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 Immunodeficiency disease question  



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Author11 Posts
  #1

A 7-year-old boy presents to the clinic with a staphylococcal infection. He is well known at the clinic because he has had recurrent staphylococcal infections for most of his life. He is started on an antibiotic regimen and the infection subsides. Three weeks later, the boy is diagnosed with pruritic papulovesicular dermatitis. Which of the following immune deficiency syndromes would account for this patient’s recurrent staphylococcal infections and pruritic papulovesicular dermatitis?

(A) Ataxia-telangiectasia
(B) Bruton’s agammaglobulinemia
(C) Job’s syndrome
(D) Thymic aplasia
(E) Wiskott-Aldrich syndrome




  #2

the skin lesions point to Job syndrome?


  #3

Ya.It's Job's Syndrome.


  #4

Wiskott-Adrich


  #5

Why cant it be Wiskott Aldrich Syndrome ?


  #6

He would have had symptoms of thrombocytopenia if it were wiskott aldrich.


  #7

I just did a read in Harrison. WAS doesnt always have a triad. In fact, it is very rare to have a classical triad which is written in kaplan LN. So I am really confused. I pasted this question from First Aid Q and A book. I doubt the standard if this book because there were some issues with other questions too. The above question is not wrong but acc to me, its not possible to choose from WAS and Job's.

If someone has better idea and correct me, I will be glad. Thanks a lot.

BTW, in the book, correct answer is given as Job's Syndrome.


  #8

It is true that in WAS very few pts have the triad. However, 90% have thrombocytopenia. Agreed there is some ambiguity, however, the best possible answer is Jobs.


  #9

Thanks !


  #10

anyone can explain the pathophysiology behind this job syndrom? it is totally new to me....is defected cell macrophage or neutrophil,,,CMI or hummoral/?,

it says problem is in low levels of INFgamma ,which cause impaired neutrophil chemotaxis! how IFNgamma affects neut chemotaxis?,,


Edited by doctorforever on Jun 10, 2010 - 9:38 AM

  #11

E- BE CAREFUL IN EXAME NOT NECESSORY GET YOU ALL DIAGNOSTIC CRITERIA USU. GIVE ONE DIRECT OR INDIRECT CLUE





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