Review Book Flashcards

Question

Il-18 actions

Answer 1

- IFN-gamma production by T and NK cells - inhibits IgE synthesis

Answer 2

Alarmin (w/ TSLP, Il-25) T cells - Th2 development ILCs - activate group 2 ILCs

Answer 3

With SMAD3 in nucleus - inhibits transcription pro-inflammatory mediators

Answer 4

Binds IL-1R (IL-1Ra) to prevent Il-1 pro-inflammation in autoinflammatory syndromes

Answer 5

Pyogenic infections, esp strep pneumonia

Answer 6

Activates monocytes to release il-6, TNF-alpha, ROS promotes Th2

Answer 7

From Th-17 cells Epithelial cells increase defensins and barrier function Induces MHC class I antigen expression

Answer 8

- Stim ifn-gamma production by T and NK cells - promotes Th1 cells (that produce ifn-gamma) - increases production il-18 receptor (il-18 aka ifn-gamma inducing factor)

Answer 9

- important in protection klebsiella PNA - promotes differentiation/maintenance Th17 cells (that produce il-17)

Answer 10

- Promotes Th1 - promotes ifn-gamma production by t cells

Answer 11

- inhibit viral replication - increase class I MHC expression - stimulate Th1 cells - promote sequestration lymphocytes in LNs

Answer 12

Survival of T cells (esp cd4)

Answer 13

Class switching and Ig production by B Cells

Answer 14

- required for survival/function Tregs - proliferation of NK cells - induces Bcl-2 expression

Answer 15

- Promotes Th1 differentiation - Inhibits Th2 differentiation - Inhibits class switch IgE, IgG4 - increases MHC on APCs - promotes macrophage inflammation and inhibits IgE-dep eosinophil rich reactions

Answer 16

Promotes Th1/dampens Th2

Answer 17

- increases th17 - increases chemokines/cytokines - increases gm-csf and g-csf

Answer 18

Alarmin Increases il4, 5, 13

Answer 19

- th2 differentiation - class switch to IgE and IgG4 - it’s receptor, il-4R signals through Jak-stat (stat6)

Answer 20

- activates eos - B cell proliferation and IgA production

Answer 21

- fibrosis as tissue repair of chronic inflammation - mucus by lung epithelial cells - IgE and IgG4 class switching

Answer 22

- pro-inflammatory - stimulates acutely phase protein synthesis in liver - production neutrophils in BM - T cells to produce il-17 - signaling involves jak1/stat3

Answer 23

- survival of memory CD8 T cells, NK cells, NKT cells

Answer 24

- anti-inflammatory cytokine - inhibits proliferation/function T cells - inhibits macrophage activation - induces apoptosis - regulates tissue repair and activates fibrosis - uses Smad receptors

Answer 25

Promotes granulocytes, monocytes, macrophage activation

Answer 26

Promotes monocytes maturation

Answer 27

Granulocyte maturation

Answer 28

Th2 development Activates group 2 ILCs

Answer 29

- promotes mast cell development BM - basophil-differentiating cytokine - eos development

Answer 30

- mast cell growth factor - sustains viability T cells in thymus

Answer 31

Overproduction Il-6 LN hyperplasia, Fever, microcytic anemia, LAD, hypoalbuminemia, high CRP

Answer 32

Hot T-Bone stEAk Il-1 - fever Il-2 - stimulates T cells Il-3 stimulates bone marrow Il-4 stimulates IgE Il-5 stimulates IgA

Answer 33

GammaC, part of IL-2R. Important for il-2r, il-4r, il-7r, il-9r, il-15r, -il-21r

Answer 34

Il-2Ralpha Il-2R beta GammaC Engages jak3-stat5

Answer 35

Epithelial cells, endothelial cells, leukocytes, fibroblasts

Answer 36

CCL2, CCL5, ccl7, ccl11 Cxcl8

Answer 37

Warts Hypogamm Infections Myelokathexis (retention neutrophils in BM) Assoc w/ defects in CXCR4

Answer 38

Recruits eos, monos, basos Lymphocytes for ALLERGY

Answer 39

Recruits Neutrophil Lymphocytes for INFLAMMATION

Answer 40

Rolling of leukocytes and binding carbs

Answer 41

Aka CS62**P** On **P**latelets, ligand **P**GSL (and sialyl-lewis), binds **P**mns, T cells, monocytes

Answer 42

Aka CD62**E** and ELAM On Endothelium Binds ESL-1, cd15, pgsl, sialyl lewis Homes T cells to sites inflammation

Answer 43

Aka **L**AM-1, CD62**L** On Lymphocytes Leukocytes Ligands gLycsm-1, madcam-1, cd34, Sialyk-lewis Homes to LN HEV (via Glycam) and PMN rolling (via madcam)

Answer 44

PMNs can’t express carb ligands for E and P selectin

Answer 45

Gut homing Binds madcam

Answer 46

CCR5 if homozygous mutation - no HIV infection If heterozygous - slow infection progression CXCR4 CCL3L1 - if low level, higher HIV acquisition and worse disease

Answer 47

CXC3CL1 CX3CR1 - v249I mutation —> increased risk acs - T280M —> protects against plaque

Answer 48

GIIbIIIa defect leads to plt defect

Answer 49

C3bBb Stabilized by properidin

Answer 50

C3 tick-over to C3b in microbial surfaces

Answer 51

Properidin Stabilizes factor B

Answer 52

Antigen-Ab complex binds C1 (Binds CH2 domain IgG or CH3 of IgM)

Answer 53

IgM > IgG3 > IgG1 > IgG2 Cannot bind IgG4

Answer 54

Classical pathway activation

Answer 55

MBL binds mannose on microbial polysaccharides and MBL-assoc protease 1 (MASP-1, and MASP-2)

Answer 56

MASp-2 cleaves c4 and c2

Answer 57

C4bC2a (Same as classical)

Answer 58

C3bBbC3b in AP C4b2aC3b in CP and LP

Answer 59

C5b-c8 polyC9

Answer 60

S protein and CD59

Answer 61

C5a C4a C3a Induce inflammation and vascular permeability

Answer 62

Most potent mediator basophil and citaneois mast cell degranulation (>c3a>c4a) Chemoattractant C3a chemotactic for eos only

Answer 63

Binds c3d and is a 2nd signal for B cell activation Decreases antigen any needed for B cell activation. Hemos trap ag-ab complexes in germinal centers Receptor for EBV

Answer 64

Binds c3b, c4b Regulates c3 convertase dissociation Phagocytosis of c3b and c4b coated particles Clears immune complexes (cr1 on RBCs) HIV uses to infect monocytes

Answer 65

Binds c3d Keeps ag-an complexes in terminal centers for B cell activation EBV uses to facilitate cell entry

Answer 66

Binds iC3b, Icam-1 Phagocytosis and leukocyte adhesion to endothelial cells LAD 1 Mediates phagocytosis M tuberculosis

Answer 67

Bjnds iC3b Phagocytosis Lad type 1

Answer 68

Cofactor to factor I, inactivated C3b and C4b Assoc w/ aHUS

Answer 69

Binds c3b c4b Prevents Bb binding c3b Prevents 2b binding c4b

Answer 70

Prevents c9 binding C5b-8

Answer 71

Autoantibodies that stabilize c3bBb Consumption of c3 Assoc w/ c3 glomeruloneohropathy and partial lipodystrophy - subendothelial deposits Ig and C3 Lipodystrophy - adipose source of factor D, completes formation c3bBb (Lipodystrophy affects waist up, where more adipocytes) Clue - low c3/nml c4 (alternative pathway)

Answer 72

Auto ab to c1q In hypocomplementemic urticarial vasculitis (huvs) Immune complex deposition skin, joints, kidneys, lungs Tx = hydroxychloroquine, dapsone steroids

Answer 73

Encapsulated orgs (strep pneumonia!) Lupus **Accelerated atherosclerosis** (Early mi second cause death)

Answer 74

Low C1q, c1q, c1q, c2, c4

Answer 75

Low factor D, factor b, properidin

Answer 76

Low c3, c5-c9 If c3 absent —> low factor H or I

Answer 77

C1q >>> Also see low c1/s, c4, c2

Answer 78

C9: 1/1000 Japanese C2: 1/10,000 caucasian

Answer 79

C1 inhibitor Therefore inhibits kallikrein production and bradykinin

Answer 80

C1 inhibitor, therefore decrease kallikrein and bradykinin

Answer 81

C1 inhibitor, therefore decrease kallikrein and bradykinin

Answer 82

Cell antigens Hemolytic anemia Good pastures

Answer 83

Sle GN Serum sickness Arthur’s rxn Immune complexes

Answer 84

IgG and IgM + complement/phagocytes

Answer 85

1- opsonization (IgG) and phagocytosis (FcepsilonRI) (eg hemolytic anemia) 2 - complement activation (eg goodpasture) - IgG/M activate classical complement Anti-basement membrane 3 - abnormal response w/o injury (MG, Graves)

