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Kaplan Qbank USMLE



Author15 Posts
  #1

hey guys in lippincott's 3rd edition biochemistry book it's written that acute intermittent porphyria is caused by deficiency of hydroxymethylbilane synthase (hydroxymethylbilane is formed from porphobilinogen) and in kaplan notes it's due to deficiency of uroporphyrinogen I synthase ...which one is correct...

which enzyme deficiency causes congenital erythropoietic porphyria???






  #2

hydroxymethylbilane (HMB) synthase (also called PBG deaminase)

Erythropoietic porphyria
The erythropoietic porphyrias include:

X-linked sideroblastic anemia (XLSA): a deficiency in ALA synthase
congenital erythropoietic porphyria (CEP): a deficiency in URO synthase
erythropoietic protoporphyria (EPP): a deficiency in ferrochelatase


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If you yourself are at peace, then there is at least some peace in the world.

  #3

thanks for asking this question. if this one comes in the exam, i will give it a TKO!!! i am not the best in bioch but i got this reference

http://www.biochem.ucl.ac.uk/bsm/enzymes/ec4/ec03...

they are the same enzyme.
Porphobilinogen deaminase.
Pre-uroporphyrinogen synthase.



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  #4

the previus post was from my search of the net..
and this is what te Q bank said
Congenital erythropoietic porphyria (low activity of uroporphyrinogen III synthase)

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If you yourself are at peace, then there is at least some peace in the world.

  #5

thank study-ing and tolito

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The Key to Succeed is Patience.

  #6

Thanx guys for your gr8 input....gr8 buddies to have like u people on this forum....

  #7

If any doubt, go with Kaplan

If Goljan an Kaplan doubt, go with Goljan.

Never FA. smiling face

Sorry, I just saw your Q.


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Our greatest glory is not in never falling, but in rising every time we fall.

  #8

Was moving to a new apartment sorry I could not contribute. As with "Porphyria cutaneous tarda" Im "OUT OF THE LIGHT" with this one. *key words- Uroporphyrinogen decarboxylase, blister, phlebotomy, a little chloroquine. Was taught by grandfather its better to say something even boo! rather then say nothing at all.

*you guys did good job!


Edited by mjl1717 on 07/20/06 - 09:33 PM

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  #9

MJ, your grandpappy must be a very interesting man.. boo!!!! ( now i can understand why you dont like people who dont contribute muchgrin:

___________________
It has been a looooong hard journey but I am inches away from my destination...

  #10

I respect what you're saying. Its just that {in my opinion} in medicine the art of good communication {which many will take for granted} is 50% of the battle {even with the exams).

[Some struggle to communicate properly even in there own indigenous country!] But Ill tone it down.


___________________
Smell the coffee! "Is That an Osler move??"

  #11

Those porphyrias are like "another can of worms" to master.

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Smell the coffee! "Is That an Osler move??"

  #12

http://www.prep4usmle.com/forum/comments.php?id=6...
source of mnemonic
very comprehensive


___________________
If you yourself are at peace, then there is at least some peace in the world.

  #13

2 important porphyreas..
AIP and pCT
difference in clinical presentation
Pct is Photosensitive.

___________________
If you yourself are at peace, then there is at least some peace in the world.

  #14

good one study-ing

___________________
It has been a looooong hard journey but I am inches away from my destination...

  #15

Good studying-we must start somewhere! PCT is most common.

___________________
Smell the coffee! "Is That an Osler move??"







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