Childhood Immunodeficiencies Critical Questions
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Questions and Answers

Which of the following immunodeficiencies is X-linked and typically diagnosed around 6 months of age?

  • Hyper-IgM
  • Hyper-IgE
  • Common Variable Immunodeficiency (CVID)
  • Bruton’s (X-linked agammaglobulinemia) (correct)

    • Which immunodeficiency is characterized by an absent IgG response to vaccination?

  • IgA Deficiency
  • Hyper-IgE
  • Hyper-IgM
  • Bruton’s (correct)

    • Which of the following immunodeficiencies is typically diagnosed in a teen or older child and presents with a decreased response to vaccination?

  • IgA Deficiency
  • Hyper-IgM
  • Hyper-IgE
  • Common Variable Immunodeficiency (CVID) (correct)

    • What is a key 'tip-off' for Bruton’s immunodeficiency?

    <p>Absent tonsils</p> Signup and view all the answers

    Which of the following immunodeficiencies is characterized by a decreased number of B cells?

    <p>Bruton’s</p> Signup and view all the answers

    Which immunodeficiency is characterized by a decreased number of B cells and absent tonsils?

    <p>Bruton’s</p> Signup and view all the answers

    Which of the following immunodeficiencies is characterized by a decreased number of B cells but not by absent tonsils?

    <p>Common Variable Immunodeficiency (CVID)</p> Signup and view all the answers

    Which immunodeficiency is characterized by a decreased number of B cells and a decreased response to vaccination?

    <p>Common Variable Immunodeficiency (CVID)</p> Signup and view all the answers

    Which immunodeficiency is characterized by a decreased number of B cells and recurrent sinopulmonary and GI illnesses?

    <p>Bruton’s</p> Signup and view all the answers

    Which immunodeficiency is characterized by a decreased number of B cells and persistent diarrhea or sinusitis?

    <p>Bruton’s</p> Signup and view all the answers

    What is the underlying defect that leads to the high levels of IgM in Hyper-IgM patients?

    <p>Deficient expression of CD40L on helper T cells</p> Signup and view all the answers

    What is the characteristic presentation of a patient with Hyper-IgE?

    <p>Atopy symptoms, such as eczema and cold abscesses</p> Signup and view all the answers

    What is the primary treatment for a patient with IgA deficiency?

    <p>Removing IgA from all future blood transfusions</p> Signup and view all the answers

    What is the underlying cause of Severe Combined Immunodeficiency (SCID)?

    <p>A deficiency in both B and T cells</p> Signup and view all the answers

    What is the characteristic presentation of a child with DiGeorge Syndrome?

    <p>Recurrent otitis media and learning delay</p> Signup and view all the answers

    What is the underlying defect that leads to impaired chemotaxis in neutrophils of Hyper-IgE patients?

    <p>A defect in the JAK-STAT signaling pathway in neutrophils</p> Signup and view all the answers

    What is the primary treatment for a patient with Severe Combined Immunodeficiency (SCID)?

    <p>IVIG to thymic transplant</p> Signup and view all the answers

    What is the underlying cause of a patient with a normal B cell count and a decrease in 1/3 of all immunoglobulins?

    <p>IgA deficiency</p> Signup and view all the answers

    What is the characteristic presentation of a patient with Hyper-IgM?

    <p>Severe pyogenic infections</p> Signup and view all the answers

    What is the primary treatment for a patient with DiGeorge Syndrome?

    <p>IVIG to thymic transplant</p> Signup and view all the answers

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