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 USMLE EXAM QUES.! (31st march exam)  



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Author17 Posts
  #1

a patient having mouth ulcer and eruption,cracking at mouth corner and ataxia..which of the following will be seen ...
1) inc. succinyl coa
2)inc. cystathione
3)dec methymalonyl coa

answer with explanation only(plz)

this ques came in exam as told by my friend..he doesnt know 4th choice but was saying it was irrelevant..




  #2

I think ans is no.1 inc succinylcoa b/c its a B6 def which is involved in heme synthesis.


  #3

i too think of this ...no other choice is relevant with symptoms of patient. its either B2 deficincy OR B6 defi. and in anyways only succinyl coa has relation with them .
cystathione should decrease
and methymalonyl coa dec only if biotin is defficient..


  #4

I think this is due to vitamin B1 (thiamine) deficiency. this vit is essential for dehydrogenases like Succ. CoA Dehydrogenase involved in TCA cycle. B6 def may be more related to AST,ALT and ALA synthase . so i will go to Succ CoA


  #5

kaphoury wrote:
I think this is due to vitamin B1 (thiamine) deficiency. this vit is essential for dehydrogenases like Succ. CoA Dehydrogenase involved in TCA cycle. Ataxia may develop in B1 def as a consequence of Wernickes-Korsakoff.. rolling eyes B6 def may be more related to AST,ALT and ALA synthase . so i will go to Succ CoA




  #6

Deficiency is definitely riboflavin. Cheilitis (cracking and fissures) is a giveaway. Ataxia is consistent also. PDH, TCA and beta oxidation would be impaired. Not sure I like any of the answers. One might argue that since FAD is diminished that succinic DH deficiency would cause succinyl CoA to increase, but since acetyl CoA production is decreased and alpha-KGA DH is impaired I wouldn't really expect to see succinyl CoA increase. Are you sure those were the answers?


  #7

yeah..these were the choices...


  #8

i agree this is riboflavin deficiency however none of the answers looks the best choice. I would pick 1) inc. succinyl coa


  #9

IF that's what had been shown in the exam i will review biochemistry one more time, are you sure that's the entire Q?


  #10

answer is decreased methylmalonate. I forgot about the MTIV amino acids that channel into succinyl CoA through methylmalonate. I am pretty sure that isoleucine and valine are oxidatively decarboxylated requiring riboflavin. Wow that was a tough question.


  #11

blah! what just happened here!!
shockedshocked



  #12

could you please explain in more detail.. i seem to be missing something here.


  #13

its B2 (riboflavin) deficiency.....B2 is used to make FAD and FMN.....and in the citric acid cycle, FAD is used by succinate dehydrogenase.....which converts succinate into fumarate......no B12 leads decreased FAD which leads to decreased activity of succinate dehydrogenase which leads to an increase in succinate and its precursors (eg. succinyl CoA).......hope this helped clear the confusion.....to those that are studying....GOOD LUCK!!!


  #14

and its definitely not B12 deficiency, because a B12 deficiency would cause INC methylmalonyl-Coa and DEC succinly CoA....so b12 is ruled out.....


  #15

Riboflavin will not cause ataxia
The answer is Increase in cystathione (page 110, First Aid 2009)
if b12 is deficient, homocysteine accumulates, hence forms cystathione.



  #16

nitts2009 wrote:
Riboflavin will not cause ataxia
The answer is Increase in cystathione (page 110, First Aid 2009)
if b12 is deficient, homocysteine accumulates, hence forms cystathione.


nod


  #17

agree with nitts





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