IM Exam Flashcards
(40 cards)
3 functions of antibodies
opsonization, complement activation, neutralization
presentation of antibody primary immunodeficiencies
recurrent encapsulated bacterial infections, especially sinus and pulmonary
average age of onset of common variable immunodeficiency
20s
characteristics of CVID
hypogammaglobulinemia, poor response to vaccines, increased risk of autoimmunity and malignancy
selective IgA deficiency characteristics
may be asymptomatic, normal IgG and IgM levels, normal response to vaccines, increased sinusitis/respiratory infections, GI infections, autoimmunity and allergies,
mechanism of XLA
mutation or absence of Bruton’s tyrosine kinase gene, causing arrest of early B-cell development
XLA characteristics
onset of recurrent bacterial infections at 1 year, deficiency of all serum immunoglobulins
characteristics of primary cellular immunity deficiencies
more severe than humoral, overwhelming viral/fungal infections, present with failure to thrive and disseminated infection
mechanism of diGeorge syndrome
deletion in 22q11 causing thymic aplasia and subsequent T-cell deficiency
SCID characteristics
severe, opportunistic infections, diarrhea, and failure to thrive due to deficiency in T-cells and B-cells
SCID inheritance
can be X-linked or autosomal recessive
Wiscott-Aldrich syndrome inheritance
X-linked
Wiscott-Aldrich syndrome characteristics
thrombocytopenia, small/dysfunctional platelets, eczema, susceptibility to infections
Ankylosing spondylitis is associated with which HLA allele
B27
chronic granulomatous disease characteristics
phagocytic primary immunodeficiency, more common in males, common death by aspergillus infection
chronic granulomatous disease mechanism
deficiency of an element of phagocytes causes decreased ability to eliminate extracellular pathogens
patients with defects in alternative complement pathway commonly present with what type of infection
Neisseria
primary immunodeficiency treatments
humoral: IVIG, cellular: Bone marrow transplant, both: prophylactic antibiotics
complement deficiency infections
encapsulated bacteria
common infections due to asplenia
H. influenzae, strep pneumoniae
asplenia characteristics
encapsulated infections, normal response to vaccines, abx prophylaxis
What is Chediak-Higashi syndrome
disorder of innate immunity
what is common to all connective tissue disorders
synovitis
test for dermatomyositis/polymyositis
anti-Jo antibodies
