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Flashcards in Immune Deficiency Questions Deck (5)
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A two month old boy is brought to your emergency department with respiratory distress, tachypnea, and hypoxia. The child is noted to have a dry scaly rash and is less than 5th percentile for weight. Chest x-ray shows bilateral hazy infiltrates and an absent thymus.

What is the best test to evaluate this patient's diagnosis?

A: CH50 with quanititative C3 and C4
B: a1-antitrypsin level
C: Quantitiative IgG sybtypes
D: Flow cytometry for T and B cells
E: Flow cytometry for CD11 and CD18

D: This is SCID so you want to look for presence of T and B cells.

CD11 and CD18 are associated with LAD, not SCID.


A four-year-old boy comes to your clinic with a history of eczema, multiple admissions for pneumonia, and the history of easy bruising. What test could help you confirm the diagnosis?

A: Dihydrorhodamine test
B: Small platelets on peripheral smear
C: C1Q Inh levels and function
D: Diphtheria vaccine titers

B because this is Wiskott-Aldrich.

A: Chronic granulomatous disease
B: Wiskott-Aldrich
C: Hereditary angioedema
D: B-cell deficiency such as CVID, transient hypogammaglobulinemia of infancy, IgG subclass deficiency, or more seriously, Bruton's agammaglobulinemia


A four-year-old boy comes to your clinic with a history of frequent falls, recurrent pneumonias requiring multiple admissions, as well as an elevated alpha-fetoprotein. What is the most likely diagnosis?

A: Wiscott-Aldrich Bombay variant
B: ataxia telangiectasia
C: lymphocyte adhesion defects type II
D: Duncan's syndrome

B: ataxia telangiectasia

Duncan's syndrome is another name for X-linked lymphoproliferative disease. This is the one where EBV wreaks havoc.


An eight month old boy comes to be evaluated for a history of recurrent otitis media and pneumonia and a family history of an uncle with a similar problem who died at about one year of age. Which of the following tests should be done next in the workup?

A: Quantitative immunoglobulins
B: Flow cytometry for T cells
D: Chest x-ray to evaluate for the presence of a thymus

A: Quantitative immunoglobulins.

He has x-linked agammaglobulinemia.


A four month old boy is admitted to the pediatric intensive care unit for pneumocystis jiroveci pneumonia. the patient is 75th percentile for height and weight. Initial workup shows absent IgG, IgA, but normal IgM levels. What is the most likely diagnosis?

C: Specific antibody deficiency
D: Hyper-IgM syndrome
E: X-linked agammaglobulinemia

D: Hyper-IgM syndrome