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 (D)</p> Signup and view all the answers

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

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

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

<p>Bruton’s (D)</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) (B)</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) (C)</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 (D)</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 (B)</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 (D)</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 (D)</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 (B)</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 (C)</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 (C)</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 (C)</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 (D)</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 (C)</p> Signup and view all the answers

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

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

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

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

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