Answer 86

Local vasculitis from immune complexes deposition (type III hypersens) Local edema, neutrophil infiltration, hemorrhage, necrosis Peak intensity 4-10hrs

Answer 87

Type iii hypersens Rash, joint swelling, LAD, fever 7-12d after trigger start Kidneys, liver, hemolysis Re-exposure, sx onset 12-36 hrs

Answer 88

Immune complex deposition - vasculitis - sle - GN - serum sickness

Answer 89

Iva - cd4 th1 (ifn-gamma), TNF a, il2); macrophages, NK cells — contact dermatitis, TB IVb - cd4 Th2 — eos, B cells — DRESS, morbillifirm drug rash IVc — cd8, perforin, granzyme — psoriasis, drug induced Hep B IVd — T cells — neutrophils — contact derm, agep

Answer 90

Cd56bright - 10%, cytokine producing Cd56dim - 80%, activated

Answer 91

Cathelicidins Defensins Produced by keratinocytes

Answer 92

Il-1a, Il-12, -23, APRIL, BAFF

Answer 93

TNF-alpha, Il-1, Il-6, Il-8, il-12

Answer 94

Il-1, il-6, il-23, TNF

Answer 95

Major basic protein Eosinophil cationic protein

Answer 96

Ifn gamma, il6, TNF alpha, tgf beta, PAF, tryotase

Answer 97

Il-4, il-6, BAFF, tslp

Answer 98

TNF alpha, il-10, ifn-gamma

Answer 99

Damage associated molecular patterns Endogenous molecules released by injured cells (NOT from apoptosis)

Answer 100

In APCs Recognize DAMPs and PAMPs

Answer 101

Il-1 and Il-18

Answer 102

PAMPs Activate innate immunity

Answer 103

TLR 2, 6, 7

Answer 104

GNR (lipopolysaccharides) , GP bacteria (peptidoglycans), rsv, acute phase proteins (Hsp 70, 90)

Answer 105

MyD88 Activates NFkB and AP-1

Answer 106

TRIF, TRAF Activates IRF3 and IRF7, induce type I IFN

Answer 107

Live in a NEST Nine Eight Seven Three Play big role in viral recognition

Answer 108

MyD88, IRAK4

Answer 109

Canonical incisors, ectodermal dysplasia, recurrent bacterial infections IKKgamma

Answer 110

CARD9 - (cascade activation and recruitment domain), part of inflammasome

Answer 111

NLRP3 - inflammasome

Answer 112

Via AIRE in MTEC

Answer 113

Receptor editing with help RAG1/2 and light chain rearrangement

Answer 114

If bind strongly enough to peptides bound by MHC on **cortical** Thu if epithelial cells

Answer 115

AIRE induces expression self antígena Occurs in **medulla** (Negative selection in cortex)

Answer 116

When leave cortex and go to medulla

Answer 117

Their CCR7 (on mature naive T cells) binds ccl19/21 from dendritic cells/stromal cells

Answer 118

Via S1P gradient

Answer 119

Lipids and heat shock proteins

Answer 120

Gut tissue

Answer 121

Recognize glycolipids Produce ifn gamma, IL-4, -3, -17

Answer 122

Induced by il12, ifn gamma Produce ifn gamma Txn factor Tbet, stat4 For intracellular defense

Answer 123

Induced by il4, Produce il4, 5, 13 Txn factors gata3, stat6

Answer 124

Induced by il-6 Produces il-17, Txn factor RORgammaT, stat3 Extracellular defense Psoriasis

Answer 125

Induced by tgf beta Produces il9

Answer 126

Induced by/produces il-21 Txn factor bcl-6 Helps B cells make antibodies

Answer 127

Induced by tgf-beta Produces tgf-beta, Il-10 Txn foxp3, stat5

Answer 128

Natural T regs in thymus - mediate self tolerance Induced Treg (Tr1) in periphery - suppress effector cells iTreg (Th3) - in periphery, help IgA production in gut

Answer 129

Fetal liver @7eks

Answer 130

Express cxcl5 that binds cxcl13 expressed by follicular dendritic cells i

Answer 131

Activated by antigen presenting dendritic cells, increase ccr7 expression

Answer 132

Cd40 (b cell) with cd40L (follicular helper T cell) Also need AID and UNG

Answer 133

Deficiencies in cd40, cd40L, AID, UNG Ig gets stuck in IgM form (doesn’t have tools for class switching CD40L on T cells affected

Answer 134

T cell dep (need cd40-cd40L) In germinal center Point mutations in V regions

Answer 135

BLYSS, APRIL, BAFF receptors on B cells are BR3, TACI, BCMA

Answer 136

Adds nucleotides randomly during vdj recombination At hairpins Responsible for heterogeneity

Answer 137

In peritoneal cavity and fetus Independent of T cells Doesn’t need antigen - constitutive secrete Ig

Answer 138

Ig Heavy chains and TCR beta

Answer 139

In vdj recombination, a gene segment flanked by 12 nt spacer can only be joined by gene segment flanked by 23 nt spacer

Answer 140

Recombination signal sequences 12 or 23nt long

Answer 141

Align 2 RSSs Cleave dsDNA between coding segment and RSS

Answer 142

Opens hairpins at coding ends (with DNA-pk Vdj rearrangement

Answer 143

Ligate coding and rss joints

Answer 144

Intrinsic - Bim binds Bax and bak to activate caspase 9 Extrinsic - FasL binds Fas, activates caspase 8

Answer 145

Ifn alpha, beta, il12

Answer 146

Ilc2 recruited to lung and skin after allergen, il5 dep eos recruitment, il13 smooth m contraction, mhc II th2 enhancement —> allergy and fibrosis ILC3s -> increased in BAL of asthmatics

Answer 147

Not on eosinophils

Answer 148

Stem cell factor - mast cells Il-3 to basos Il-5 to eos

Answer 149

Ckit+ (cd117), cd34+ stem cells Develop after arrival in tissue

Answer 150

<15 mins - preformed mediators (tryptase, histamine, heparin, chymase) 10-30mins - lipid mediators (PGD2, LTC4, LTB, paf) Hrs-days - cytokines and chemokines

Answer 151

Mast cell activation Alpha, beta, and 2 gamma chains IgE binds via alpha chain

Answer 152

4-6wks after peak pollen season

Answer 153

Bullous pemphigoid WAS Omenn IPEX Atypical incomplete DiGeorge Netherton

Answer 154

Susceptible mast cells activated by this receptor with certain medications - cipro, atracurium, vanco, narcotics (except fentanyl)

Answer 155

CD123 (il-3 receptor) Il-3 important for expansion/differentiation

Answer 156

Mbp, eos cationic protein, eos peroxidase, eos-derived neurotoxin

Answer 157

Siglec-8, CCR3, EMRI

Answer 158

RANTES (ccl-5) and eotaxin (eot-1/ccl11 and eotaxin-2/ccl24)

Answer 159

>500 cells/ul

Answer 160

Hyper-IgE Omenn IPEX Zap70 def Netherton synd

Answer 161

US - drug reactions WW - parasites (NOT protozoa - giardia, amoeba)

Answer 162

Fever, steroids, most viral/bacterial infxn (HIV raises eos)

Answer 163

eos >1500 x2 at least 4 wks apart AND end-organ damage OR tissue involvement

Answer 164

NIAAA Neoplasm Infection Allergic Autoimmune Adrenal insufficiency

Answer 165

CD14 (part of TLR4 and a LPS receptor)

Answer 166

CD14, CD16, CD11b/CD18, CD33

Answer 167

Classical M1: Induced by LPS or Th1 cytokines (IFN-gamma), phagocytose and present antigen, secrete Th1 cytokines (TNFa, IL6, IL1beta _> T cell activation Alternative M2: induced by steroids and Th2 cytokines, reponse to parasitic infections

Answer 168

uncontrolled activation macrophages with increased cytokines. Predisposing ID and low NK cells + immune activation --> damage High fever, HSM, high ferritin and TG, low fibrinogen, hepatitis

Answer 169

>/5 of the following: fever splenomegaly cytopenia in 2+ lines, hyperTG hypofibrinogenemia hemophagocytosis low/absent NK cels ferritin <500 soluble CD25 >2400 (activated T cells)

Answer 170

Marker CD123, secrete IFNa and are in viral infxn (unique dendritic cell subset, as no CD11c expression)

Answer 171

dendritic cell subset, marker CD11c, Birbeck granules Prime CD8 T cells

Answer 172

Immature in the tissue, where uptake antigen Mature in lymphoid organ

Answer 173

class I MHC like molecules that present nonpeptide molecules to T cells

Answer 174

Confirm LC identity on bx with CD1a and CD207

Answer 175

IL-8 (CXCL8), LTB4 Also with sialyl-lewis, e-/p-selectings, LFA1/ICAL1

Answer 176

Basos - basic blue Eos acidic, bright red Neutrophils neutral pink

Answer 177

affects CD11b in granulocyte elastase in neutrophils

Answer 178

primary - MPO, defensins, cathelicidin/cathepsin Secondary - lysozyme, collagenase, elastase, lactoferrin, Cd11b

