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Immunodeficiency disorders
Immunodeficiency disorder quiz
33
Immunology
Professional
09/20/2011

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Term
Erythroblastosis Fetalis
Definition
Rh mismatch between mother and fetus leads to a Type II hypersensitivity hemolytic anemia in the fetus. Hepatosplenomegaly, elevated bilirubin, petechiae
Term
Arthus reaction
Definition
Localized Type III hypersensitivity
Term
Celiac disease
Definition
Type IV hypersensitivity to gliadin leading to villous atrophy in the small bowel and malabsorption
Term
Myasthenia gravis
Definition
autoreactive B and T cells destroy nicotinic acetylcholine receptors in the neuromuscular junction
Term
Graves' Disease
Definition
anti-TSH receptor antibodies causes hyperthyroidism
Term
Thrombocytopenic purpura
Definition
anti-platelet antibodies cause bruising and hemorrhage
Term
Goodpasture syndrome
Definition
antibodies against the kidneys leads to glomerulonephritis, a Type II hypersensitivity reaction
Term
Type I diabetes
Definition
Type IV destruction of the beta islet cells of the pancreas leads to inability to produce insulin. May be precipitated by a Coxsackie viral infection.
Term
Pemphigus vulgaris
Definition
Type II hypersensitivity against epidermal cadherin leads to a blistering skin rash
Term
Anti-Ro antibodies
Definition
anti-nuclear autoantibodies implicated in lupus and Sjogren's syndrome
Term
Guillain-Barre Syndrome
Definition
lgM, IgA, IgG1 and IgG3 produced against peripheral nerve gangliosides. May be caused by antigenic cross reactivity of C. jejuni lipooligosaccharides, Mycoplasma pneumoniae, EBV, and CMV
Term
Ankylosing spondylitis
Definition
affects more men than women, certain HLA B27 alleles increase risk
Term
Acute rheumatic fever
Definition
Type II hypersensitivity initiated by Strep infections that attacks cardiac muscle
Term
Rheumatoid arthritis
Definition
Type III and IV hypersensitivity mediated by IgG-rheumatoid factor complexes
Term
Systemic lupus erythematosus
Definition
Type III hypersensitivity mediated by anti-dsDNA antibodies
Term
Multiple sclerosis
Definition
Type IV hypersensitivity against myelin basic protein
Term
Hashimoto's Thyroiditis
Definition
antibodies against anti-thyroglobulin, anti-microsomal antibodies
Term
Raynaud's phenomena
Definition
livedo reticularis, a lacy patterned rash that is associated with SLE and scleroderma
Term
Asthma
Definition
Type I hypersensitivity reaction leading to bronchial constriction
Term
Hay fever
Definition
Type I hypersensitivity reaction leading to edema of the nasal conjunctiva
Term
Scleroderma
Definition
systemic autoimmune disease caused by anti-centromere antibodies
Term
Bruton's Agammaglobulinemia
Definition
Defective tyrosine kinase gene blocks B-cell maturation, causing failure of opsonization and recurrent bacterial infection. X-linked inheritance.
Term
C3 Deficiency
Definition
Characterized by recurrent Gram+ infections e.g. staphylococcus, increased susceptibility to Type III Hypersensitivity Reactions
Term
Chronic Granulomatous disease
Definition
Lack of NADPH oxidase, few reactive species, no respiratory burst. Susceptibility to catalase-positive infection.
Term
Chronic Mucocutaneous candidiasis
Definition
T-cell dysfunction causes pathognomic skin infection
Term
Common Severe Immune Deficiency
Definition
Multiple recurrent infections. IL-2 Receptor downregulated in X-linked disease.
Term
Common Variable Immune Deficiency
Definition
Defects in B-cell maturation, increased risk of AI disease and lymphoma.
Term
DiGeorge Syndrome
Definition
22q11 deletion and failure of the 3rd and 4th pharyngeal pouches to develop, recurrent viral and fungal infections, thymic aplasia.
Term
Hereditary Angioedema
Definition
Caused by C1 Inhibitor Deficiency
Term
IL-12 Deficiency
Definition
Defect in Th1 response, decreased IFN-gamma, disseminated mycobacterial infection.
Term
Late Complement Deficiency
Definition
Characterized by recurrent Gram- infections e.g. neisseria
Term
Leukocyte Adhesion Deficiency (Type 1)
Definition
Defect in CD18, delayed separation of umbilicus, neutrophilia and recurrent intracellular infection.
Term
Neutropenia and Hyposplenism
Definition
Quantitative defects in phagocytic cells
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