Some weird DiGeorge practice questions

[Kobebucsfan]

which of the following finding u suspect a patient having DiGeorge anomaly ?
A. hypocalcemia + congenital heart disease
B. hypocalcemia + abnormal facies
C. hypocalcemia + B-cell defects.
D. hypocalcemia + candida infection.

A,B,D all seem correct. am i missing something ? lol

Which of the foll. is most likely to be nearly normal in a child with digeorges'syndrome?

1 Atopic allergy
2 cytotoxic t lymph production
3 delayed type hypersn
4 gamma delta t cell nos
5 ig A product.


DiGeorge syndrome is characterized by malformations attributed to abnormal development of the pharyngeal arches and pouches. Most commonly, the third and the fourth pharyngeal pouches are affected. What is a child with DiGeorge syndrome most likely to present with?

A. Cardiac Malposition
B. Hypercalcemia
C. Deficiency of neutrophils
D. Hypothyroidism
E. Behavioral disorders


A?

Another question, are the Ig levels normal or low in DiGeorge ?

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[tasar1898]

1st is D, just wants to tell you that Di George is different from velocardiofacial syndrome, even if they commonly occur together since the genes are nearby in Chr. 22

2nd .. All these depend on T cells in one way or another ..could be choice D but isn't clear what you mean.

3rd this is a ****** question.. None of these has any relationship with the pouches.. I d go with E since chronic hypocalcemia could mess your brain up

IgM should be normal , IgA , IgE,IgG should be low with complete absence of T cells due to class switch defect, but usually there are some T cells to perform these functions

 

[Phloston]

Can't guarantee these are correct (only got low 260s on the Steps):

1) I'd go with A. I think D is referring to chronic mucocutaneous candidiasis. We know C is wrong cuz DiGeorge is T-cell predominant. So it's between A and B. My guess would be A is slightly more specific for overt DiGeorge syndrome over velocardiofacial syndrome (B).

2) I'd go with A. We know atopic manifestations are more directly Ab-mediated, whereas type-IV HS is strictly T-cell-mediated. I'd say choice E is second best, as Ig levels are often normal in DiGeorge (but don't have to be).

3) B, C and D are wrong. So between A and E, I'd go with A. E is not incorrect, but based on how they ask the question, it would be over-thinking things to choose it (which I was tempted to because up to 20% of DiGeorge syndrome cases present with ASD-like features).

4) If it's a black and white situation, I'd say normal. But there are definitely cases with abnormal Igs.

 

[Transposony]

Not sure chronic mucocutaneous candidiasis has hypocalcemia ?
When faced with a question where all choices seem correct, they are asking for the most common features.
I'll go with B. hypocalcemia + abnormal facies

2nd is unlikely to be A since in Atopy T cells are "hyper functioning" and that's why Tacrolimus is sometimes used in atopy.
I'll go with IgA by since there are still some T cells left to class switch.

3rd by exclusion is E. Behavioral disorders (associated with schizophrenia, autism, and bipolar disorder).

 

[Phloston]

Not sure chronic mucocutaneous candidiasis has hypocalcemia ?
When faced with a question where all choices seem correct, they are asking for the most common features.
I'll go with B. hypocalcemia + abnormal facies



3rd by exclusion is E. Behavioral disorders

I wasn't linking hypocalcaemia to candida, but T-cell issues tend to cause diffuse infections of the latter. I just think candida related more to that condition than DiGeorge. And my impression is that cleft palate, rather than lip (facies), is more associated with DiGeorge. And I looked up the third question; there's a couple textbooks that mention "cardiac malposition" with DiGeorge.

 

[Tajima]

Where'd these questions come from? I mean, the source of these things may be some ValueMD FMG honeytrap website.