Answer 179

Enlarged primary granules

Answer 180

against plt antigen PF4

Answer 181

eczema, infections, thrombocytopenia defective WASp gene

Answer 182

Airway smooth muscle unregulates glucocorticoid receptor (GR)-beta, a negative inhibitor

Answer 183

Auto reactive/nonfunctional B cells removed

Answer 184

Subcapsular zone - lymphocyte progenitors Cortex - lymphocytes in division, expression, TCR section - positive selection with TCR re-arrangement compatible with MHC - negative selection begins in cortex - auto reactive/nonfunctional T cells/TCRs apoptose Medulla - where final stages selection/maturation - most of negative selection - has Hassles corpuscles

Answer 185

T cell precursors that go to cryopatches instead of thymus - lymphoid aggregates under intestinal epithelium to form specialized T cell populations

Answer 186

Primary - site resting B cells Secondary - germinal center of B cell proliferation Ratio primary to secondary LNs defines LN activity

Answer 187

White pulp

Answer 188

B cells T cells undergo positive and negative

Answer 189

Nasal valve, which is controlled by swelling inferior turbinate

Answer 190

Maxillary/ethmoid present at birth Sphenoid develop at 3yo Frontal at 6yo

Answer 191

Frontal, maxillary, ant ethmoid drain into middle meatus Posterior ethmoid, sphenoid drain into sphenoethmoidal recess above superior turbinate

Answer 192

Asthma: smooth m enlarged in LARGE airways, have increased cd4 and eos, increased il-5/4, Copd: enlarged smooth m in SMALL airways, increased acidic glycoproteins, cd8, NO increased il5

Answer 193

CCR4/ccl17 and ccr10/ccl27

Answer 194

IgG ab to desmoglein 1/3 Blisters flacid - erupt IgG and C3 staining on suprabasal layers

Answer 195

Tense Prominent pruritus IgG BP180, bp230 Linear basement membrane IgG and C3

Answer 196

Small bullae elbows/knees Pruritic Assoc w celiac IgA to epidermal transglutaminase

Answer 197

Increase neutrophils, elevated methemoglobin Friable mucosal with villous atrophy

Answer 198

Il-5, eotaxin -3

Answer 199

Gliadon sensitivity Anti-tTG IgA (tissue transglutaminase) Total IgA (if IgA deficient, false neg anti-ttg iga

Answer 200

Cross-sectional - prevalence Case-control - odds ratio Cohort (subjects with common characteristic - who develops dz) - relative risk Rct - absolute risk reduction, suggest causality

Answer 201

Falsely reject null hypothesis

Answer 202

Falsely accept null (commonly from underpowered studies)

Answer 203

Axd/ b x c (True pos x true neg) / (false pos x false neg)

Answer 204

Incidence in exposed / inc unexposed Ppv / c/ c+d

Answer 205

Number events in treated or control group / number people in the group

Answer 206

Competence, disclosure, understanding, voluntariness, consent

Answer 207

FHx atopy IgE > 100 before age 6 Increased SES Maternal smoking

Answer 208

Histamine - itch, sneeze, rhinorrhea PGD2 and leukotrienes - nasal congestion Kinins- nasal congestion/blockage

Answer 209

Chronic inflammation leads to lower dose of allergen to elicit sx (AR)

Answer 210

Perennial - intranasal steroids Seasonal - inteanasal antihistamines

Answer 211

Jntranasal ipratropium

Answer 212

No allergic rhinitis w eosinophils Eos>20% on nasal smear Middle aged men Yr-rnd sx Abnml PG metabolism Tx - topical steroids

Answer 213

Stimulation sensory c-fibers Parasympathetic reflex, Chokinergic muscarinic receptors Tx - ipratropium

Answer 214

Noninflammatory Progressive atrophy in middle aged men in warm countries (Saudi Arabia) Klebsiella colonization Foul smell nasal vault

Answer 215

Causes: multiple ess empty nose syndrome granulomatous disease

Answer 216

Intranasal corticosteroids are ineffective

Answer 217

Eosinophilic process (mbp, Il-5, etc) Asymmetric? Peanut butter mucus (brown and viscous) Ct - central hypoattenuation Bony demineralization MRI - hyper intense areas in mucin (heavy metals iron mgn within mucin) - heterogenous opaciticTikn)

Answer 218

All 5: 1. Hypersens to fungi confirmed - asp., alternaría, curvularia, penicillium, fusarium, bipolaris 2. Nasal polyposis 3. Ct findings 4. Noninvasive fungus in mucin or in cx 5. Eosinophil mucin w/o invasión into sinus tissue

Answer 219

Sight threatening chronic conjunctiva inflammation Young males in warm dry climates Severe photophobia, intense itching Worse with antihistamines (increased dryness) +verna;/spring sx

Answer 220

Sight threatening Commonly with a topic dermatitis Keratoconus Anterior subcapsular cataracts Gritty photophobia

Answer 221

From contacts mechanical trauma - protein buildup on lenses - allergic reaction Get redness, itching after contact removal, morning mucus discharge Tx - remove contacts and use artificial tears

Answer 222

Hordoleum (stye) = pain Chalazion = not painful

Answer 223

50% ocular sx Conjunctivitis, corneal ulcers, pain

Answer 224

Be concerned about cutaneous T cell lymphoma (ctcl)

Answer 225

Il-31 Induced by staph a

Answer 226

Cutaneous t-cell attracting chemokine (CTACK and ccl27) Thymus and activation regulated Chemokine (TARC)

Answer 227

Acute - IL-4, -13 — th2, also il25 and -33 Chronic - Il-5, Il-12, ifn-g Il-17, -22 “Education is **chronic** starting at **5** through **G**rade **12** — th1

Answer 228

Cd3, cd4, cd45ro, Activated eos Increased RANTES and TSLP decreased antimicrobial peptides

Answer 229

FcepsilonR1 lack classic beta chain Have Birbeck granules

Answer 230

Composed of serum, proteins, inflammatory cells, mucus and cellular debris

Answer 231

>/4 wheeze episodes past yr PLUS 1 major - parent w/ asthma - AD - aeroallergens OR 2 minor - food sensitivity - peripheral eos (>/4%) - wheeze not related to infection

Answer 232

Cat, DM, cockroach, Alternaria

Answer 233

Increase in fev1 and/or fvc > 12%

Answer 234

PC <4 mg/ml diagnostic 4-16 suggestive

Answer 235

Increase - viral uri - allergic rhinitis - nitrate-rich foods Decrease foods - caffeine - smoking - CF - alcohol - spirometry Eos stimulate epithelial cells to produce NO

Answer 236

Sx onset after 10mins activity Resolves 15-30mins after Fev1 decrease at least 15% after exercise challenge

Answer 237

Ovomucoid, gal d 1

Answer 238

Ara h 8 (bet v 1 homologue)

Answer 239

Ara h 12, 13

Answer 240

Gly m 5/6 Gly m 3/4 = bet v 1 homologues

Answer 241

Food allergies in 30-50% BACK banana, avocado, chestnut, kiwi

Answer 242

Stone, fruits, apple, almond, hazelnut, so I’ve been some peanuts, celery, carrots, fennel, coriander

Answer 243

Melons, zucchini, banana

Answer 244

Celery, carrot, parsley, mustard, cauliflower, cabbage, garlic, onion

Answer 245

Cows milk, soy, wheat, egg — 85% by age 5 Peanut - 20% Tree nuts - 9%

Answer 246

Milk, soy, rice, oat 50% outgrow after 2 yrs

Answer 247

Increased neutrophils and Il-10, TNF-g Decreased eos

Answer 248

1 major + 3 minor Major: - vomiting 1-4 hrs after w/o IgE sx Minor - 2nd episode when eaten again - repetitive vomiting 1-4 hrs after eating different food - lethargy, pallor, ed visit requiring ivf - diarrhea, hypotn, hypothermia

Answer 249

HLA-DQ2 HKA-DQ8

Answer 250

Recurrent PNA, pulm infiltrates, hemosiderosis,Fe def anemia, FTt Peripheral Eosinophilia Lung bx Milk precipitans Elimination diet

Answer 251

Children - food and venom Adults - meds and venom

Answer 252

Receptor on mast cells. Can be directly activated by small molecular weight drugs - NBMAs, morphine, fluiriquinolone, vanco -> mast cell degranulation and non-IgE anaphylaxis

Answer 253

Il-6 and -10, Tnf receptor 1 Mast cell tryptase Histamine C3a

Answer 254

Severe initial reaction, Multiple doses epi Wide pulse P Delay in epi administration Unknown trigger

Answer 255

TCAs - prevent catecholamine re-uptake MAOIs - prevent epi degradation

Answer 256

Total/mature > 20 —-> mastocytosis Total/mature < 10 —-> other

Answer 257

Peaks 60-90’ Falls after 5 hrs Check 15’-3hrs after reaction Can confirm but not r/o anaphylaxis

Answer 258

Rises w/in 5 mins, falls in 30-60’

