Uworld 2017 Flashcards

1
Q

Disseminated mycobacterial disease in infancy and early childhood

A

Defect involving IFN-gamma signaling pathway

IL-12 receptor deficiency

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2
Q

Anaphylaxis treatment

A

IM epinephrine
Airway management and volume resuscitation
Adjunctive therapy (glucocorticoids, antihistamines)

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3
Q

Impairment of complement-mediated cell lysis by…

A

Staphylococcal protein A

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4
Q

Promotion of intracellular survival by…

A

Inhibition of phagosome-lysosome fusion or resistance to lysosomal enzymes.

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5
Q

Responsible for the apoptosis of cells with decreased or absent MHC class I proteins (virus-infected or tumor cells)

A

Natural Killer cells

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6
Q

Which IL is responsible for B-cell growth, isotype switching, secretion of IgE, predisposition to HS type I, and differentiation into Th2 helper?

A

IL-4

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7
Q

The first IL produced by T-cells after contact with Ag

A

IL-2

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8
Q

5 manifastations of Ataxia-Telangiectasia

A
Cerebellar ataxia (atrophy)
Oculocutaneous telangiectasia
Severe immunodeficiency (IgA, IgE, IgG def)
Repeated sino-pulmonary infections
Increased risk of cancer (hemato)
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9
Q

5 inherited disorders caused by deficient DNA-repair enzymes

A
Ataxia-Telangiectasia
Xeroderma pigmentosum
Fanconi anemia
Bloom syndrome
HNPCC
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10
Q

Infection following live vaccine

A

Deficiency of T-cell mediated immunity

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11
Q

Recurrent infections by Neisseria

A

Deficiency of C5b-C9

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12
Q

Triad of Wiskott-Aldrich syndrome

A

Eczema
Thrombocytopenia
Immunodefficiency (recurrent inf)

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13
Q

3 manifestations of Leukocyte Adhesion Deficiency (absence of CD18)

A

Recurrent skin and mucosal infections without purulence
Delayed seperation of umbilical cord
Persistent leukocytosis

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14
Q

5 mechanisms that trigger apoptosis

A

Deprevation of growth factors
DNA damage
Intracellular accumulation of misfolded proteins
Mediation by cytotoxic T lymphocytes
Activation of receptors in the TNF receptor family (Fas)

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15
Q

2 lab findings and the only treatment for Severe Combined ImmunoDeficiency

A

Lymphopenia
Hypogammaglobulinemia

Stem cell transplant

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16
Q

Triad of Hemolytic Uremic Syndrome

A

Microangiopathic hemolytic anemia
Thrombocytopenia
Acute renal failure

17
Q

2 diseases caused by blood type O mother and blood type A or B fetus

A

Erythroblastosis fetalis

Hemolytic disease of the newborn

18
Q

4 seronegative spondylarthropathies (absence of serum rheumatoid factor) + higher incidence of HLA-B27

A

Ankylosing spondylitis
Reactive arthritis
Arthritis associated with IBD
Psoriatic arthritis

19
Q

2 most important opsonins and 2 others

A

IgG
C3b

Mannose-binding lectin
CRP

20
Q

Classic triad of reactive arthritis

A

Nongonococcal urethritis
Conjunctivitis
Arthritis

21
Q

2 causes of Angioedema

A
Mast cell activation (ass w/ pruritus and urticaria)
Excess bradykinin (No pruritus, No urticaria)
22
Q

Pneumococcal vaccines

A

Conjugate: strongly immunogenic in infancy: B + T cells recruitment; Higher, longer-lasting Ab

Polysaccharide: poorly immunogenic in infancy (immature humoral response)

23
Q

Ataxia telangiectasia

A
Triad: Cerebellar ataxia, Telangiectasias, Increased risk of sinopulmonary infections
ATM gene: role in DNA break repair
IgA deficiency (sinopulm inf)
24
Q

Disseminated mycobacterial ds in infancy/early childhood

A

Inherited defect of IFNg signaling

Lifelong ttt w/ antimycobacterial agents

25
Q

3 findings in Selective IgA deficiency

A

Recurrent sinopulmonary + GI infections
Autoimmune disease
Severe: anaphylaxis during transfusion of blood products w/ IgA

26
Q

3 findings in Rheumatoid arthritis

A

AutoAb against autoAg in joints
Infiltrating CD4+ cells secrete cytokines + stimulate B cells
B cells produce RF (IgM against Fc of IgG) + anti-citrullinated protein Ab (chronic infl)

27
Q

IFNa + IFNb

A

In response to viral infections

Help suppress viral replication: halt protein synthesis + promote apoptosis of inf cells

28
Q

Expression of MHC II

A

Foreign Ag taken into APC by phagocytosis or endocytosis
Loaded onto MHC II within acidified endosomes
Ag-MHC II expressed on cell surface

Failure to acidify lysosomes: deficient expression of Ag-MHC II + of interaction btw APC-Tcell

29
Q

High-affinity IgE receptor (FcERI)

A

On surface of mast cells + basophils
Binds Fc of IgE (HS I)
Aggregation of FcERI receptors when cross-linking of multiple bound IgE by multivalent Ag then degranulation + allergic reaction

30
Q

4 findings in Chronic renal allograft rejection

A

Months to years
Worsening HTN, slowly progressive rise in creatinine
Chronic, indirect immune response against donor alloAg
Obliterative intimal thickening, tubular atrophy, interstitial fibrosis

31
Q

4 characteristics of Kartagener sd

A

Form of primary ciliary dyskinesia
Impaired structure/function of cilia (dynein arms)
Triad: situs inversus, chronic sinusitis, bronchiectasis
Infertility (sperm flagella, fallopian tube cilia)

32
Q

Giant cells in Mycobacterium tuberculosis inf

A

Langhans giant cells formed by macrophages

These macroph activated by CD4+ Th1 lymphocytes

33
Q

Fas receptor role in apoptosis

A

Initiate extrinsic pathway of apoptosis

Mutations in Fas recept or Fas ligand: prevent apop of autoreactive lymphocytes, high risk of autoimmune ds (SLE)

34
Q

Calcineurin

A

Activation of IL2 which promotes growth+differentiation of T cells
Cyclosporine + tacrolimus inhibit calcineurin activation

35
Q

Acute hemolytic transfusion reaction

A

Ab-mediated HS (type II) by pre-existing anti-ABO Ab
Against donor RBCs
Complement activation: RBCs lysis, vasodilation, fever, hypotension, chest/back pain, hemoglobinuria

36
Q

Pancytopenia + nephritis in SLE

A

Pancytopenia: autoAb against blood cells (HS type II)
Nephritis: immune complex deposition in glomerulus (HS type III)

37
Q

Cells + cytokines

A

Sensitized TH2: IL4, IL13 together promote B-cell switching for IgE synthesis. IL5 activ eosino + IgA synthesis
Excess of TH2 products: extrinsic allergic asthma
Macrophages: IL1 stimulates Th cells
Th cells: IL3 recruits BM stem cells + IFNg activates macrophages
TGFb for tissue regen + repair

38
Q

Chemotactic agents

A

Leukotriene B4: neutro migration to sites of infl
5-HETE
C5a
IL8