drduck
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A 24 yr old woman with a histrory of allergic rhinitis is involved in an automobile accident and sustains a spleenic laceration. She undergoesabdominal surgery and is then transfused with 4 units of blood of the appropriate ABO and Rh type. As the transfusion progresses, she becomes rapidly hypotensive and develops airway edema, consistent with anaphylaxis. which of the following pre existing conditions best accounts for these symptoms??
AIDS
C1 esterase inhibitor deficiency
di george sydrome
selective IgA deficiency
Wiscott Aldrich syndrome
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| unique1
InGodITrust
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CI esterase inhibitor deficiency
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| new_n_lost
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selective IgA deficiency
Agree Lidocaine is the MC precipitant in Hereditary Angioedema but the same thing happens in Selective IgA deficiency.
Pt's past history is not suggestive of H. Angioedema but rather of Selective IgA def. cos thats where u have Allergic Rhinitis and most fo the pts r asymptomatic until they r transfused with blood.
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| drduck
Forum Guru
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BANG!!!!
yup.....its selective IgA def....
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| drduck
Forum Guru
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no need for explaination..........most important point was no prior H/O swelling of body parts......or srtridor......
as HA......has to be RECURRENT.....
good job NNL....
even i went for C1 est......for the first time.....
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| rock
Forum Elite
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good question. even i went for B. will be more careful now
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| usmlefever
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the most important clue is the histiry of allergic rhinitis b/c igA is the ig found in secretions only saliva,milk,mucus,tears etc.
but if there is a history of rhinitis/sinisitis in a child then most probably cysticfibrosis.
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| unique1
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Wow !!! Thanks drduck .
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| nochoice
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i don't understand.
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| studygeek76
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This is selective IgA deficiency. I will quote Robbins:
1) "histrory of allergic rhinitis" - "IgA-deficient patients have a high frequency of respiratory tract allergy and a variety of autoimmune diseases..."
2) "As the transfusion progresses, she becomes rapidly hypotensive and develops airway edema, consistent with anaphylaxis" - "When transfused with blood containing normal IgA, some of these patients develop sever, even fatal, anaphylactic reactions, because the IgA behaves like a foreign antigen [since the patients do not produce it and are not tolerant to it]."
Robbins, 7th ed, pg 242.
To explain the incorrect answer choices:
A) AIDS is a disorder affecting helper T cells. Patients typically do not have antibodies directed against immunoglobins in AIDS.
B)C1 inhibitor deficiency gives rise to heriditary angioedema. The compliment cascade and other vasoactive peptides are uninhibited. Pts have episodes of edema affecting the skin and mucosal surface such as the larynx and GI tract. You would not find a reactive inflammation to components of serum/blood with this disorder.
C)Di-George syndrome: results from a deletion in chromosome 22. The 3rd and 4th pharyngeal pouches fail to develop resulting in T cell deficiency and hypocalcemia. Di-George pts usually have chronic fungal/viral infections. There is no evidence to indicate auto-antibodies against serum immunoglubins.
E) Wiskott-Aldrich syndrome: An x-linked recessive syndrome marked by an inability to make antibodies to polysaccharide antigens. There are low IgM levels, but paradoxically high levels of IgA, G and E. Pts present with eczema, thrombocytopenia and infections. They are prone to lymphomas.
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| lq2006
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selective IgA deficiency
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| inkspot
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good question! it was easy but still i got it wrong ! lack of revision i guess 
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