Answer 259

H1 - coronary artery vasoconstriction, bronchial construction, GI smooth m contraction H2 - coronary vasodilation, increased cardiac inotropy/chronotropy (rate, force) H1 and H2 - systemic vasodilation, flushing HA

Answer 260

Epinephrine Glucagon and IVF if not responding - glucagon has inotropic and chronotropic effects not mediated by beta receptors

Answer 261

Antibiotics Europe - nmba

Answer 262

Hev b 1-13 Hev b 2 - bell pepper, olive Hev b 5 - kiwi, potato, beet Hev b 6 - avocado, banana, chestnut, sweet pepper Hev b 7 - tomato potato Hev b 14 - potato 5-7 most common healthcare workers

Answer 263

Celery Wheat (omega 5 gliadin), Avoid exercise 4-6hrs of ingesting food

Answer 264

Very aggressive Insects around garbage cans/food —> yellow jackets Common in autumn Most common cause of stings Hornets sting when vibration sensed

Answer 265

Dol m 5 - antigen 5

Answer 266

Paper wasp Narrow waist, dangling legs Pol a

Answer 267

No urticaria/angioedema Only 50% chance if reacted to prior sting will react to subsequent sting

Answer 268

50% have rxn in buildup phase 5-15% with systemic rxns <5% require epi

Answer 269

Reduces risk subsequent rxn to <5%

Answer 270

Anaphylaxis Can consider in large local reactions f impaired life, frequent exposures Not indicated: Large local rxn (4-10% risk systemic rxn) Urticaria/angioedema

Answer 271

Adults - 100ug (can be increased to 200) Children - 59 if 300ug for mixed vespid Fire ants - whole body extracts

Answer 272

Large local reactions to mosquitos + fever

Answer 273

At least six weeks

Answer 274

EIA will not react with passive heating

Answer 275

Cold – cyproheptadine Heat – hydroxyzine

Answer 276

Associate with a fever and or arthritis Typically painful and leave markings

Answer 277

Major (have both): - urticaria > 6 **months** - hypocomplementemia AND Minor (at least 2) - venulitis of dermis - arthralgia/arthritis - mild glomerulonephritis - uveitis/episcleritis - recurrent abdominal pain - positive c1q precipitan with assoc suppressed c1q (Anti-c1q ab)

Answer 278

OA - latency period, Reactive - subset OA, occurs after single high level exposure, sx w/in 24 hrs. Eg gases

Answer 279

Carpenters/woodworkers - plicatic acid (activates complement) roofers/insulators/painters - isocyanates Nail salon/dental hygienists/plastics - acrylates, epoxy Manufacturers of paint, plastic, epoxy — anhydrides

Answer 280

Measure pft at work and at home Fev1< 20% after exposure suggestive Methacholine challenge (neg test excludes OA) Gold standard - inhalation challenge

Answer 281

Acute - hrs after exposure, - NONproductive cough, fever, chills malaise, - diffuse fine crackles - cr nml Subacute/intermittent - wks-mo - PRODUCTIVE cough, dyspnea, fatigue, wt loss - diffuse crackles - ct micronodules, air trapping, mild fibrosis Chronic - mo-yrs - cough, dyspnea, wt loss - digital clubbing - ground glass, emphysema, honeycombing, fibrosis

Answer 282

Thermophilic actinomycetes In moldy hay, grain, compost

Answer 283

Smoking decreases risk of HP

Answer 284

CD8 predominance (cd4:8 < 1) Lymphocytosis>20% Increased eos, neutrophils, mast cells, IgA/G/M Noncaseating granulomas Decreased fev1, fvc, dlco

Answer 285

Noninfectious Febrile illness after exposure to dust with toxin-producing fungi (grain, hay, textiles) 30-50x more common than HP Complete recovery

Answer 286

Need sensitivity to A fumigatus (Asp f 1) Elevated total IgE > 1000 When not responsive to abx

Answer 287

Doubling of baseline IgE

Answer 288

FEV1/FVC < 70% after bronchodilation

Answer 289

Gold 0 (at risk): sx but nml spiro Gold 1 (mild): fev1>/80% pred Gold 2 (moderate): 50-80% pred fev1 Gold 3 (severe): 30-50% pred fev1 Gold 4 (v severe): fev1<30% or <50% + chronic resp failure

Answer 290

Neutrophils, macrophages, cd8 T cells Neutrophils/macrophages destroy alveolar attachments that help keep airway open and elastic recoil

Answer 291

Copd type, assoc w alpha1 antitrypsin deficiency - think in young non-smokers with copd

Answer 292

Smoking cessation

Answer 293

Smoking cessation, supplemental oxygen 24 hours per day and lung volume reduction surgery

Answer 294

Copd - decreased Fev1/fvc - increases tlc - increased frc - clubbing uncommon - wheezing ILD - nml/increased fev1/fvc - decreased tlc - decreased frc - clubbing common - Velcro rales

Answer 295

Chemo - busulfan, bleomycin Abx - nitrofurantoin, sulfa Antiarrhythmics - amiidarone, procainamide Immunosuppressives - mtx, cyclophosphamide, azathioprine

Answer 296

Increase IgG4 plasma cells and lesions in lung, liver, pancreas IgG4>135 Fibrosis

Answer 297

Lymphocytes and spongiosis in upper dermis

Answer 298

Corticosteroids, nickel, neomycin, gold

Answer 299

Acd more pruritic, reactions increase between 1st and 2nd readings Icd - more prevalent, more stinging, reaction almost immediate and decrease between 1st and second readings

Answer 300

IL1 alpha, IL1 Beta, TNF alpha, GM- CSF, IL8

Answer 301

Toxicodendron dermatitis To urushiol (an oleoresin) Cross reactivity to mango peels

Answer 302

Most common cause Cd on hairdressers and henna tattoo

Answer 303

Group A - hydrocortisone type Group B - triamcinolone type Group C - betamethasone tupe Group D - hydrocortisone-17-butyrate type

Answer 304

Epoxy - sensitizing only when not cured Colophony - from pine trees, in cosmetics, topical meds, industrial products Ethylenediamine dihydrochloride - in topical creams, aminophylline Paraphenylenediamine (ppd) - from benzene, in henna

Answer 305

Type I - decreased c4, low/absent c1 inh with decreased function; 85% cases Type ii - deceased c4 and increased/nml c1 inh that’s nonfunctional; 15% cases

Answer 306

Ab to c1-inh Assoc w nonhodgkins lymphoma Low c4 Low c1q C1 inhibitor low/normal C1 inh function decreased

Answer 307

Il2rg - gamma c def, x-linked, 50-60% of scids, cd132- Jak3 def - AR, cd132+

Answer 308

IL7R - AR, 5% scids, no thymus Cd3E - ar, no game m/delta T Cd45 (ptprc gene) - ar, normal gamma/delta T cells, no thymus Coronin 1a def - coro1a - **normal** thymus

Answer 309

Ada def - ar, 20% scids, rachitic rosary, abnormal iliac bones, deafness Activated rac2 def - defective hematopoietic energy metabolism Reticular dysgenesis (ak2) - severe neutropenia, sensorineural deafness

Answer 310

Xlf def (nhej1) - microcephaly, facial dysmorphism, radiation sensitivity Artemis - DCLRE1C (Artemis), no microcephaly, AR

Answer 311

RAG1/2 mutation, can't rearrange Ig genes Decreased B cells Erythroderma Increased lymphoid tissues HSM Eosinophilia, increased IgE Neonatal red rash

Answer 312

Stat3 GOF AD triad: 1. recurrent skin/lung infections (a aureus, a pneumonia, H flu, C albicans) 2. Severe eczema 3. IgE >2000 Retained teeth, scoliosis Absent Th17

Answer 313

DOCK8 Severe eczema Cutaneous viral and bacterial infxns (hpv, hsv, vzv, molluscum) Decreased IgM, NK cells, TRECs Increased eos, B cells, Tregs

Answer 314

Tyk2 - similar to type II with disseminated BCG lymphadenitis, salmonellosis (Tyk2 in il12 signaling to produce ifn-g)

Answer 315

X-linked recessive Biliary tract and liver dz No germinal centers Cryptosporidium Neutropenia IgM normal or high, others decreased Cd40 deficiency is AR

Answer 316

X-linked recessive, Triad: immune def (bacterial/viral), eczema, small plts EBV lymphoma

Answer 317

WIPF WAS protein absent Thrombocytopenia

Answer 318

STAT3 LOF AD cold skin abscesses, pneumatoceles Scoliosis Hard to differentiate from AD STAT3 GOF

Answer 319

SPINK5 Icchthyosis Bamboo hair

Answer 320

Btk gene Sinopulm infxns, GI (enteroviruses) No germinal centers - BTK req'd for survival All Ig decreased Nml T cells Nml pro-B cells - Boys with BTK will have monocytes, but no BTK expression in them

Answer 321

Several forms, occur in pre-B cell stage when heavy chain recombines

Answer 322

High IgM, low IgG/IgA

Answer 323

Adult onset hypogamm, thymoma Decreased B, T, eos, neutros, panhypogamm Thymenctomy does not help immunodeficiency

