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USMLE
General Pathology 3
| Question | Answer |
|---|---|
| Bruton's agammaglobulinemia | X-linked recessive. No B cells, No Igs. Recurrent staph, haemophilus and strep infections after 6 months. Increased pre-B cells. Mutation of B-cell Bruton tyrosine kinase (btK). |
| Common variable immunodeficiency | B-cell maturation defect. Hypogammaglubulinemia, recurrent bacterial infections, giargia lamblia. |
| DiGeorge syndrome | Failure to develop 3rd and 4th pharyngeal puches results in absence of parathyroid and thymus glands, hypocalcemia, tetany, T-cell deficiency, recurrent viral infections, heart defects, chronic candidiasis |
| SCID | B and T cell deficiency due to mutation of IL-2 receptor (x-linked), adenosine deaminase deficiency (AR) or failure to make MHC II. Recurrent infections and susceptibility to candida, CMV and p. carinii |
| Wiskot-Aldrich syndrome | X-linked recessive. "WIPE": recurrent infections, thrombocytopenic purpura, eczema, risk of lymphomas, low IgM |
| Ataxia-Telangiectasia | Ataxia, spider angiomas, low IgA, defect of DNA repair enzyme |
| Chronic granulomatous disease | Low NADPH oxidase. Recurrent catalase+ infections, negative nitroblue tetrazolium test. |
| Leukocyte adhesion deficiency | Defect of CD-18 (LFA-1 beta chain), no pus formation, failure of umbilical cord to detach |
| Chediak-Higashi | Defect in microtubulus with no phagocytosis by lysosome. Partial albinism, peripheral neuropahty, recurrent infections |
| Hereditary andioedema | Edema at mucosal surfaces. Defect of C1-INH (esterase inhibitor). Decreased C1, C2, C4 |
| Hyper IgM | Defect of CD-40L on T-lymphocytes. No isotope switching, increased IgM |
| MHC-I deficiency | Normal CD4, no CD8. Failure of TAP-1 to transport peptides to MHC-I groove |
| Amyloid stains | Stains red with Congo-red stain then apple green birefringence under polarized light |
| Signs and symptoms of amyloidosis | Nephrotic syndrome, renal failure, arrythmias, CHF, hepatosplenomegaly, macroglosia |
| Mediators of leukocyte margination | Selectins mediate margination. P and E selctins on endothelium bind Sialyl-Lewis on leukocyte; GlyCAM/CD34 on endothelium binds L-selectin on leukocyte |
| Mediators of leukocyte adhesion | Integrins mediate adhesion. ICAM, VCAM on endothelium bind LFA-1 and VLA on leukocyte |
| Regulators of leukocyte margination and adhesion | Histamine upregulates P-selectin. IL-1 and TNF induce E-selectin, ICAM and VCAM. Chemotactic agents cause conformational change of LFA-1 |
| Leukocyte adhesion deficiency | Defect of CD18 (beta chain subunit of LFA-1 integrin on leukocytes). Recurrent infections, no pus formation, failure of umbilical cord to detach |
| Chemotactic factors | N-formyl methionine, leukotriene B4, C5a, IL-8 |
| Opsonins | Fc portion of IgG, c3b, C reactive protein |
| Chediak-Higashi syndrome | Defect of microtubule polymerization causes defect in chemotaxix and degranulation. Partial albinism, peropheral neuropathy |
| CGD | NADPH oxidase deficiency. No production of superoxide for respiratory burst. Recurrent catalase+ infections, negative nitroblue tetrazolium test |
| Arachidonic acid products | AA producedd by phospholipase A2 (inhibited by steroids). Produces leukotrienes, prostaglandins and thromboxane A2 |
| Lypoxigenase pathway | Arachidonic acid is converted to leukotrienes by 5-lypoxigenase. LTB4 --> chemotaxis. LTC4, D4, E4 --> bronchoconstriction |
| Cycloxigenase pathway | Arachidonic acid is converted to TXA2 and prostaglandins (NSAIDs block). TXA2 --> vasoconstriction, platelet aggregator. PGI2, PGE2, PGF2 |
| Mediators of vasodilation | Histamine , bradikinin, PGI2, PGD2, E2, F2 |
| Mediators of pain | Bradikinin, PGE2 |
| Mediators of increased permeability | Histamine, Bradikinin |
| Mediators of vasoconstriction | TXA2, LTC4, D4, E4 |
| Mediators of bronchoconstriction | LTC4, D4, E4, bradikinin |
| Mediators of fever | IL-1, PGD2, E2, F2 |
| Anaphilotoxins | C3a, C5a. Directly stimulate histamine release from basophils, mast cells and platelets |
| C3b | Opsonin; neutrophils, macrophages and monocytes have C3b receptros |
| Bradikinin synthesis and actions | Synthesized from activation of prekalikrein by factor XII (Hageman). Kalikrein cleaves HMWK into bradikinin. Vasodilator, increased permeability, brnchoconstrictor, pain |
| PGE2 | Vasodilation in kidneys, increases renal blood flow, increases gastric mucosal blood flow (mucoprotection), activates osteoclasts, fever, pain, maintains ductus arteriosus |
| Prostacyclin (PGI2) | Vasodilation and inhibits platelet aggregation |
| IL-1 | Stimulates PGE2 synthesis in hypothalamus --> fever; B-cell stimulation to synthesize Ig; osteoclast activation (released by osteoblasts under PTH stimulation); lytic bone lessions of multiple myeloma; increases adhesion molecules in endothelium; increase |
| Hageman factor | Activates intrinsic coagulation system, kinin cascade and fibrinolytic system |
| PGF | Uterine muscle contraction (cause pf primary amenorrhea) |
| gamma interferon | Produced by CD4 cells and NK cells. Activates macrophages; antiviral properties; class I and class II antigens; increases IL-2, IL-12 production by CD4 cells |
| IL-2 | Produced by CD4 cells. T cell growth factor. Promotes B cell and NK cell proliferation |
| IL-6 | Synthesis of acute phase reactants |
| Factors that increase adhesion molecule synthesis | C5a, LTB4, IL-1, TNF |
| Key cells in acute and chronic inflammation | Acute: neutrophil has IgG and C3b receptros; Chronic: macrophage has receptors for IgG and C3b, process antigen and secrete IL-1, IL-12 and TNF |
| Chronic granulomatous inflammation | Epitheloid cells and multinucleated giant cells surrounded by a rim of lymphocytes with central caseous necrosis |
Created by:
Asclepius
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