-
Achondroplasia has a defective?
Fibroblast Growth Factor (FGF) receptor
-
TGF-beta has what effect on osteoblasts and osteoclasts?
Increase osteoblast, decrease osteoclasts
-
Insulin-like grwoth factor (IGF-1, IGF-II) has what effect on osteoblasts and osteoclasts?
Increase osteoblast, none on osteoclast
-
Matrix metalloproteinase-13 (MMP-13) is inhibited by?
IGF-I
-
mediates viral immunity and pairs with beta2-microglobulin
MHC I
-
-
HLA-B27
PAIR: Psoriasis, Ankylosing spondylitis, Inflammatory bowel disease, Reiter's syndrome
-
-
HLA-DR2
MS, Hay fever, SLE, Goodpasture's
-
HLA-DR3
Diabetes Mellitus Type 1
-
HLA-DR4
Rheumatoid arthritis, Diabetes Mellitus Type 1
-
HLA-DR5
Pernicious anemia --> B12 deficiency, Hashimoto's thyroiditis
-
HLA-DR7
Steroid-responsive nephrotic syndrome
-
Superantigens from S. pyogenes and S. aureus infect by
cross-link the beta-region of the T-cell receptor to th eMHC Class II on APCs. Results in the uncoordinated relase of IFN-gamma from Th1 cells and subsequence release of IL-1, IL-6, TNF-alpha from macrophages
-
Endotoxins/lipopolysaccharide from gram-negative bacteria exert their effect by:
directly stimulating macrophages by bind to endotoxin receptor CD14. (CD14 are only on macrophages)
-
Proinflammatory agent that induces insulin resistance by activation of serine kinase, which inhibits downstream signaling from macrophages
TNF-alpha
-
Agent important for tissue regeneration & repair from T-cells, platelets, endothelial cells, smooth muscle cells, fibroblasts & keratinocytes
TGF-beta
-
stimulates macrophages and Th1 cells while inhibiting Th2 cells - secreted by antigen presenting cells
IFN-gamma
-
Agent that mediates septic shock, causes leukocyte recruitment, vascular leak and secreted by macrophages
TNF-alpha
-
Hereditary angioedema
Deficiency of C1 esterase inhibitor
-
Deficiency of C5-C8 often leads to?
Neisseria bacteremia
-
Deficiency of C3 leads to?
recurrent pyogenic sinus and respiratory tract infections; increase susceptibility to type III hypersensitivity reactions
-
These agents inhibit biral protein syntheis by inducing the production of ribonuclease and activating NK cells
IFN-alpha & IFN-beta
-
fever, hives, joint pain, proteinuria, lymphadenopahty 5-10 days after antigen exposure (usually a drug)
Serum sickness
-
Local subacute antibody-mediated hypersensitivy characterized by edema, necrosis and activation of complement
Arthus reaction
-
Antinuclear antibodies (ANA)
SLE
-
Anti-dsDNA, anti-Smith
Specific for SLE
-
Antihistone
Drug-induced lupus
-
Anti-IgG
Rheumatoid factor - rheumatoid arthritis
-
anticentromere
Scleroderma (CREST)
-
anti-Scl-70 (anti-DNA topoisomerase I)
Scleroderma (diffuse)
-
Antimitochondrial
primary biliary cirrhosis
-
Anti-basement membrane
Goodpasture's syndrome
-
Anti-desmoglein
Pemphigus vulgaris
-
Antimicrosomal
hashimoto's thyroiditis
-
Antithyroglobulin
Hashimoto's thyroiditis
-
anti-Jo-1
Polymyositis, dermatomyositis
-
Anti-SSA, Anti-SSB
Sjogren's syndrome
-
Anti-U1 RNP
Mixed connective tissue disease
-
Anti-smooth muscle
Autoimmune hepatitis
-
Anti-glutamate decarboxylase
Type 1 diabetes mellitus
-
c-ANCA
Wegener's granulomatosis
-
p-ANCA
other vasculitides
-
B-cell disorders
Bruton's agammaglobulinemia, Hyper-IgM syndrome, Selective Ig Deficiency, Common variable immunodeficiency
-
Defection CD40L on helper T cells with the inability to class-switch B cells