Answer 324

Warts Hypogamm Infections Myelokathexis (neutros retained in BM)

Answer 325

Tlr3 Unc93b19 HSE with primary hsv infection Usu 3-6yo Decreased ifn-a and -b

Answer 326

Complete absence = AR + affects extra cellular domain AD = heterozygous, intracellulat domain

Answer 327

IFNgR1/2 deficiency

Answer 328

Infection, atherosclerosis and MI (C1q plays role in cholesterol clearance)

Answer 329

Complement activated on epithelium Treated with c5 blockers (eculizumab and ravilozumab) Microangiopathic hemolytic anemia, thrombocytopenia, kidney injury Disseminated neisserial ingections

Answer 330

CCR5 - monocytes/macrophages, acute infection CXCR4 - T cells, advanced hiv dz

Answer 331

Requires 2/3 bands present for + result: Anti-p24, anti-gp41, anti-go160/gp

Answer 332

At birth, 2-3wks, 1-2mo, 4-6mo

Answer 333

High sensitivity/mod specificity Requires confirmatory False + from multiple pregnancies, autoimmune dz, multiple blood Transfusion False -: window period

Answer 334

Ritonavir, inhibits cyp3a4 and cyp2dg, Causing increased levels ICS (fluticasone) to systemic levels Cause Cushing syndrome

Answer 335

Life threatening allergic reaction

Answer 336

Decrease in clinical status 4-8wks after ART initiation Reaction of reconstituting immune system and it’s reaction to opportunistic infection with cytokine storm

Answer 337

IL-5, IL-13, eotaxin-3 (ccl26) Eotaxin3 most overexpressed gene in EoE, receptor is CCR3. Chemotactic for eos

Answer 338

Milk, wheat, egg, soy

Answer 339

May reverse esophageal dysmotility/dilation

Answer 340

Myeloproliferative variant HES Mainly men Higher risk cardiac cx Increased serum B12, tryptase Sensitive to imatinib

Answer 341

Prominent skin findings (eczema, urticaria) May progress to T cell lymphoma - hypergamm Elevated TARC/CCL17 Aberrant il5 producing T cells Cd3-cd4+ T cells

Answer 342

1 major + 1 minor or 3 minor: Major: bx w dense infiltrates >15 mast cells Minor: - bx >25% mast cells with spindle shape - c-kit mutation (kit D816V) - expression CD2 and/or CD117+ mast cells - tryptase >20

Answer 343

3 criteria needed: A- typical s/s systemic mast cell activation (eg anaphylaxis) B - involvement MC with biochemical studies (elevated baseline typtase, urinate metabolites, etc) C- symptomatic response to mast cell stabilizers

Answer 344

Lone star tick SE, south central, mid Atlantic Can trigger HLh Smear showing intreukocytic morulae Tx / tetracyclineor chloramphenicol

Answer 345

Mycobacterial disease clearance depends in ifn-g activating T cells Defects in il-12/-23/ifn-g cause msmd

Answer 346

Primary - painless chancre o genitals, skin, mucosa Secondary - rash on palms/soles, condyloma lata (warts) in genitals, malaise, fever Tertiary - cv, neuro

Answer 347

Nontreponemal ab (vdrl, rpr) tests reactivity to antigen followed by more specific treponemal test looking for antibodies (fta-abs, EIA)

Answer 348

Pregnancy Iv drug use Tb, nonsyphillus treponemal, rickettsia, hiv Endocarditis

Answer 349

Any signs in those <6mo B/l involvement in those 6-23mo Rest - shared decision making First line uncomplicated - high dose amox x 10d Other - augmentin

Answer 350

Acute - up to 4wks Subacute - 4-12wks Chronic - >12 wks

Answer 351

Strep pneumonia Moraxella H flu

Answer 352

2 major or 1 major +2 minor Major: Purulenta rhinorrhea Nasal congestion/obstruction Facial congestion/fullness Facial pain/pressure Fever Hyposma/anosmia Minor: Headache Ear ache/pressure/fullness Bad breath Dental oaon Fatigue Cough (post nasal drip)

Answer 353

Augmentin (amox-clavulanate) High dose augmentin for those at higher risk/higher risk areas Alternative - fluoriquinolone/doxy Alternative children - clinda + ceohalosporin

Answer 354

>4 episodes/yr

Answer 355

Produce il-17, -22, and synergies with TNF-a and il-1b to enhance inflammation Induced by il-6, tgf-b Survival increased by il-23 Cytokines recruit neutrophils and enhance local innate response

Answer 356

Calcinosis cutis Raynaud’s Esophageal dysmotility Sclerodactyly Telangectasia Sx of sclerosis/crest synd

Answer 357

Ana, SS-A (Ro), SS-B (La)

Answer 358

Anticentromere Ab

Answer 359

RF, anti-CCP

Answer 360

NSAIDs Steroids Dmards - mtx, sulfasalazine, hydroxychloroquine Jak inhibitors (tofacitinib, baricitinib) TNF-a blockers (infliximab, adalimumab) Anti-Il-6 - tocilizumab Ctla-4-Ig - abatacept - ritux

Answer 361

10% of those with JIA have MAS

Answer 362

Oculocutaneous albinism Neuro defects, infxn (cutaneous, sino-pulm) with GNR, staph, strep Trafficking protein defect, granules in neutrophils fuse make enlarged granules, can’t move granules

Answer 363

Perforin and cd107a

Answer 364

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy Chronic mucocutaneous candidiasis, recurrent thrush, dystrophic nails, endocrineopathies (DM, alopecia…) AIRE mutation Anti-interferon omega Ab very specific for test

Answer 365

Autoimmune lymphoproliferative syndrome - AI cytopenias, LAD, HSM, lymphomas Genes: FAS Dx - high double neg T cell - TCR alpha/beta (CD3+CD4-CD8-), high B12, il-10

Answer 366

Immunodysregulation polyendocrinopathy enteropathy x-linked Severe diarrhea, for allergy, early onset DM, cytopenias, skin lesions foxp3 (in Tregs) AR

Answer 367

Older patients with chronic nonspecific dermatitis or generalized erythroderma Need: Erythroderma >/80% body Clonal TCR rearrangement Sezary count >/1000 or increased cd4 cells with loss cd26 cells

Answer 368

High IgM Assoc w chromosome 6 abnormalities

Answer 369

Giant cell LN hyperplasia Angiofollicular LN hyperplasia HHV-8 implicated in pathogen May have POEMS syndrome (Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin abnormalities)

Answer 370

IgM and complement

Answer 371

Minor blood group antigens (eg Rh) 5-10d after transfusion Increased LDH, indirect bili

Answer 372

Severe resp distress 1-4hrs after Donor Ab directed at recipient neutrophil antigens or HLA determinants

Answer 373

IgG against RBCs Positive DAT

Answer 374

IgM binds to I antigen on RBCs at cold temperatures, activates complement

Answer 375

ITP + AIHA w/o underlying etiology Assoc w PID (eg cvid, alps)

Answer 376

Triad: hemolytic anemia (non immune), thrombocytopenia, renal disease

Answer 377

Adamts13 deficiency FAT RN fever Anemia (microangiopathic hemolytic) Thrombocytopenia Renal failure Neuro changes

Answer 378

TSH receptor Ab Assoc w autoimmune polyglandular syndrome type 2

Answer 379

Ab to TPO, TG, TSH receptor Assoc w APS-2

Answer 380

Most common nephrotic syndrome adults Subepithelial Ig deposits activate complement Thickening glomerular basement membrane

Answer 381

Thickening GBM Assoc w hep c

Answer 382

Crescentic GN with focal necrotizing lesions Can progress days-wks Adults - pauci-immune Kids - immune complex mediated more common

Answer 383

In RPGN or part of Goodlasture’s synd (pulm-renal vasculitic syn) Ab (IgG1 and IgG4) against alpha3 chain of type IV collagen 1/3 perinuclear antineutrophil cytoplasmic ab (pANCA) and cytoplasmic antineutrophil cytoplasmic ab (c-anca)

Answer 384

Target desmogleins-1 and -3 and/or desmosome with IgG and C3 In EPIdermis Life threatening Flaccid bullae

Answer 385

60-80yo SUB-epidermis Tense bullae on urticarial base Target BPs - BP180 and BP230, hemidesmosome Eosinophilic infiltrate and increased IgE

Answer 386

In areas of trauma Target type VII collagen

Answer 387

HLA DQ2 and DQ8 IgA to tissue transglutaminase or endomysium

Answer 388

Type 1: Ss- or dsDNA Smooth muscle Actin Soluble liver or pancreas Ag Type 2: Anti-liver-kidney-microsomes-1 (ALKM-1) Liver-cytosol antigen (ALC-1 or LC1)

Answer 389

Middle aged women HLA A 0201 and HLA DR8 Progressive inflammation of **intra**hepatic ducts —> chronic cholestasis, fibrosis, cirrhosis, failure

