Uworld 2017 Flashcards
Disseminated mycobacterial disease in infancy and early childhood
Defect involving IFN-gamma signaling pathway
IL-12 receptor deficiency
Anaphylaxis treatment
IM epinephrine
Airway management and volume resuscitation
Adjunctive therapy (glucocorticoids, antihistamines)
Impairment of complement-mediated cell lysis by…
Staphylococcal protein A
Promotion of intracellular survival by…
Inhibition of phagosome-lysosome fusion or resistance to lysosomal enzymes.
Responsible for the apoptosis of cells with decreased or absent MHC class I proteins (virus-infected or tumor cells)
Natural Killer cells
Which IL is responsible for B-cell growth, isotype switching, secretion of IgE, predisposition to HS type I, and differentiation into Th2 helper?
IL-4
The first IL produced by T-cells after contact with Ag
IL-2
5 manifastations of Ataxia-Telangiectasia
Cerebellar ataxia (atrophy) Oculocutaneous telangiectasia Severe immunodeficiency (IgA, IgE, IgG def) Repeated sino-pulmonary infections Increased risk of cancer (hemato)
5 inherited disorders caused by deficient DNA-repair enzymes
Ataxia-Telangiectasia Xeroderma pigmentosum Fanconi anemia Bloom syndrome HNPCC
Infection following live vaccine
Deficiency of T-cell mediated immunity
Recurrent infections by Neisseria
Deficiency of C5b-C9
Triad of Wiskott-Aldrich syndrome
Eczema
Thrombocytopenia
Immunodefficiency (recurrent inf)
3 manifestations of Leukocyte Adhesion Deficiency (absence of CD18)
Recurrent skin and mucosal infections without purulence
Delayed seperation of umbilical cord
Persistent leukocytosis
5 mechanisms that trigger apoptosis
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)
2 lab findings and the only treatment for Severe Combined ImmunoDeficiency
Lymphopenia
Hypogammaglobulinemia
Stem cell transplant
Triad of Hemolytic Uremic Syndrome
Microangiopathic hemolytic anemia
Thrombocytopenia
Acute renal failure
2 diseases caused by blood type O mother and blood type A or B fetus
Erythroblastosis fetalis
Hemolytic disease of the newborn
4 seronegative spondylarthropathies (absence of serum rheumatoid factor) + higher incidence of HLA-B27
Ankylosing spondylitis
Reactive arthritis
Arthritis associated with IBD
Psoriatic arthritis
2 most important opsonins and 2 others
IgG
C3b
Mannose-binding lectin
CRP
Classic triad of reactive arthritis
Nongonococcal urethritis
Conjunctivitis
Arthritis
2 causes of Angioedema
Mast cell activation (ass w/ pruritus and urticaria) Excess bradykinin (No pruritus, No urticaria)
Pneumococcal vaccines
Conjugate: strongly immunogenic in infancy: B + T cells recruitment; Higher, longer-lasting Ab
Polysaccharide: poorly immunogenic in infancy (immature humoral response)
Ataxia telangiectasia
Triad: Cerebellar ataxia, Telangiectasias, Increased risk of sinopulmonary infections ATM gene: role in DNA break repair IgA deficiency (sinopulm inf)
Disseminated mycobacterial ds in infancy/early childhood
Inherited defect of IFNg signaling
Lifelong ttt w/ antimycobacterial agents
3 findings in Selective IgA deficiency
Recurrent sinopulmonary + GI infections
Autoimmune disease
Severe: anaphylaxis during transfusion of blood products w/ IgA
3 findings in Rheumatoid arthritis
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)
IFNa + IFNb
In response to viral infections
Help suppress viral replication: halt protein synthesis + promote apoptosis of inf cells
Expression of MHC II
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
High-affinity IgE receptor (FcERI)
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
4 findings in Chronic renal allograft rejection
Months to years
Worsening HTN, slowly progressive rise in creatinine
Chronic, indirect immune response against donor alloAg
Obliterative intimal thickening, tubular atrophy, interstitial fibrosis
4 characteristics of Kartagener sd
Form of primary ciliary dyskinesia
Impaired structure/function of cilia (dynein arms)
Triad: situs inversus, chronic sinusitis, bronchiectasis
Infertility (sperm flagella, fallopian tube cilia)
Giant cells in Mycobacterium tuberculosis inf
Langhans giant cells formed by macrophages
These macroph activated by CD4+ Th1 lymphocytes
Fas receptor role in apoptosis
Initiate extrinsic pathway of apoptosis
Mutations in Fas recept or Fas ligand: prevent apop of autoreactive lymphocytes, high risk of autoimmune ds (SLE)
Calcineurin
Activation of IL2 which promotes growth+differentiation of T cells
Cyclosporine + tacrolimus inhibit calcineurin activation
Acute hemolytic transfusion reaction
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
Pancytopenia + nephritis in SLE
Pancytopenia: autoAb against blood cells (HS type II)
Nephritis: immune complex deposition in glomerulus (HS type III)
Cells + cytokines
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
Chemotactic agents
Leukotriene B4: neutro migration to sites of infl
5-HETE
C5a
IL8