20 Questions
Which of the following immunodeficiencies is X-linked and typically diagnosed around 6 months of age?
Bruton’s (X-linked agammaglobulinemia)
Which immunodeficiency is characterized by an absent IgG response to vaccination?
Bruton’s
Which of the following immunodeficiencies is typically diagnosed in a teen or older child and presents with a decreased response to vaccination?
Common Variable Immunodeficiency (CVID)
What is a key 'tip-off' for Bruton’s immunodeficiency?
Absent tonsils
Which of the following immunodeficiencies is characterized by a decreased number of B cells?
Bruton’s
Which immunodeficiency is characterized by a decreased number of B cells and absent tonsils?
Bruton’s
Which of the following immunodeficiencies is characterized by a decreased number of B cells but not by absent tonsils?
Common Variable Immunodeficiency (CVID)
Which immunodeficiency is characterized by a decreased number of B cells and a decreased response to vaccination?
Common Variable Immunodeficiency (CVID)
Which immunodeficiency is characterized by a decreased number of B cells and recurrent sinopulmonary and GI illnesses?
Bruton’s
Which immunodeficiency is characterized by a decreased number of B cells and persistent diarrhea or sinusitis?
Bruton’s
What is the underlying defect that leads to the high levels of IgM in Hyper-IgM patients?
Deficient expression of CD40L on helper T cells
What is the characteristic presentation of a patient with Hyper-IgE?
Atopy symptoms, such as eczema and cold abscesses
What is the primary treatment for a patient with IgA deficiency?
Removing IgA from all future blood transfusions
What is the underlying cause of Severe Combined Immunodeficiency (SCID)?
A deficiency in both B and T cells
What is the characteristic presentation of a child with DiGeorge Syndrome?
Recurrent otitis media and learning delay
What is the underlying defect that leads to impaired chemotaxis in neutrophils of Hyper-IgE patients?
A defect in the JAK-STAT signaling pathway in neutrophils
What is the primary treatment for a patient with Severe Combined Immunodeficiency (SCID)?
IVIG to thymic transplant
What is the underlying cause of a patient with a normal B cell count and a decrease in 1/3 of all immunoglobulins?
IgA deficiency
What is the characteristic presentation of a patient with Hyper-IgM?
Severe pyogenic infections
What is the primary treatment for a patient with DiGeorge Syndrome?
IVIG to thymic transplant
Challenge your knowledge on childhood immunodeficiencies with critical questions focusing on age of presentation, key symptoms, causes, diagnostic tests, and treatment options. Explore diseases like Bruton's agammaglobulinemia and Common Variable Immunodeficiency (CVID).
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