disease

214
Q

major integrin on gut homing B and T cells
what it binds to and other related proteins

A

major integrin: alpha4-beta7
binds to MADCAM1 on postcapillary venular endothelial cells
homing also requires CCR9 receptor on B and T cells and CCL25 ligand on epithelial cells
retinoid acid induces expression of these gut homing molecules

215
Q

MOA of vedolizumab

A

monoclonal Ab against Alpha chain of alpha4-beta7
used in IBD tx

216
Q

antihistamines to sue in renal or hepatic failure

A

renal: Claritin or 1st gen
hepatic: alllllegra (lllliver) *avoid first gen due to inc adverse effects

217
Q

what type of receptor does albuterol bind to

A

G protein coupled receptor –> increases cAMP

218
Q

what type of inhalers are contraindicated for asthma treatment

A

mono LABAs

219
Q

what is the duration of action of beta agonists dependent on

A

bulk and size of beta agonist structure

220
Q

examples of ultra long acting beta agonists (3)

A

carmoterol
indaceterol
vilanterol

221
Q

what monitoring is needed on zafirlukast and zileuton therapy

A

liver function

222
Q

which part of allergic response to antigen challenge is preventing with LTRA and mast cell stabilizers?

A

both early and late response

223
Q

effect of blocking M2 vs M3 muscarinic receptors

A

M2 (inh) -> inc acteylcholine -> bronchoconstriction

M3 (stim) -> dec acteylcholine release -> bronchodilation

224
Q

which part of allergic response to antigen challenge is prevented with steroids?

A

late phase response only

225
Q

ciclesonide is what type of drug

A

prodrug with little binding affinity
requires conversion by esterase’s in the airway to the active metabolite (desisobutyryl-ciclesonide) for efficacy, reducing potential systemic side effects

226
Q

how many heavy chains are in the constant regions of IgG/A/M/E

A

4 heavy chains in IgE and IgM
3 heavy chains in IgA and IgG

227
Q

what infection has been associated with mepolizumab

A

herpes zoster

228
Q

through what pathway does Alum work as an adjuvant as

A

the NLRP3 pathway involving the inflammasome

229
Q

at what dose is acetaminophen typically tolerated in AERD pts

A

<1000mg

230
Q

ID skin testing to __ has risk of anaphylaxis

A

food
latex

231
Q

venom ID skin tests are performed with up to what [ ]?

A

1mcg/mL

232
Q

effect of Xolair on total and sIgE

A

increases IgE

233
Q

what Ig don’t activate complement

A

IgG4
IgE
IgD

234
Q

when/where are CD19 (what does it associate with) and CD20 seen

A

CD19: early B cell progenitors until Cell plasma blast stage
CD20: pre-B cells and B cell blasts but not in early B cells or plasma cells

CD19, CD21, CD81 is coreceptor complex that functions to amplify BCR generated signals

235
Q

what does activating dominant mutation in CXCR4 cause

A

WHIM syndrome
warts
hypogamm
infection
myelokathexis

236
Q

what defect is responsible for LAD type 1

A

defect in common beta chain in CD18

237
Q

what is OKT3

A

Monoclonal antiCD3 Ab used in treatment of solid organ transplant rejection and acute T cell ALL

Leads to activation then apoptosis of T cells causing immunosuppression

238
Q

high levels of what type of B cells occur in some CVID patients

A

CD21-low B cells

239
Q

what receptor does IVIG exerts its action through

A

binding to FcyRIIb

240
Q

most common genetic variant in CVID

A

TACI

241
Q

what is the most potent complement receptors and what does it bind

A

CR3 = most potent
binds iC3b (opsonin like Ig)

242
Q

hereditary C3 deficiency leads to:

A

defective phagocytosis of encapsulated bacteria
presents with autoimmune disease and recurrent infections

243
Q

coreceptors for HIV entry into CD4+ T cells

A

CCR5
CXCR4

244
Q

CGD - gene mutations affect __
susceptible to what infections

A

NADPH oxidase system
catalase + organisms

245
Q

most common cause of low complement

A

poorly handled specimen

246
Q

when does anaphylaxis to cetuximab occur

A

when there are preexisting IgE Ab to the oligosaccharide galactose alpha 1, 3, galactose (on the cetuximab Fab heavy chain)

247
Q

effect of HIV-1 infection on TRECs

A

decreased TREC levels
successful treatment with HAART leads to increased levels

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