Answer 390

Inflammation, fibrosis, bile duct stricture Affects Intra and extrahepatic ducts Men>women Genetics - HLA B8, DR3 Assoc w UC Increased risk cholangiocarcinoma Dx- ercp

Answer 391

Autoinflammatory - innate immune system, no autoantibodies or auto reactive T cells - early age onset - recurrent bouts similar in severity Autoimmune - later onset, have autoantibodies and auto reactive T cells, worse outbreaks over time - activation adaptive immune system

Answer 392

MEFV gene (codes pyrin) - severe mutations - M694V - Tx = daily colchicine which can decrease likelihood amyloidosis Tonsillectomy can be curative

Answer 393

Tumor necrosis factor receptor associated periodic syndromes - AD, in TNFRSF1A gene - febrile episodes can last weeks, periorbital swelling, conjunctivitis, pleuritis, HA - amyloidosis - Tx = Il 1 blockers, anakinra, steroids

Answer 394

Tx steroids, tonsillectomy Fevers last 3-6d, occur q3-8wks

Answer 395

Mutation MVK gene - cholesterol and steroid synthesis pathway Fevers 3-7d, painful LAD, apthous ulcers, and pain, arthritis Flares from stress and vaccinations Tx - steroids, anakinra prn

Answer 396

Mutations in NLRP3 3 diseases: cinca/nomid = most severe Muckle wells - moderate Familial cold induced autoinflammatory syndrome (FCAS) - mildest Fever, rash, arthralgia, amyloidosis Nomid with dysmorphism, dev delay, bony overgrowth, sensorineural hearing loss

Answer 397

Il-1 blockade - anakinra, canakinumab

Answer 398

AD, mutation in CD2 binding protein 1– prolonged inflammasome activation Early life arthritis Then sterile pyoderna gangrenosum, cystic acne, Pyogenic sterile arthritis Tx - il-1 blockade

Answer 399

Uninhibited Il-1 activity -> skin and bone disease skin and bone disease Tx - anakinra (anti-il-1) replace lost protein

Answer 400

Lipin2 gene Chronic recurrent multi focal osteomyelitis Pain, fever, psoriatic appearing rash and acne Tx - nsaids and steroids

Answer 401

Nod2 GOF - involved in nfkb activation Triad: granulomatous poly arthritis Uveitis Pustular skin lesions Later in life joint swelling and contractures

Answer 402

Related to ubiquitous dysregulation Granulomas Oral ulcers Uveitis Immunodeficiency

Answer 403

Interferonopathy - mutations in proteasome system (PSMB8, PSMG2, etc). Proteasomes clear ubq waste proteins Sx - daily fevers, skin plaques, eyelid swelling, ftt, lipodystrophy Autoimmunity - hemolytic anemia, hypothyroid Tx - jak inhibitors interrupt interferón signaling, some success

Answer 404

Mutations cecr1 - gene codes for ada2 Def -> adenosine accumulation, neutrophil activation, increased m1 macrophage polarization and inflammatory cytokine production Fevers, lacunar strokes (before age 5), organomegaly, cytopenias, hypogamm, polyarteritis nodosa Tx - steroids, tnf blockade, cyclophosphamide, Def ada1 -> T-B-NK- SCID

Answer 405

Native proteins that are misfolded and aggregate as insoluble, then accumulate and cause end organ damage

Answer 406

Immunoglobulins that undergo reversible precipitation at low temps

Answer 407

Meltzers triad: Purpura Arthralgia Weakness

Answer 408

Macrophage secrete molecules after activation that promote granuloma formation : ACE calcitriol (1,25dihydroxyvitamine d3) Osteopontin Tnf-a

Answer 409

Self acute limited form sarcoidosis Triad: B/l hilar adenopatía Erythema nodosum Polyarthritis

Answer 410

Triad sx: Sinus involvement - ulcers, cobblestoning Lungs - nodules, cavities lesions, pulm hemorrhage Kidneys - focal segmental crescentic GN, pauci-immune +/-c-anca, may have p-anca

Answer 411

Starts with asthma and allergic rhinitis 2- peripheral eosinophilia 3 - small vessel vasculitis dz Eos>1500 P-anca Tx mepolizumab Asthma persists even after vasculitis improved Most common cause mortality - cardiac dz

Answer 412

Pre-existing Ab Mins-hours

Answer 413

7d-3mo CD8 T cells (type 4 hypersensitivity)

Answer 414

Days-months Antibodies trigger complement, endothelial injry, thrombosis C4d deposition in capillaries (type II and iii hypersens)

Answer 415

Months-yrs T cells, cytokines Ongoing acute rejection Vascular smooth muscle proliferation, fibrosis, occlusion - cd4 vasculopathy or accelerated graft atherosclerosis

Answer 416

Neutro- 3 consecutive days with anc>0.5x10^9 or 1 day >1x10^9 Plt - >20x10^9 in 3 consecutive days

Answer 417

Donor t cells Il-10, ifn-g, tnf-a

Answer 418

Cd4 T cell mediated B cells, Th17 Prevent with T cell depletion Occurs >100 days (usu >6mo) Fibrosis and scarring

Answer 419

<100 days Cd45RO T cells Ppx mtx, cyclosporine, tacro, steroids Tissue injury - red angry rash, diarrhea, abd pain, weight loss

Answer 420

Sinusoids obstruction syndrome After transplant Most often with hematopoietic cell transplantation W/in 29 d transplant: Bili >2 Hepatomegaly/RUQ pain >2-5% wt gain from fluid retention

Answer 421

Cf unlikely sweat Cl /60

Answer 422

Ivacaftor - increases probability cftr channel opening (gating) Lumacaftor - Phe508del corrector, increases production cftr Tezecaftor - F508del corrector facilitates delivery of cftr to cell surface

Answer 423

Bla g 1, 2, 4 5 6 9 11 Per a 1

Answer 424

1. Dust mite 2. Short ragweed 3. Cat 4. Grasses - Bermuda and northern 5 Hymenoptera Extracts standardized by Center for Biologics Evaluation & Research/FDA

Answer 425

1000-1500 AU/BAU

Answer 426

Increase: IgG blocking Ab (first IgG1 then IgG4) IgA in resp secretions Cd4+cd25+ regulatory cells (il-10, tgf-b,) and % cd8 cells Initial increase and then decrease specific IgE Th1 cytokines (Ifn-g, il-12) Increased ifn-g:IL-4 ratio Histamine2 receptor Breg Dc4reg

Answer 427

Seasonal rise specific IgE Low affinity IgE receptor FceRII (cd23) B cell activation markers Basophil hyper reactivity Recruitment eos, basos, mast cells in nose/lung Allergen specific lymphocyte proliferation Th2, Th17, il-2 receptor, NFkB activation, il-8 secretion

Answer 428

Glycerine - inhibits proteolytic activity

Answer 429

Mold, cockroach Don’t mix with pollens, hdm, dander

Answer 430

5-20ug of major allergen Aka 1000-4000 BAU/AU

Answer 431

Single venom 100ug Mixed vespid 300ug

Answer 432

0.2% of shots end in reaction In 2% of patients 2% severe 2 fatalities/yr

Answer 433

5-grass pollen (Northergn grass, Kentucky blue, Timothy, orchard, perennial rye, sweet vernal) Timothy grass Short ragweed HDM

Answer 434

first RCT with Ragweed original studies from Noon & Freemen with timothy grass measuring conjunctival challenges

Answer 435

Stabilize histamine receptors in inactive state

Answer 436

renal - loratadine Hepatic - use fexofenadine Grapefruit juice decreases absorption antihistamines

Answer 437

cetirizine and levocetirizine

Answer 438

carmoterol, indacaterol - onset action 5 hrs, last >/24 Bulkier the side chain - more selective for B2 receptro larger size terminal amino group - protects from degradation by MAO

Answer 439

tremor, tachycardia, prolonged QTc, MI, transient increased hypoxia hyperglycemia, decreased K+ and Mg++

Answer 440

B16 arg/arg homozygotes - agonist receptor downregulation and resistance to SABAs

Answer 441

eg metoprolol, atenolol (not propranolol, etc)

Answer 442

LTC4, LTD4, LTE4 Form via 5-LO pathway, from LTA4 via LTC4 synthase

Answer 443

1 - improved FEV1 2 - decreased rescue inhaler use 3 - decreased asthma exacerbations

Answer 444

liver function

Answer 445

Eg cromolyn, nedocromil inhibit IgE-mediated Ca channel activation Increased intracell Ca needed for mast cell degranulation blocks activation neutrophils, eos, etc

Answer 446

M1 - on eos in those w/ copd M2 - inhibitory R on parasymp nerves; dysfunctional M2 causes increase ACh M3 - primary mediator smooth m contraction in airways - concentation bronchi >trachea > alveoli >airway epithelium ACh -> bronchoconstriction, mucus secretion, vasodilation

Answer 447

asthma - decreases hospitalizations COPD - 1st line, also in rhinitis Inhibits both M2 and M3 (muscarinic inhibitor/anticholinergic) quick onset - 7.6mins to 50% maximal broncodilation

