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  #1  
Old 10-09-2011
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Question Recurrent sinusitis + viral respiratory infection in a boy

An 8-year-old white male is brought to your office by his parents, who are concerned about the number of colds he has had. Your chart review indicates that he has had approximately 15 URI’s each year. Most were viral, but he has also had several episodes of sinusitis. Family history reveals that the mother has similar problems and has recently been diagnosed with rheumatoid arthritis. The most likely immune deficiency in this child is?

A) Bruton’s panhypogammaglobulinemia
B) DiGeorge’s syndrome
C) Wiskott-Aldrich syndrome
D) Combined immunodeficiency
E) selective IgA deficiency

Please answer it with explanation!
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Old 10-09-2011
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I'll go with B) DiGeorge’s syndrome

Reason: Thymic aplasia will have dec T cell maturation (as we know T cell matures at Thymus). So less cytotoxic T cells --> which are good against virus. So more viral inf.

SCID is also a good option but SCID are usually present with all viral, bacterial, fungal and protozoal.
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D) Combined immunodeficiency
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The question makes it seem like both the mother and the child have the same issue. If that's the case then I think Selective IgA is the best answer. If they both have SCID then it doesnt make sense the mother is producing anti-IgG antibodies (Rheumatoid arthritis).

E)selective IgA
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d) combined ID
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E)selective IgA
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Its B - DiGeorge Syndrome.
There is absent T-cell maturation, so there will be no T-cell mediated activation of NK cells and Cytotoxic T-cells, which kills the virus infected cells!
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B. Di george syndrome decrease T cell result in increase in infections of viral,fungal and protozoal
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selective IgA deficiency is the most common primary immune deficiency affecting 1:600 northern europeans. In most patients low or undetectable IgA is an incidental finding. 30 percent have recurrent mild respiratory and gastrointestinal infection. There is also a compensatory increase in serum IgG levels
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Sel.IgA...clue repeated URI,s and SINUSITIS....IgA location at mucousal surfaces.
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You know this is a good question when the responses are in a 3 way split! Whats the answer to this one im really curious now.
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Correct Answer Answer

Quote:
Originally Posted by moe2185 View Post
You know this is a good question when the responses are in a 3 way split! Whats the answer to this one im really curious now.

ANSWER: E

Selective IgA deficiency is the most common primary immunodeficiency, occurring in up to 2% of the population. Although some patients may be asymptomatic, many are bothered by recurrent respiratory infections, chronic diarrheal syndromes, and a tendency to develop autoimmune diseases such as lupus erythromatosis or rheumatiod arthritis. The deficiency can be transmitted in an autosomal dominant pattern. The other immunodeficiencies listed are much less common, have other distinguishing characteristics, and are usually much more severe in nature
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