Diaggnosis and Management of Patients w/ Primary Immuno deficiency Disorders
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| What is the most important factor for eventually making a diagnosis of primary immunodeficiency? | Suspicion of an immunodeficiency
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| Do most primary immunodeficiency pt present with catastrophic infections? | No, except for infants with SCID
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| cutaneous manifestions of primary immunodeficiency are typically representational of what form? | T cell deficiency
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| respiratory and GI tract manifestions of primary immunodeficiency are typically representational of what form? | B cell deficiency
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| Do individuals with primary immunodeficiency require a more intensive treatment for infections? | No, regardless of cause, treatment is usually conventional (exceptions exist)
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| What type of response is typical of individuals with primary immunodeficiency | only a partial response with the exception being those with an IgA deficiency
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| 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%]
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| Is HIV a form of primary immunodeficiency? | No it is a secondary cause
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| The majority of immunodeficiency diseases are _________ problems | antibody
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| Maternal antibodies are what type(s)? | IgG only
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| Production of adult antibodies and the normal adult level occur in what age range? | 14-18 years old
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| What type of immunoglobulin is most commonly deficient? | IgA
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| 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
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| How should you treat someone with an IgA deficiency? | conservatively, treat symptoms/infections, nothing else to do
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| What Primary Immune Deficiency is associated with an increased incidence of autoimmune disorders? | IgA
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| 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
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| 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
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| What are the primary symptoms of SCID? | failure to thrive and severe diarrhea
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| 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
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| The adenoids are also commonly known as _________ | pharyngeal tonsils, or nasopharyngeal tonsils
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