Answer 448

longer action than ipratropium - dissociates more slowly than ipra

Answer 449

Decrease T > B cells, CD4>CD8 Upregulate CXCR4 slight decrease IgG/M Decrease eos, basos, monos Increase neutros (demargination) Innate immunity spared reverse decreased responsiveness to B agonists (increase receptors)

Answer 450

Stunt growth, Subcaspular posterior catacts Thrush Eye (glaucoma, cataracts, increased P), Endo (DM) Rage, raises BP Osteopenia Immunosuppresion Dysphonia, DM

Answer 451

does NOT prevent progressive loss lung function

Answer 452

mometasone > fluticasone >budesonide > triamcinolone

Answer 453

asthma >/6yo, evidence perennial aeroallergen (no improved pulm function) CU >/12 Nasal polyps >/18 Black box - anaphylaxis

Answer 454

Asthma >6yo, eos >150 HES, EGPA, CRSwNP >18yo Warning - herpes zoster

Answer 455

eos asthma >18 (eos >400) IV only Better in obese pts Black box - anaphylaxis

Answer 456

eos asthma >12yo (eos >300) Kills eos/basos via ADCC

Answer 457

Infliximab, adalimumab, certolizab, golimumab Block innate system

Answer 458

Blocks cd28 costimulation (b7-1, -2) Bu

Answer 459

Jak1/2 inhibitor

Answer 460

Jak 1/3 inhibitors Used in RA, psoriatic arthritis, UC

Answer 461

Jak 1/2 inhibitor Used in RA

Answer 462

Inhibits lymphocyte cell replication

Answer 463

Inhibits purine synthesis and metabolism Causes apoptosis T cells, inhibits t and B cell proliferation Urticarial vasculitis, chronic urticaria, glild

Answer 464

Affects purine nt synthesis and metabolism Decreased b and T cell proliferation, decreased Ab production Used in chronic urticaria, organ transplant

Answer 465

Inhibits Th function as calcineurin inhibitor, inhibits transcriptional factors (eg NFAT that is responsible for IL-2

Answer 466

Inhibits txnal factors (eg nfat), inhibits T cell activation Used in atopic derm >/2

Answer 467

Inhibits mTOR Inhibits T cell activation, proliferation, Ab production

Answer 468

Interfere with viral infections Type I - ifn a/b - antiviral Type II - ifn-g - macrophage activating effects; used in CGD Drugs - adverse effects flu like sx

Answer 469

Chimeric antigen receptor Have engineered receptors to recognize tumor cells For B cell malignancies, (cml and all) Some pts, car T cells become unresponsive over time Increased T cell activation can also lead to cytokine release syndrome

Answer 470

1 - gene therapy/addition (introduce gene into cell) 2 - gene editing, site specific (crispr/ Cas9 3 - gene silencing (does not change gene sequencing)

Answer 471

Isolation- Modified Cohn-Oncley cold ethanol fractionation

Answer 472

0.3-0.6g/kg/mo 400-600mg/kg q3-4wks For every 100mg increase —> 121mg increase in IgG trough

Answer 473

100mg/kg/wk

Answer 474

Higher dose - 2g/kg q3-4wks

Answer 475

0.01 mg/kg of 1:1000 for SC/IM 0.01 mg/kg of 1:10,000 IV

Answer 476

T indepen Pneumovax (23-valent) Meningococcal

Answer 477

T-dependent HiB, prevnar-13, meningococcal (menactra, menveo)

Answer 478

Separate pcv13 and menactra by at least 4 wks

Answer 479

At least 2 wks

Answer 480

Severe local reactions after vaccine administration due to high Ab titers Most common after 4th/5th dose DTaP Type iii hypersens - immune complex deposition

Answer 481

In Mmr, vzv, rabies, yellow fever

Answer 482

Strep pneumonia Flu Menu gococcal HPV

Answer 483

Live vaccines contraindicated Likely ineffective otherwise in complete SCID, questionable in partial defects

Answer 484

Love bacterial (can receive live viral)

Answer 485

If >20mg/d x2+wks -> wait 1 mo after stopping steroids

Answer 486

If live vaccines given first > wait 2 wks for ivig Ivig given first > wait 8+ months

Answer 487

OME 3 episodes/6mo or 4/yr Persistent OME x 3 mo (b/l) or 6 mo (unilateral) Hearing loss

Answer 488

Type I - anaphylaxis (acute IgE-mediated; requires sensitization) Type II - antibody depen cytotoxic rxns (cytopenias) Type III - immune complex (eg serum sickness) Type 4 - cell-mediated/delayed hypersens

Answer 489

IVa - th1 (ifn-g) - monocytes/macrophages - eczema, ppd IVb - Th1 (IL-4, -5) - eos - maculopapular, DRESS IVc - CTL (perforin and granzyme) - cd4/8 - bullous to pustular, increased cd8 in skin, SJS, fixed drug eruption IVd - T cells and IL8 - involve PMNs - pustular rash - AGEP

Answer 490

Most drugs by themselves no immunogenic, only when metabolized to reactive metabolite

Answer 491

When drug binds to TCR with string enough affinity, esp when interacting with MHC, then may become immunogenic

Answer 492

Latex, radiocontrast

Answer 493

HLA-B*5701 - abacavir (test mutation before starting) HLA-B*1502 - carbamazepine in Han Chinese (SJS/TEN) HLA-A*3101 - carbamazepine in Europeans HLA-B*5801 - allopurinol SCAR rxns in Asians HLA-B*1301 - dapsone

Answer 494

Quinidine, methyldooa, pcn

Answer 495

Vanco, hep (HIT, IgG to hep-plat factor 4) Sulfonamides, propulthiouracil, quinidine

Answer 496

Occurs 1-2wjs after first exposure or 1-7d in previously sensitized With pcns, sulfonamides, phenytoin Cefaclor most common cause kids Fever, arthralgia, LAD, erythema multiforme/urticaria

Answer 497

Local selling and tenderness, maybe necrosis after vaccination Typically with boosters Onset w/in hours , resolves in days

Answer 498

Anti-ro (ssa) -La (ssb) Hctz, CCB, ACE-I, antifungals

Answer 499

SJS <10% epidermal detachment TEN >30% involvement Steroids CONTRAINDICATED in TEN Pcn, sulfonamides, anticonvulsants, nsaids, allopurinol Fas/fasL mediated apoptosis epidermal cells, cd8 activation, perforin release Ivig may help (some anti-Fas Ab)

Answer 500

Anticonvulsants, antimicrobiana, sulfonamides, allopurinol, minocycline Fever, facial edema, LAD, hepatitis Weeks after therapy and May worsen even after Tx d/c

Answer 501

Mast cell receptor - certain drugs have this as a target, can cause pseudo allergic reactions (anaphylactoid) Eg nmba, fluiroquinolones

Answer 502

Acute generalized exanthematous pustulosis Pustular skin rash, fever, neutrophilia, eosinophilia Onset 24hrs of drug

Answer 503

97% rule out anaphylaxis potential

Answer 504

Major - benzylpenicilloyl polylysine (pre-pen) Minor - pen G, penicilloate

Answer 505

Mild Non IgE cutaneous sx, IgE rxn >5 yrs ago Unknown rxn

Answer 506

Tmp/smx in pts with HIV - delayed maculopapular rash - T cell mediated

Answer 507

VAncomycin May cause IgA bullous dermatitis

Answer 508

Quaternary ammonium muscle relaxants (succinylcholine)

Answer 509

Severe cutaneous reactions (eg blistering skin) immune complex mediated reactions - ten/SJS, dress, serum sickness, hemolytic anemia, drug induced hepatitis/nephritis

Answer 510

Early - histamine, tryptase, chymase, carboxypeptidase, substance P, calcitonin gene related peptide (cgrp) Late - cd4 T cell Eos

Answer 511

Pcn, chemo, muscle relaxants, insulin, heparin

Answer 512

Hgb levels Increased with exercise, supine position, L-R cardiac shunt Decreased by copd/emphysema, bronchiolitis obliterans, ild. May be high in asthmatics

Answer 513

Small P oscillations at mouth transmitted to lungs Measures resistance and reactance For those unable to cooperate with pft

Answer 514

Increase FEV1 of 12% AND >/200ml

Answer 515

Decreased FEV1/FVC with decreased DLCO

Answer 516

Concentration of Methacholine that causes decrease in fev1 by 20% = PC20 PC20 of

Answer 517

Methach**o**line with high sensitivity, rules **o**ut asthma Mann**i**tol with high specificity rules **i**n asthma (particularly exercise induced)

Answer 518

Increase : asthma, atopy, Uris, >12yp, copd exacerbation, Eosinophilic bronchitis Decrease: bronchiectasis, tobacco smoke, drugs (steroids, antileukotriene, NOS inhibitors), exercise, moderate altitude, hypothermia

