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NBME 21 Answers

nbme21/Block 1/Question#2 (53.3 difficulty score)
A 6-month-old girl is brought to the ...

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submitted by seagull(1112),
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! I aeht ehset itwh a biugnnr Fn***ig Tshbmu up fi uyo erage

mcl  Amen brother +1  
praderwilli  Every morning: "I think i'll go over glycogen storage diseases, lysosomal storage diseases, and dyslipidemias after questions this afternoon." Every afternoon: Nah +26  
mcl  oh my god are you me +1  
praderwilli  I recently found a program called Pixorize. It's pretty much Sketchy for biochem. Wish I discovered it sooner cuz it has helped for a lot of the painful things like this! +6  
burak  Cherry red spot basically means niemann-pick or tay sachs. Two differences between is: 1- No HSM in Tay Sachs, HSM in niemann-pick. 2- Both of them has muscle weakness but there is hyperreflexia in Tay Sachs, but areflexia in niemann pick disease. In stem cell HSM is not described and hyperreflexia noted. +4  
abhishek021196  What is HSM? +  
mysticsoul  HSM - HepatoSplenoMegaly. Cherry red spots think of Tay Sachs, deficient enzyme - HeXosaminidase A, accumulated substrate GM2 ganglioside. Niemann-Pick - Spingomyelinase, Spingomyelin <- which is not even a choice. FA18 Pg 88 +  
lakshmi  Dirty USMLE has an incredible video that makes these super easy to get. +1  

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1) eyVr pttaormin ot onte htta haasTS-yc deasesi ash oerm nesitvesi etsarlt oomr)( eefxrl ni easonetn hwti rcsnaieed hade cfem.urnceirec

2) aryFb eisesad = tned ot aehv i-adliusovau ftcsdee ow/ lrive nnvvemetiol

3) nkieipcma-Nn iasesde = aminea a;mp& pntyaoohi htiw lfareiaxe

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submitted by mcl(517),
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Pttinae omts lklyie ash cha-TySas ses.iaed Thsi urfige cyinle hsosw eht aeiolbmchci ytahaw.p Rlclea thta btoh -caTyshaS adn emNnani iPkc deseasi eneptrs ihwt a cryhre der tpso on cso,yufopnd utb yTa hScas cksal het gmnlaalhoetypeepos eesn ni P.N

hungrybox  Man this is such a nice figure except it doesn't have Krabbe disease :( +  
mcl  Here's another one with Krabbe! :) +5  
hungrybox  thank u +  

Nobody has mentioned the super high yield dirtymed video... these mnemonics have saved me countless times... In this case, "a GANG of 6 small jews" helps me remember that Tay-Sachs(six) is a deficiency in the enzyme HEXosaminidase A (six), has an increase in GM2 GANGlioside, and doesn't present with hepatosplenomegaly (small).

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I khnit het taedexgager ealsrtt ni aty csash si aatllcyu due to eht ,cheyrssuiap siht is ntodeneim in ftris dia

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submitted by swandy(2),

Great figure with all the lysosomal storage diseases!

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submitted by burak(42),
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eCrrhy red pots baiasyllc menas pmkencanii-n ro tay hcsas. Two dcsferenfie tenwebe si: 1- No SMH ni yaT s,acSh SMH in pe.ninknicm-a -2 hoBt fo meht ahs semclu ssawenek tub rehte si apiyflerrheex in aTy hSs,ac btu rxiaelafe in inanemn kcpi .aesdise In stem lcle MHS is tno erdbeicsd nad hleerifyxreap t.ndoe

burak  i mean, hyperacusis+hyperreflexia+sensitive startle +  

Since they both have cherry-red spots, two easy ways to differentiate between Tay-Sachs (TS) and Niemann Pick (NP):

(1)liver/spleen: TS: normal ; NP: large

(2) age: TS: <6 months; NP: childhood/adolescence

Bonus: it takes time (older age in NP) to develop hepatosplenomegaly (HSM in NP)