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-   -   Tay Sachs and Niemann Pick (https://www.usmle-forums.com/usmle-step-1-forum/418-tay-sachs-niemann-pick.html)

In-N-Out 09-19-2009 06:43 PM

Tay Sachs and Niemann Pick
 
Hey,

How to tell them apart?

They both common in Ashkenazi Jews
They both cause Cherry red spot
They both cause blindness
They both kill early in life
They both cause mental retardation
They both cause seizures
They both are autosomal recessive
They both are lysosomal storage disorders

:confused::confused::confused:

ali-99 09-19-2009 09:25 PM

Nieman pick has reticuloendothelial system involvement
No Nieman pick child comes to the hospital without huge liver and spleen

Also in Nieman pick you have the cytoplasmic vacuolation and the bone marrow involvement

All these are not seen in Tay Sach

In-N-Out 09-27-2009 01:10 PM

OK but then we'll mix up Nieman with Gaucher, again seen in Ashkenazi Jews!

Sabio 09-27-2009 01:23 PM

Quote:

Originally Posted by In-N-Out (Post 1038)
OK but then we'll mix up Nieman with Gaucher, again seen in Ashkenazi Jews!

Only type I (the adult type) of Gaucher is seen in Ashkenazi Jews. This type is far more compatible with life and children may live to the second decade of their life, while in Nieman pick they usually die before the second birthday.

The other types of Gaucher (the infantile and the juvenile) are as killing as Niemman but they are not seen in Ashkenazi Jews

In any case Gaucher does not affect the eyes with cherry red spots




dr z 01-22-2011 08:09 PM

some more differences
 
Tac sachs has no hepatospleenomagaly,
lysosome with onion skin , mostly in jews of eastern europeon descent

NiemnnPick has hepatospleenomagaly,
zebra body inclusion and cytoplasmic vacolization (foam cells) present :)


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