Answer 519

Filiar shaft contains fibrils composed of nine outer pairs of microtubules and 2 central microtubules Adjacent doublets connected by dynein arms Microtubules composed of tubulin

Answer 520

CRS Sitús inversus Bronchiectasis Auto R Saccharine transit test shows slower mucociliary clearance/transit time Bx preference is from Carina

Answer 521

Charcot Leyden crystals Curschmamms spirals (corkscrew twists of condensed mucus) Creola bodies (clumps sloughed epithelial cells) ECO, MBP

Answer 522

Eos >3% Neutros > 76%

Answer 523

IgM > IgG3 > IgG2 > IgG2

Answer 524

AlphaBeta double negative T cells (Cd4-cd8-)

Answer 525

B and NK cells

Answer 526

Cd3+ cd25+ foxp3+

Answer 527

Cd19+ surface IgM+ IgD+

Answer 528

Cd19+ cd21+

Answer 529

Cd19+ cd27+

Answer 530

NK: cd45+ cd2+ cd16+ cd56+ NKT: cd45+ cd3+ cd16+ cd56+

Answer 531

CD21/cr2 also binds rhinovirus

Answer 532

Inhibits MAC in complement (binds c8, c9)

Answer 533

Defect in common beta chain in CD18

Answer 534

Defect in CD62E and P (NOT L)

Answer 535

MAdCAM-1 in gut

Answer 536

CH3 domain on FREE IgE At FCepsilonRI binding site

Answer 537

CCR5, CXCR4

Answer 538

Prevnar - conjugated polysaccharide, B and T cell Pneumovax 23 - unconjugated polysaccharide, B cell only

Answer 539

Dim - mature NK cells - majority of NK cells, higher cytokinin activity Bright - immature NK cell

Answer 540

Staph aureus Salmonella Serrati Pseudomonas

Answer 541

C1q/r/s, c4, c2

Answer 542

Factor B/D, properdin

Answer 543

Hereditary - ch50/ah50 absent. Only one component missing Acquired - levels low, not absent Multiple components low

Answer 544

Ab testing unreliable Use PCR

Answer 545

Extension of ethmoid sinus and air cell Along medial floor of orbit Can increase risk acute rhinisinusitis

Answer 546

Impaction - most common; indoor/outdoor sampling Impingement - liquid impingers draw air particles in; collect bioaerosols Filtration - suck particles through a filter; detect dust, endotoxins, antigens, fungi, microorganisms

Answer 547

Gravitational sampler; simplest method Slides coated w adhesive, exposed x 24h Cheap, durable, no power source needed Biased towards larger particles Cannot determine air concentration ( Don’t know v of air)

Answer 548

Sedimentation sampler Particles settle on agar plate, then incubated and counted (bacteria, molds) Identified viable airborn organisms Indoor use only, biased larger particles, can’t determine airborn concentration

Answer 549

Burkhard - suction slit, for pollen and spores, frequently on rooftops, efficient for particles <10um, but need consistent wind speed, sampled from 24h-7d; expensive Allergenico sampler - not wind-oriented, suction sampler collects on lab slides, primarily indoor use

Answer 550

Rotorod - Rods sweep through air to collect particles on surfaces with adhesive tape, Able to calculate air volume and concentration! Not affected by wind Not good for small particles (<10um) eg molds (they air stream around the rods)

Answer 551

Anderson sieve impinger - series of sieves, air drawn in and passes through stages/sieves with progressively smaller holes, separated based in size Used for culture based sampling fungi Expensive

Answer 552

20-45um Smooth surface with one pore

Answer 553

Timothy - Phl p 1-14 orchard rye - Lol p 1-14 fescue bluegrass - Poa p 1-14

Answer 554

Bermuda- cyn d 1-14 Bahia - Pas n 1, 13 Johnson - Sor h 1-14

Answer 555

Amb a 1-10, profilin, cystatin Spiny exine (outer surface), 15-25um Pollen-food allergy with banana, cantaloupe, watermelon

Answer 556

Art v 1-3, profilin Smooth surface, tricolporate OAS - celery spice, peach, mustard Mugwort celery spice syndrome - celery, coriander, peppers, parsley, fennel

Answer 557

25-30um Similar to ragweed with external spikes, but larger and smaller spines

Answer 558

One of smallest pollens 12-16um Tri-tetraporate Look wrinkled/air been sucked out of cell

Answer 559

20-40um Many pores Pore cap makes it look like doughnut

Answer 560

20-30um Tricolporate Starch inclusion granules - looks bubbly

Answer 561

Fra a 1 Furrows make square/pentagon appearance

Answer 562

Bet v 1-7 Triporate Looks like lemon when only 2 pores visible Oas - Apple, apricot, carrot, celery, cherry, coriander, fennel, hazelnut, kiwi, nectarine, parsley, parsnip, pear, pepper, plum, peach, potato, walnut

Answer 563

Pal, remember the ABC Pear, peach, plum, potato As well as apricot, apple are linked to Birch Along with Celery, cherry, And carrot

Answer 564

Jun a 1-3 With cypress, juniper Thick outer layer, granular inside, outer layer can break, look like PAC man

Answer 565

Que a 1 Oak, beech, chestnut Triangular, 3 white furrows slightly protruding from surface 25-35um Oak - O with corners - triangle

Answer 566

Pla a 1 Round, tricolpate, thin exine (outer covering)

Answer 567

Mickey Mouse Large - 50-100um

Answer 568

Tricolpate Look like beach balls with coloring

Answer 569

4-7 oval shaped pores Outer surface looks wavy

Answer 570

Poplar/cottonwood - round, outer granular surface; no furrows Willow - furrows

Answer 571

Soccer ball with many white pores

Answer 572

Small 11-20um 2 pores, Look like a lemon from the side (more granular than birch)

Answer 573

Walnut - Periporate - 9-15 pores

Answer 574

Covered in nerf pads

Answer 575

Club shaped Dry day mold - in hay, grains Alt a 1-12

Answer 576

Sausage chain, budding off Delray day spore - inside and out 6-25um Cla h 1-12

Answer 577

Indoor mold Looks very similar to penicillin Like dandelion puff Asp f 1-22

Answer 578

Paintbrush Hypersens pneumonitis Pen ch 13,18,20

Answer 579

Single spores with thick walls Look like worm Allergic fungal sinusitis

Answer 580

Round multicell dry day spore Dark brown, circular, warts on surface

Answer 581

Colorless Wet day spore 20-50um Spindle shaped and curved with tapered ends - string bean

Answer 582

Lots of shapes and sizes 8 ascopores to an ascus

Answer 583

ACE Alternaria Cladosporidium Epicoccum

Answer 584

Ascopores Basidiospores

Answer 585

Der p 1 and Der f 1 89% homology Cysteine proteases Der p/f2 - 80% homology Lipopolysaccharise binding proteins

Answer 586

Bla g Per a

Answer 587

Ozone, NO2, SO2, CO, PM

Answer 588

0-50 - good 51-100 - mod 101-150 - unhealthy for sensitive groups 151-200 - unhealthy 201-300 - v unhealthy 301-500 - hazardous

Answer 589

Precursor to o3 Decreased in lung function, increased airway neutrophils, pro inflammatory cytokines Allergen response at 0.4ppm

Answer 590

Peaks summer and in afternoon In asthmatics, increases sx, hospitalizations, b agonist use Decreased lung function acutely and chronically, Increases neutrophils in airway, Il-6, Il-8, leukotrienes, Increased airway reactivity and inflammation Antioxidants (alpha-tocopherol, ascorbate) can help decrease ozone response, esp in gtsm1 def children

Answer 591

Decreased lung function in children Bronchospasm in asthmatics at 0.5ppm, reverses with beta agonists

Answer 592

Increased allergen sensitization Airway inflammation Asthma CV disease Strep pneumonia binding to airway epithelium Pollutants activate innate immunity, increase TLRs and DAMPs -> inflammasome -> il-6, il-8

Answer 593

Assoc w recurrent OM, uri, lri, wheezing, cancer Increases th2 and eos

Answer 594

DM, cockroach, mold Can’t mix mold/cockroach with pollens (DM ok with pollen)

Answer 595

eos asthma >6yo AD >/6months. (can cause conjunctivitis) CRSwNP > 18 EOE >12

Answer 596

Staphylococcus aureus, Serratia marsescens, Burkholderia cepacia, Nocardia, Aspergillus.

Answer 597

pdC1-INH, subcutaneous

Answer 598

pdC1-INH, subcutaneous

Answer 599

0.3-0.5 of 1:1,000

Answer 600

NF-KB Ub of Nemo allows IKK-beta to become active —> P inhibitory component and allows NFkB to local size to nucleus

Answer 601

Factor H deficiency

Answer 602

TH1 -- STAT3, Tbet TH2 - STAT6, GATA3 TH17 - RORgT, STAT3 Tfh - BCL-6 Treg - STAT5

Answer 603

Incidence among exposed/incidence unexposed A/(a+b) ———— C/(c+d)

Answer 604

1-RR (ARC-ART)/ARC

Review Book Flashcards

(664 cards)