USMLE (Fach) / Immunology (Lektion)
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- Autoantibodies - Anticentromere Limited scleroderma (CREST syndrome)
- Autoantibodies - Antisynthetase (anti-Jo-1), anti-SRP, ... Anti-Jo-1 = anti-histidyl-tRNA synthetase Polymyositis, dermatomyositis
- Autoantibodies - Antimicrosomal, antithyroglobulin, ... Hashimoto thyroiditis
- Autoantibodies - Antimitochondrial Primary biliary cirrhosis
- Autoantibodies - Anti-Scl-70 Anti-DNA topoisomerase I antibody. Diffuse scleroderma
- Autoantibodies - Anti-smooth muscle Autoimmune hepatitis type 1
- Autoantibodies - Anti-SSA, anti SSB (anti-Ro, anti-La) ... Sjögren syndrome
- Autoantibodies - Anti-U1 RNP (ribonucleoprotein) Mixed connective tissue dissease
- Autoantibodies - MPO-ANCA/p-ANCA - Microscopic polyangiitis - Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) - Ulcerative colitis
- Autoantibodies - PR3-ANCA/c-ANCA Granulomatosis with polyangiitis (Wegener)
- Autoantibodies - Rheumatoid factor, anti-CCP (more ... Rheumatoid arthritis
- X-linked (Bruton) agammaglobulinemia Developmental failure to produce mature B-cells and plasma cells, resulting in agammaglobulinemia.- Defect in BTK, a tyrosine kinase gene → no B-cell maturation - X-linked recessive (↑ in boys) - ...
- Selective IgA deficiency - Most common 1° immunodeficiency - Majority asymptomatic- Airway and GI infections (eg, Giardia)- Autoimmune disease (eg, celiac disease, inflammatory bowel disease)- Atopy- Anaphylaxis to IgA-containing ...
- Common variable immunodeficiency Defect in B-cell differentiation. Cause unknown. - Usually presents after age 2 and may be considerably delayed (age 25-35). - Sinupulmonary infections- ↑ risk of autoimmune disease (eg, rheumatoid ...
- Thymic aplasia (DiGeorge syndrome) 22q11 deletion; failure to develop 3rd and 4th pharyngeal pouches → absent thymus and parathyroids - Tetany (hypocalcemia)- Recurrent viral/fungal infections (T-cell deficiency)- Conotruncal abnormalities ...
- Severe combined immunodeficiency Several types including defective IL-2R gamma chain (most common, X-linked), adenosine deaminase deficiency (autosomal recessive) - Failure to thrive- Chronic bacterial diarrhea- Chronic candidiases (eg, ...
- Ataxia-telangiectasia Defects in ATM gene → failure to detect DNA damage → failure to halt progression of cell cycle and perform non-homologous end-joining → mutations accumulate - Autosomal recessive Triad:- Cerebellar ...
- Hyper-IgM syndrome Most commonly due to defective CD40L on Th cells → class switching defect - X-linked recessive - Severe pyogenic infections early in life- Opportunistic infection with Pneumocystis, Cryptosporidium, ...
- Wiskott-Aldrich syndrome Mutation in WASp gene; leukocytes and platelets unable to reorganize actin cytoskeleton → defective antigen presentation - X-linked recessive - Eczema- Thrombocytopenia- Recurrent (pyogenic) infections- ↑ ...
- Leukocyte adhesion deficiency Defect in LFA-1 integrin (CD18) protein on phagocytes; impaired leukocytic migration and chemotaxis (especially tight binding, adhesion). - Autosomal recessive - Recurrent nonsuppurative bacterial skin ...
- Chronic granulomatous disease Defect of NADPH oxidase → ↓ reactive oxygen species (eg, superoxide) and ↓ respiratory burst in neutrophils. - X-linked form most common - ↑ susceptibility to catalase ⊕ organisms (Nocardia, ...
- Chédiak-Higashi syndrome Defect in lysosomal trafficking regulator gene (LYST).Microtubule dysfunction in phagosome-lysosome fusion. - Autosomal recessive - Lymphohistiocytosis- Partial albinism- Recurrent pyogenic infections ...
- C3 deficiency Increases risk of severe, recurrent pyogenic sinus and respiratory tract infections ↑ susceptibility to type III hypersensitivity reactions
- C1 esterase inhibitor deficiency Causes hereditary angioedema due to unregulated activation of kallikrein → ↑ bradykinin. Characterized by ↓ C4 levels. ACE inhibitors are contraindicated. - Facial swelling without urticaria- Life-threatening ...
