Term
| What structures of the inner ear are responsible for the sensation of linear and angular acceleration? |
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Definition
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Term
DiGeorge Syndrome
(Thymic Aplasia) |
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Definition
| 22q11 deletion; failure to develop 3rd and 4th pharyngeal pouches; thymus and parathyroids fail to develop --> decrease T cells, decrease PTH, decrease Ca2+; tetany, congenital heart and great vessel defects |
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Term
| Job's Syndrome (Hyper IgE syndrome) |
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Definition
| Th cells fails to produce IFN-gamma --> failure of neutrophils to respond to chemotactic stimuli; FATED: coarse Facies, staph Abcesses, retained primary Teeth, increase IgE, Dermatological problems; increase IgE |
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Term
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Definition
| Defective CD40L on Th cells = inability to class switch; severe pyogenic infections early in life; increase IgM; decrease IgG, IgA, IgE |
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Term
Severe Combined Immunodeficiency
(SCID) |
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Definition
| Several types: IL-2 receptor deficiency (decrease T cell activation); Adenosine deaminase deficiency (increase adenosine toxic to T and B cells); |
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Term
Define:
Autograft
Syngeneic graft
Allograft
Xenograft |
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Definition
From self.
From identical twin or clone.
From nonidentical individual of same species. From different species. |
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Term
| Chronic granulomatous disease |
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Definition
| Lack of NADPH oxidase --> decrease reactive oxygen species and absent burst of respiratory neutrophils; increase susceptibility to catalase-positive organisms |
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Term
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Definition
| Defect in microtubular function w/ decrease phagocytosis; recurrent pyogenic infection w/ Staph and Strep, partial albinism, peripheral neuropathy |
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Term
| Hyperacute Transplant Rejection |
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Definition
| Antibody mediated (Type II) due to presence of antidonor antibodies in the recipient; occurs within minutes; occludes graft vessels, causing ischemia and necrosis |
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Term
| Acute Transplant Rejection |
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Definition
| Cell mediated due to cytotoxic T lymphocytes reacting to foreign MHC; occurs within weeks; reversible w/ immunosuppressants like Cyclosporine and OKT3; vasculitis of graft vessels w/ dense interstitial lymphocyte infiltration |
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Term
| Chronic Transplant Rejection |
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Definition
| T cell and antibody-mediated vascular damage; occurs in months to years; Irreversible; Class I MHCs perceived by CTLs as class I MHCs presenting a non-self antigen; fibrosis of graft tissue and vessels |
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Term
| Graft-versus-Host Disease |
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Definition
| Grafted immunocompetent T cells proliferate in irradiated immunocompromised host and reject host cells as "foreign", resulting in severe organ dysfunction; symptoms include maculopapular rash, jaundice, hepetosplenomegaly, and diarrhea; usually in bone marrow or liver transplant |
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Term
Cyclosporine
MOA?
Clinical Use?
Toxicity? |
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Definition
Binds to cyclophilins; complex blocks differentiation and activation of T cells thus preventing prevention of IL-2 and IL-2R.
Suppress organ rejection, slected autoimmune disorders.
Predisposes pts to viral infections and lymphoma; nephrotoxic. |
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Term
Tacrolimus (FK506)
MOA?
Clinical Use?
Toxicity? |
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Definition
Binds FK-binding protein, inhibiting secretion of IL-2 and other cytokines.
Potent immunosuppressant for organ transplantation.
Significant nephrotoxicity, peripheral neuropathy, hypertension, pleural effusion, hyperglycemia. |
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Term
Sirolimus (Rapamycin)
MOA?
Clinical Use?
Toxicity? |
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Definition
Binds to mTOR, inhibits T cell proliferation in response to IL-2.
Immunosuppression after kidney transplant in combination w/ cyclosporine and corticosteroids.
Hyperlipidemia, thrombocytopenia, leukopenia. |
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Term
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Definition
| Monoclonal antibody w/ high affinity for IL-2R on activated T cells. |
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Term
Azathioprine
MOA?
Clinical Use?
Toxicity? |
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Definition
Antimetabolite precursor of 6-mercaptopurine that interferes w/ metabolism and synthesis of nucleic acid. Toxic to proliferating lymphocytes.
Kidney transplantation, autoimmune disorders.
Bone marrow suppression; active metabolite mercaptopurine is metabolized by xanthine oxidase so toxic effects are increased by Allpurinol. |
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Term
Muromonab-CD3 (OKT3)
MOA?
Clinical Use?
Toxicity? |
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Definition
Monoclonal antibody that binds to CD3 on surface of Tcells; blocks cellular interaction w/ CD3 protein responsible for T cell signal transduction.
Immunosuppression after kidney transplant.
Cytokine release syndrome, hypersensitivity reaction. |
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Term
| Amino acids needed for purine synthesis? |
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Definition
Glycine
Aspartate
Glutamine |
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Term
| Adenosine Deaminase Deficiency |
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Definition
| Excess ATP and dATP imbalances nucleotide pool via feedback inhibition of ribonucleotide reductase --> prevents DNA synthesis and thus decreases lymphocyte count; major cause of SCID |
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Term
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Definition
| Defective purine salvage due to absence of HGPRT, which converts hypoxanthine to IMP and guanine to GMP; results in excess uric acid production; findings: retardation, self-mutilation, aggression, hyperuricemia, gout, choreoathetosis |
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Term
| DNA and RNA synthesis direction |
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Definition
| Both DNA and RNA are synthesized 5' -->3'; 5' of the incoming nucleotide has the triphosphate (energy source for bond formation) |
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Term
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Definition
| Immotile cilia due to dynein arm defect; results in male and female infertitlity, bronchoectasis, and recurrent sinusitis; associated w/ sinus inversus |
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Term
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Definition
Type I: Bone, Skin, Tendon
Type II: Cartilage (Car-TWO-lage)
Type III: Reticulum
Type IV: Basement membrane (Four under the floor)
(Be So Totally Cool, Read Books) |
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Term
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Definition
Variety of gene defects resulting in abnormal Type IV collagen.
Characterized by hereditary nephritis and deafness; may be associated w/ ocular disturbances. |
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Term
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Definition
Lack of vitamin C.
Inhibits hydroxylation of specific proline and lysine residues in collagen synthesis. |
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Term
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Definition
Defect in glycosylation of pro-alpha-chain lysine residues and formation of procollagen.
Will cause: multiple fracture w/ from minimal trauma, blue sclerae, hearing loss, dental imperfections. |
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Term
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Definition
Defect in cross-linking of collagen fibrils.
Will cause: hyperextensible skin, tendency to bleed, hypermobile joints.
Type III collagen most affected. |
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Term
Polymerase Chain Reaction
(PCR) |
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Definition
Used to amplify a desired fragment of DNA.
1. Denaturation
2. Annealing
3. Elongation |
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Term
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Definition
| DNA sample is electrophoresed on a gel and then transferred to a filter then denatured and subsequently exposed to a radiolabeled DNA probe that is recognizes and anneals to complementary strand. The resulting double-stranded piece of DNA is visualized when the filter is exposed to film. |
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Term
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Definition
| Radiolabeled DNA probe binding to sample RNA. |
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Term
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Definition
| Sample protein is separated via gel electrophoresis and transferred to a filter. Labeled antibody is used to bind to the relevant protein. |
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Term
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Definition
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