Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
14 Cards in this Set
- Front
- Back
|
x-linked recessive defect un tyrosine kinase gene assoc with low levels of all Ig classes. > after 6 months in bacterial infections (maternal IgG declines), mostly in BOYS
|
Bruton's agammaglobulinemia - Decreased PRODUCTION B cells
|
|
|
Presents with tetany from HYPOCALCEMIA. Viral and fungal infs from T CELL DEFICIT.
Thymus and parathyroid failure to dev - failure of 3rd and 4th pharyngeal pouches to form. Congenital defects of heart and great vessels. 22q11 del. |
DiGeorge Syndrome - decreased PRODUCTION of T cells - Thymic aplasia
|
|
|
Prob in early stem cell differemtiation. recurrent bact, viral, fungal, protozoan infections. may have multiple causes (failure to synth MHC II antigens, defective IL2 receptors, adenosine deaminase deficiency)
|
SCID - B and T cells decreased PRODUCTION
|
|
|
disseminated mycobacterial infections
|
Decreased ACTIVATION of T cells - IL-12 receptor deficiency
|
|
|
Early severe pyogenic infections.
High IgM -- Low everything else. Defective CD40L on CD4 T-helpers so cannot class switch. |
Hyper IgM syndrome - decreased ACTIVATION of B cells
|
|
|
Triad of symps:
1. recurrent pyogenic Infecs 2. Thrombocytopenic purpura 3. Eczema x-linked. Not able to mount IgM response to capsular polysaccs of bacteria. High IgA. NORMAL IgE. Low IgM. |
Wiskott-Aldrich - decreased ACTIVATION of B cells
|
|
|
Recurrent "cold"/non-inflamed staph abcesses, eczema, course facies, retained primary teeth.
HIGH IgE. Failure of gamma interferon production by T helpers. |
Job's syndrome - decreased ACTIVATION of macrophages.
|
|
|
Early severe pyogenic and fungal infections and DELAYED SEPARATION OF UMBILICUS.
Defect in LFA-1 adhesion proteins on phagocytes. |
PHAGOCYTIC CELL DEFICIENCY -
Leukocyte adhesion deficiency syndrome |
|
|
Presents recurrent pyogenic staph and strep, PARTIAL ALBINISM, peripheral neuropathy.
Autosomal Recessive. Defect in MICROTUBULAR FUNCTION AND LYSOSOMAL EMPTYING OF PHAGOCYTES. |
PHAGOCYTIC CELL DEFICIENCY -
Chediak-Higashi dz |
|
|
Marked suscepitibility to opport. infecs with bacteria - esp S. aureusm E. coli, and Aspergillus. Dx with NEGATIVE nitroblue tetrazolium dye reduction test - lack of NADPH oxidase activity in PMNs.
|
PHAGOCYTIC CELL DEFICIENCY -
Chronic granulomatous disease |
|
|
Candida - skin and mucous membranes. T cell dysfunc against Candida specifically.
|
IDIOPATHIC DYSFUNCTION OF:
T Cells. Chronic Mucocutaneous Candidiasis |
|
|
Sinus and lung infections, milk allergies and diarrhea common. Deficiency in one specific Ig class - IgA def is most common. Could be due to defect in isotype switching.
|
IDIOPATHIC DYSFUNCTION OF:
B Cells. Selective Immunoglobulin Deficiency |
|
|
Presents as cerebellar problems (ataxia) and spider angiomas (telangiectasia). Defect in DNA repair enzymes with associated IgA deficit.
|
IDIOPATHIC DYSFUNCTION OF:
B cells. Ataxia-telangiectasia |
|
|
Acquired in 20-30's.
NORMAL B cell numbers circulating. LOW PLASMA CELLS LOW Ig |
IDIOPATHIC DYSFUNCTION OF:
B cells. Common variable immunodeficiency |