Hyper-IgM syndrome
-
Defective BTK gene that blocks b-cell differentiation/maturation
Bruton's agammaglobulinemia - decrease immunoglobulins in all classes
-
Sinus and lung infections, milk allergies, and diarrhea
Selective Ig deficiency, IgA most common
-
Defective B cell maturation, many causes usually acquired in the 20s-30s, with decrease plasma cells and immunoglobulins even though there are normal B cells
CVID
-
Tetany, recurrent viral/fungal infections, congenital heart and great vessel defects
Thymic aplasia (DiGeorge Syndrome)
-
Decrease Th1 response causing disseminated mycobacterial infections
IL-12 receptor deficiency
-
Th cells fail to produce IFN-gamma --> inability of neutrophils to respond to chemotactic stimuli
Hyper-IgE syndrome
-
FATED: coarse facies, cold staphylococcal abscesses, retained primary teeth, increased IgE, Dermatologic problems (eczema)
Hyper-IgE syndrome (Job's syndrome)
-
Chronic mucocutaenous candidiasis on the skin and mucous membranes
T-cell dysfunction
-
Cerebellar defects, spider angiomas, IgA deficiency
ataxia-telangiectasia
-
Defect in DNA repair enzymes
ataxia-telangiectasia
-
thrombocytopenic purpura, infections, eczema
wiskott-aldrich syndrome
-
X-linked recessive defect with progressive deletion of B and T cells, and decrease in IgM while increases in IgE and IgA
Wiskott-Aldrich syndrome
-
Neutrophilia with recurrent bacterial infections, absent pus formation, and delayed separation of umbilicus
Leukocyte adhesion deficiency (type 1)
-
Defect in LFA-1 integrin protein
Leukocyte adhesion deficiency
-
recurrent pygoenic infections by staphylococci and streptococcil partial albinism, and peripheral neuropathy
Chediak-Higashi syndrome
-
AR defect in microtubular function with decrease phagocytosis
Chediak-Higashi syndrome
-
Increase susceptibility to catalase-positive organisms (S. aureus, E.coli, Aspergillus)
Chronic granulomatous Disease
-
Negative Nitroblue tetrazolium dye reduction test indicates:
Chronic granulomatous disease
-
Which immunosuppressant binds to cyclophilins?
Cyclsporine
-
Which immunosuppressant blocks the differentiation and activation of T cells by inhibiting calcineurin, thus preventing the production of IL-2 and its receptor?
Cyclosporine
-
Which immunosuppressant binds to FK-binding protein and inhibits the secretion of IL-2
Tacrolimus
-
Nephrotoxicity, peripheral neuropahty, hypertension, pleural effusion and hyperglycemia are all side effects of:
Tacrolimus
-
What immunosuppressant is an antimetabolic precursor of 6-MP that interferes with the metabolism and synthesis of nucleic acids?
azathioprine
-
Which immunosuppressants toxic effects can be increased by allopurinol?
Azathiprine
-
Which monoclonal antibody binds to CD3?
Muromonab
-
Which binds to mTOR and inhibits response to IL2?
Sirolimus (rapamycin)
-
What inhibits de novo guanine synthesis and blocks lymphocyte production?
Mycophenolate mofetil
-
What monoclonal antibody has a high affinity for the IL2 receptor on activated T-cells?
Daclizumab
-
Aldesleukin (IL-2)
- renal cell carcinoma
- metastatic melanoma
-
Gilgrastim (GCSF)
recovery of bone marrow
-
Sargramostim (GMCSF)
recovery of bone marrow
-
alpha interferon
- hepatitis B and C
- Kaposi's sarcoma
- leukemias
- malignant melanoma
-
-
gamma interferon
Chronic granulomatous disease
-
Oprelvekin (IL-11)
Thrombocytopenia
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