rheum.txt

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dewarsb
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212791
Filename:
rheum.txt
Updated:
2013-04-18 15:14:47
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dewarsb Boardreview rheum allergy immunology
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Allergy, immunology, rheumatology
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  1. Onset within minutes
    cross-linking of IgE on mast cells
    anaphylaxis, angioedema, urticaria
    treat with epinephrine and steroids
    Type I Hypersensitivity
  2. Onset within hours
    cytotoxic antibody
    hemolytic anemia
    Type II Hypersensitivity
  3. Onset within hours
    Immune complex deposition with activation of complement
    Serum sickness (fever, urticaria, arthritis)
    Low C3, C4, proteinuria
    treat with antihistamines, NSAIDs or prednisone
    Type III Hypersensitivity
  4. Onset within days
    T-cell mediated
    Contact dermatitis
    Type IV Hypersensitivity
  5. FPIES
    bloody diarrhea or profuse diarrhea
    usually due to soy or milk protein
    diagnose with double blind placebo-controlled food challenge
    Food protein-induced enterocolitis syndrome
  6. Vaccines to avoid if egg allergic
    • Influenza
    • Yellow fever
  7. 4 Allergic causes of eosinophilia
    • allergic rhinits
    • eczema
    • asthma
    • drug allergy
  8. Endocrine causes of eosinophilia
    adrenal insufficiency
  9. 3. Infectious causes of eosinophilia
    • parasitic
    • mycobacterial
    • fungal
  10. 5 Immune dysfunction causes of eosinophilia
    • IBD
    • SLE
    • Churg-Strauss syndrome
    • Omenn syndrome
    • hyper-IgI syndrome
  11. 4 Neoplastic causes of eosinophilia
    • AML
    • ALL
    • lymphoma
    • mastocytoma
  12. 3 Idiopathic causes of eosinophilia
    • hypereosinophilic syndrome
    • eosinophilic pneumonia
    • eosinophilic gastroenteritis
  13. Endomyocardial fibrosis secondary to the toxic effects of eosinophilic granules on the heart
    Loffler's endocarditis
  14. Congenital abscence of B cells
    commonly X linked
    very low IgA, IgM and IgG
    absent vaccine responses
    pyogenic bacterial and enteroviral infections after six months of age
    Bruton's agammaglobulinemia
  15. acquired disorder of antibody production
    unkonwn mechanism
    Low IgA, IgG or IgM
    absent vaccine responses
    B cells are present
    presents after age 2 with severe infections
    associated with autoimmune disease and lymphoma
    Common variable immunodeficiency CVID
  16. Most commonly inherited immunodeficiency
    unknown mechanisms
    may have relatives with CVID
    Isolated low IgA
    Minor infections or asymptomatic
    associated with celiac disease
    IgA deficiency
  17. Slow to develop normal levels of antibodies
    Low IgA, IgG, or IgM
    present vaccine responses
    minor infections or asymptomatic
    resolves by four years of age
    Transient hypogammablobulinemia of infancy
  18. presents in infancy, FTT
    both cellular and humoral deficiency
    low antibody levels
    abscent vaccine responses
    opportunistic infections
    require bone marrow transplant
    IVIG until transplant
    Severe Combined Immunodeficiency
  19. thymic hypoplasia
    association with conotruncal anomalies
    dysmorphic face, cleft palate and hypoparathyroidism
    22q deletions
    DiGeorge syndrome
  20. X-linked
    eczema
    thrombocytopenia
    severe infections
    Wiskott-Aldrich syndrome
  21. DNA breakage repair deficiency
    affects T and B cell gene rearrangement
    ataxia, telangiectasias, leukemia, lymphona, immunodeficiency
    Ataxia-telangiectasia
  22. neutrophils lack NADPH oxidase
    NBT or DHR fluorescence test
    NOBA neutrophil oxidative burst activation test
    skin and organ abscesses
    Chronic granulomatous disease
  23. neutrophils cannot migrate out of vessels
    high neutrophil count
    abscesses without pus
    Leukocyte adhesion deficiency
  24. abnormal phagosome trafficking
    giant granules in PMNs
    partial albinism
    Chediak-Higashi syndrome
  25. mutations to elastase gene ELA2
    neutropenia in cycles of 3 weeks
    stomatitis, OM, abscesses
    Cyclic neutropenia
  26. ELA2 and HAX1 mutations
    low absolute neutrophil count
    stomatitis, OM, abscesses, pneumonias
    Kostmann's syndrome (congenital neutropenia)
  27. SBDS gene mutations
    ineffective hematopoiesis
    neutropenia
    fat malabsorption due to pancreatic insufficiency
    respiratory infections, diarrhea, FTT, metaphyseal dysplasia
    Shwachman-Diamond syndrome
  28. a failure of lymphocyte apoptosis
    lymphadenopathy
    splenomegaly
    anemia
    thrombocytopenia
    Autoimmune lymphoproliferative syndrome ALPS
  29. staphylococcal microabscesses
    pneumonia with pneumatoceles
    coarse facies
    dermatitis
    markedly increased IgE
    Hyper-IgE syndrome (Job syndrome)
  30. dry eyes, dry mouth, recurrent parotitis
    positive ANA, anti Ro, anti La, RF and IgG
    increased risk of JIA, SLE, scleroderma, thyroid, PN, lymphoma
    Sjogrens syndrome
  31. regular fevers, abdominal pain
    occaisional pericarditis, pleuritis and arthitis
    long-standing inflammation leads to amyloidosis
    treat with prophylactic colchicine
    Familial Mediterranean Fever
  32. fevers every 4-8 weeks lasting 4-6 days
    aphthous ulcers
    lymphadenopathy
    pharyngitis
    tonsillitis
    • PFAPA
    • Periodic Fever with Aphthous Stomatitis, PHaryngitis, and Adenitis
  33. oral ulcers
    genital ulcers
    eye disease
    Pathergy test-pustules form at needlestick sites
    Behcet's Disease

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