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B2 CCC 1 - 12/7 Dr.Z

Diaggnosis and Management of Patients w/ Primary Immuno deficiency Disorders

What is the most important factor for eventually making a diagnosis of primary immunodeficiency? Suspicion of an immunodeficiency
Do most primary immunodeficiency pt present with catastrophic infections? No, except for infants with SCID
cutaneous manifestions of primary immunodeficiency are typically representational of what form? T cell deficiency
respiratory and GI tract manifestions of primary immunodeficiency are typically representational of what form? B cell deficiency
Do individuals with primary immunodeficiency require a more intensive treatment for infections? No, regardless of cause, treatment is usually conventional (exceptions exist)
What type of response is typical of individuals with primary immunodeficiency only a partial response with the exception being those with an IgA deficiency
What are the diffrent types of primary immunodeficiency and what is the order of their prevalence? 1.Antibody (Humoral/B-cell) deficiency [50%] 2.Cell-mediated (T-cell) disorders [20%] 3.Severe combined (B & T) immunodeficiency [20%] 4.Phagocytic disorders [10%] 5.Complement disorders [<1%]
Is HIV a form of primary immunodeficiency? No it is a secondary cause
The majority of immunodeficiency diseases are _________ problems antibody
Maternal antibodies are what type(s)? IgG only
Production of adult antibodies and the normal adult level occur in what age range? 14-18 years old
What type of immunoglobulin is most commonly deficient? IgA
How do individuals with IgA deficiency typically present? They are generally asymptomatic, associated with mild respiratory infections, increased UTI's and diarrhea. They are dx by low IgA
How should you treat someone with an IgA deficiency? conservatively, treat symptoms/infections, nothing else to do
What Primary Immune Deficiency is associated with an increased incidence of autoimmune disorders? IgA
What primary immunodeficiency is often fatal by the age of 1, and what is the typical treatment SCID (Severe Combined Immunodeficiency), and it is treated with a bone marrow transplant
Is Severe Combined Immunodeficiency rare? Yes. Though combined T-cell and B-cell deficiency make up 20% of the primary immunodeficiency cases, SCID is a severe subset of this group
What are the primary symptoms of SCID? failure to thrive and severe diarrhea
In radiological imaging what can absent adenoids or other lymph tissue possible indicate about an individual that they may have B and T cell deficiency
The adenoids are also commonly known as _________ pharyngeal tonsils, or nasopharyngeal tonsils
Created by: VCOM2013
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