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Immunodeficiency
First Aid Immunodeficiencies
14
Science
Professional
05/19/2010

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Cards

Term
Severe Combined Immunodeficiency
Definition
Defect: IL-2 receptor deficiency(x-linked; the cause 80% of the time)
adenosine deaminase deficiency
Failure ot synthesize MHC II antigens

Presentation: recurrent, VIRAL, BACTERIAL, & FUNGAL infections.

Treatment: bone marrow transplantation. There is no possibility of graft rejection
Term
Hyper IgE(Job's Syndrome)
Definition
Defect: Th1 cells fail to produce inf-gamma leading to inability of neutrophils to resod ot chemotatic stimuli

Presentation: FATED
coarse FACES
cold staph ABSCESSES
retained PRIMARY TEETH
hyper IGE
DERMATOLOGIC problems
Term
Chronic Granulomatous Disease
Definition
Defect: Lack of NADPH oxidase-->decrease in reactive oxygen species.

Presentation: susceptible to catalse positive organisms
Staph, E. coli, aspergillus

Lab: Nitroblue tetrazolium test
Term
Bruton's agammaglobulinemia
Definition
Defect: BTK(tyrosine kinase)-->blocks B-cell maturation

Presentation: recurrent bacterial infections after 6 months. DECREASED number of B cells
Term
Hyper IgM syndrome
Definition
Defect: CD40L on T-helper cells cannot interact with B-cell and force a class switch.

Presentation: Severe pyogenic infections early in life
Term
Selective Ig deficiency
Definition
Most common is IgA deficiency. Severe anaphylactic reaction upon exposure to IgA containing blood products
Term
Common Variable immunodeficiency
Definition
Defect: B-cell immaturity, many causes

Presentation: The occurance of immundeficiency presenting in the 20-30's. NORMAL number of B cells.
Term
Thymic aplasia(DiGeorge syndrome)
Definition
Defect: 22q11 deletion

Presentation: Tetany, recurrent VIRAL & FUNGAL infections, congential heart and great vessel defects
Term
IL-12 deficiency
Definition
Defect: inability to produce IL-12. Therefore cannot stimulate Th1 cells to produce inf-gamma.

Presentation: Lack to cytotoxic t-cell response leads to disseminated mycobacterial infections
Term
Chronic mucocutaneous candidiasis
Definition
T-cell dysfunction. Candida albicians infection of skin and mucous membranes
Term
Ataxia-telangiectasia
Definition
Defect in DNA repair enzymes

Presntation: Triad
cerebellar defects(Ataxia)
spider angiomas(telangiectasia)
IgA defiency
Term
Wiskott-Aldrich Syndrome
Definition
Defect: X-linked defect causing progressive deletion of B and T cells

Presentation: TIE
Thrombocytopenia purpura
Infections
Eczema

Labs: decreased IgM increased IgE IgA
Term
Leukocyte adhesion deficiency
Definition
Defect: missing LFA-1(CD-18) on PHAGOCYTES

Presentation: ABSCENT PUS, delayed umbilicus seperations
Term
Chediak-Higashi syndrome
Definition
Defect: microtubular funciton with decreased phagocytosis

Presentation: pyogenic infections, intracellular inclusions, PARTIAL ALBINISM AND PERIPHERAL NEUROPATHY
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