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NBME Step 2 CK Form 6 Answers

step2ck_form6/Block 4/Question#42 (reveal difficulty score)
A 6-year-old boy is brought to the physician ...
An excess of very long chain fatty acids ๐Ÿ” / ๐Ÿ“บ / ๐ŸŒณ / ๐Ÿ“–
tags: biochem

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submitted by โˆ—jlbae(159)

I didn't think it could get any more low yield after that Pompe dz question... I was wrong

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charcot_bouchard  Ha Ha....Biochem is like Terminator. Always comes back +4

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submitted by โˆ—medicalmike(82)

This child has some sort of leukodystrophy, as evidenced by widespread white matter disease on MRI. Without having reviewed biochem recently, one can educated guess this question by recalling that fatty acids like to accumulate in white matter; white matter and peripheral nerve conduction will be messed up. Accumulation of VLCFAs specifically refers to adrenoleukodystrophy. Other diseases include metachromatic leukodystrophy, refsum, zellweger.

Abnormally decreased serum cholesterol and Acanthocytes = Abetalipoproteinemia (causes ADEK deficiency)

VitE deficiency damages spinocerebellar tract, dorsal columns, and peripheral nerves (same as Friederich Ataxia)

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ht3  Good thing I know 0 biochem +2

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submitted by โˆ—step_prep7(71)
  • Child with a genetic X-linked disorder (maternal uncle had similar symptoms) found to have a variety of neural symptoms and diffuse white matter disease on MRI, consistent with Adrenoleukodystrophy (rare peroxisomal disorder of beta-ooxidation that leads to VLCFA buildup in adrenal glands, white matter of brain and testes)
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