- Hyper-IgE syndrome (Job syndrome) Deficiency of Th17 cells due to STAT3 mutation → defect in neutrophil chemotaxis - Autosomal dominant FATED:- Coarse facies- Staphylococcal abscesses (noninflamed)- Retained primary teeth- ↑ IgE- ...
- IL-12 receptor deficiency ↓ Th1 response - Autosomal recessive - Disseminated mycobacterial and fungal infections (eg, Salmonella)- May present after administration of BCG vaccine with tuberculosis infection Findings: ↓ ...
- Chronic mucocutaneous candidiasis T-cell dysfunction. Can result from congenital genetic defects in IL-17 or IL-17 receptors. - Noninvasive Candida albicans infections of skin and mucous membranes Findings:- Absent in vitro T-cell proliferation ...
- Cytokines secreted by macrophages Interleukin-1: Causes fever, acute inflammation. Activates endothelium to express adhesion molecules. Induces chemokine secretion to recruit WBCs. Also known as osteoclast-activating factor. Interleukin-6: ...
- Cytokines secreted by Th1 cells Interferon-γ: - Secreted by NK cells and T cells in response to antigen or IL-12 from macrophages. - Stimulates macrophages to kill phagocytosed pathogens.- Inhibits differentiation of Th2 cells. - ...
- Cytokines secreted from Th2 cells Interleukin-4: Induces differentiation of T cells into Th2 cells. Promotes growth of B cells. Enhances class switching to IgE and IgG. Interleukin-5: Promotes growth and differentiation of B cells. Enhances ...
- Major histocompatibility complex I and II MHC encoded by HLA genes. Present antigen fragments to T cells and bind T-cell receptors (TCRs). MHC I:- HLA-A, HLA-B, HLA-C- Binding: TCR and CD8- 1 long chain, 1 short chain, β2-microglobulin- Expression: ...
- HLA subtypes associated with diseases - A3 Hemochromatosis
- HLA subtypes associated with diseases - B8 - Addison disease - Myasthenia gravis - Graves disease
- HLA subtypes associated with diseases - B27 - Anklyosing spondylarthritis - Psoriasis arthritis - Inflammatory bowel diseases-associated arthritis - Reactive arthritis AKA seronegative arthropathies
- HLA subtypes associated with diseases - DQ2/DQ8 Celiac disease
- HLA subtypes associated with diseases - DR2 - Multiple sclerosis - Hay fever - SLE - Goodpasture syndrome
- HLA subtypes associated with diseases - DR3 - Diabetes mellitus type 1 - SLE (2,3, S-L-E) - Graves disease - Hashimoto thyroiditis - Addison disease
- HLA subtypes associated with diseases - DR4 - Rheumatoid arthritis - Diabetes mellitus type 1 - Addison disease
- HLA subtypes associated with diseases - DR5 Hashimoto thyroiditis
- Early complement deficiencies (C1-C4) Increased risk of severe, recurrent pyogenic sinus and respiratory tract infections. Increased risk of SLE.
- Terminal complement deficiencies (C5-C9) Increased susceptibility to recurrent Neisseria bacteremia.
- Paroxysmal nocturnal hemoglobinuria A defect in the PICA gene preventing the formation of GPI-anchors for complement inhibitors, such as decay-accelerating factor (DAF/CD55) and membrane inhibitor of reactive lysis (MIRL/CD59). Causes ...
- Allergic/anaphylactic reaction to blood transfusion ... Type I hypersensitivity reaction against plasma proteins in transfused blood. IgA deficient individuals must receive blood products without IgA. Presentation:- Urticaria- Pruritus- Fever- Wheezing- ...
- Febrile nonhemolytic transfusion reaction Two known mechanisms:- Type II hypersensitivity reaction with host antibodies against donor HLA and WBCs- Induced by cytokines that are created and accumulate during the storage of blood products Presentation:- ...
- Acute hemolytic transfusion reaction Type II hypersensitivity reaction. Intravascular hemolysis (ABO blood group incompatibility) or extravascular hemolysis (host antibody reaction against foreign antigen on donor RBCs). Presentation:- Fever- ...
- Transfusion-related acute lung injury Donor anti-leukocyte antibodies against recipient neutrophils and pulmonary endothelial cells. Presentation:- Respiratory distress - Noncardiogenic pulmonary edema Timing: Within 6 hours
- Autoantibodies - Anti-β2 glycoprotein Antiphospholipid syndrome
- Autoantibodies - Anticardiolipin, lupus anticoagulant ... - SLE - Antiphospholipid syndrome
- Autoantibodies - Anti-dsDNA, anti-Smith SLE
- Autoantibodies - Anti-histone Drug-induced lupus