This story is about a boy
called Niemann who was fond of fried foods
one fine morning, he decided to have cherries fried in oil for lunch
so he set out to pick cherries from the lawn. he went riding on his pet zebra
he reached the garden & started picking up cherries
while our dude Neiman was busy picking up cherries,
he was being watched by
a old hunter
The hunter wanted to steal the zebra
But
Another boy
TaySach was in the garden picking up onions
Tay sach was a brave boy
he scared away the hunter
neiman was happy on seeing this
& befriended taysachs
as a token of friendship, he gifted taysach cherries & oil for a great
lunch
he also gave taysach a ride on his zebra back home
moral of this weird story
neiman pick dis
*zebra body
*cherry red spot
*staining with oil red o
tay sach dis
*zebra bodies
*cherry red spot
*staining with oil red o
*onion ring deposits in skin
hunter disease
*zebra bodies
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1. They're all autosomal recessive, with the exception of Fabry's and Hunter's, which are both X-linked recessive.
2. Hurler and Hunter's both start with an H, and both accumulateHeparan sulfate. (Hopefully that will help you remember that they accumulate dermatan sulfate too).
3. Ashkenazi Jews have a higher risk of Gaucher's, Niemann-Pick, and Tay-Sachs.
4. Gaucher's spares the Brain., has crummpled tissue paper appearance.
5. Remember: "globoid cells" = Krabbe's.
6. "Cherry-Red" (spot on macula) is hyphenated. So are "Niemann-Pick" and "Tay-Sachs." :)
7. Hunters is X-linked. I view a Hunter shooting his bow at a X (target).
8. Niemann-Picks - Sphingomyelinase. No man picks is nose with is Sphinger (finger).
9. As above regarding Hunter's vs Hurlers. Hurlers you get corneal clouding, so you cannot be a good hunter.
10. HurLer's is alpha-L-iduronidase. Hunters, which is a similar disease, is the other one (iduronate sulfatase). "I just remember one, then know that the other one is the other one."
11. Metachromatic Leukodystrophy : Arysulfatase A deficiency
