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Bernard Soulier or Hemophilia!

4K views 4 replies 3 participants last post by  roshan dinparast 
It's not a good question

This is question is wrong. Glanzmann and Bernard Soulier are both autosomal recessive. The only thing that may point away from hemophilia is that the child should usually start bleeding at a bit younger age. But still the scenario is definitely of hemophilia (the maternal uncle, the X linked recessive, the hemarthrosis all pointing to it).

You triggered me to make this table :)
http://www.usmle-forums.com/usmle-step-1-bits-pieces/2467-bleeding-disorders-table.html#post8602
 
Platelet dysfunction

o thanks Lee... great table!!!

emm... why in late liver failure bleeding tym will be increased??? dat depends on platelets... didnt get dat point!! is dat because of von willebrand factor being less synthesised???
Patients with advanced liver disease have prolonged bleeding time even if their PT and PTT and platelet count is normal. The bleeding is due to platelet dysfunction rather than decrease in number
See this http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1663385/

Platelet dysfunction is also the reason behind prolonged bleeding time in uremia and patients on Aspirin.
 
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