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NBME Step 2 CK Form 6 Answers

step2ck_form6/Block 2/Question#14 (reveal difficulty score)
A 3-month-old boy is brought for a well-child ...
Glycogen storage disease, type II (Pompe's disease) ๐Ÿ” / ๐Ÿ“บ / ๐ŸŒณ / ๐Ÿ“–
tags: biochem

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 +7  upvote downvote
submitted by โˆ—qfever(67)

Warmest welcome back to Step 1 by NBME lol

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lilmonkey  Yep, I had almost the same question on Step 1. +



 +4  upvote downvote
submitted by โˆ—jlbae(159)

low yield low yield low yield low yield low yield low yield low yield low yield low yield low yield low yield low yield low yield low yield low yield low yield low yield

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 +3  upvote downvote
submitted by โˆ—keyseph(99)

Congenital cardiomegaly (point of maximal impulse is deviated to the left) and hypotonia are both classic features of Pompe disease.

Although GM1 gangliosidosis can present with hepatomegaly and hypotonia, it is not associated with cardiomegaly.

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 +0  upvote downvote
submitted by โˆ—bigjimbo(67)

Due to a quick google search, the only way I could see to differentiate GM1 gangliosidosis and Pompes was that Pompes does NOT have splenomegaly. Otherwise, these diseases share many common features.

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 +0  upvote downvote
submitted by satanicdo(7)

trying to use logic for this one: glycogen storage diseases cause hepatomegaly (not splenomegaly) because glycogen is stored in the liver (same goes for von Gierke disease, which is type I glycogen storage disease and is in UWorld)

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