Term
|
Definition
| X-linked, eczema, thrombocytopenia, anergy, decreased IgM, increased IgA and IgE |
|
|
Term
|
Definition
| autosomal recessive, sinopulmonary disease, decreased IgA and IgG |
|
|
Term
| Primary T-Cell immunodeficiencies |
|
Definition
Recurrent intracellular pathogens, opportunistic infx, virus, fungi, protozoa, diarrhea, GI infections, growth retardation and FTT
DiGeorge, SCID, Wiskott-Aldrich, Ataxia Telangiectasia, Hyper IgE syndrome |
|
|
Term
| Primary B-Cell immunodeficiencies |
|
Definition
Susceptible to Sinopulmonary disease, encapsulated bacteria and extracellular pathogens, Enterovirus
X-linked agammaglobulinemia, common variable immunodeficiency, hyper-IgM syndrome, Selective IgA deficiency,transient hypogammaglobulinemia of infancy |
|
|
Term
|
Definition
| Kostmann syndrome, Job syndrome, Chediak-Higashi syndrome, chronic granulomatous disease, leukocyte adhesion deficiency |
|
|
Term
|
Definition
Type I: Acute, IgE mediatied
Type II: IgG mediated, cytotoxic, includes autoantibodies
Type III: immune complex, vasculitis
Type IV: Tcell mediated, graft rejection |
|
|
Term
| Chronic granulomatous disease |
|
Definition
X-linked and recessive forms
Defect in cytochrome P-450 NADPH oxidase pathway
Inability to kill catalase producers including S. aureus, deep tissue abscess, osteomyelitis, pneumonia with pneumatoceles, septicemia
Hepatosplenomegaly and granuloma formation
Dx with nitroblue tetrazolium dye |
|
|
Term
|
Definition
| Defective granulocyte function and recurrent staph infection, partial oculocutaneous albinism |
|
|
Term
| Leukocyte adhesion deficiency |
|
Definition
| Recurrent staph infection, lack of pus, delayed separation of umbilical cord |
|
|
