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NBME 20 Answers

nbme20/Block 4/Question#48 (reveal difficulty score)
A 24-year-old man comes to the emergency ...
Lipoprotein lipase ๐Ÿ” / ๐Ÿ“บ / ๐ŸŒณ / ๐Ÿ“–
tags: biochem repeat

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 +4  upvote downvote
submitted by โˆ—hayayah(1212)
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Pt. has Familial dyslipidemias. Type Iโ€”Hyperchylomicronemia.

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estefanyargueta  Lipoprotein lipase: degradation of TGs circulating in chylomicrons and VLDLs. +2
breis  FA 2019 pg 94 +4
lulumomovicky  Hyperchylomicronemia can be due to LPL or Apolipoprotein C-II deficiencies --> leading to high levels of QM, TG, and Cholesterol +

http://nlaresourcecenter.lipidjournal.com/Content/PDFs/Tables/1.pdf

type 1 hyperchylomicronemia ( pancreatitis and TG's in the 1000's.)



 +3  upvote downvote
submitted by blanee1380(4)
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Very large increase in TGs causes pancreatitis.
Deficiencies in:

Apo B-100: Type II familial hypercholesterolemia, increases mostly LDL which increases cholesterol

Apo E: Decreased remnant uptake, which increases mostly chylomicrons and VLDL, but it's milder so no pancreatitis.

HSL: deficiency would decrease TGs, not increase

LDL receptor: Type II hypercholesterolemia also

So lipoprotein lipase deficiency is the anser, which causes large increase in chylomicrons and TGs, leading to pancreatitis and has a creamy layer in supernatant.

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 +1  upvote downvote
submitted by โˆ—chj7(11)
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Side note for those of you who hate familial dyslipidemias: don't usually watch dirty USMLE videos but I couldn't keep the familial dyslipidemias straight in my head and someone on reddit recommended his video on this. Personally found it really helped.

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 +0  upvote downvote
submitted by โˆ—pg32(218)
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Can anyone explain why the lipase concentration is so high if there is an issue with LPL in hyperchylomicronemia?

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garima  due to pancreatitis +7
neovanilla  ELI5? +
suckitnbme  @neovanilla Type 1-hyperchylomicronemia has increased risk of pancreatitis +
makingstrides  I don't understand why this isn't hypertriglyceridemia because this also causes acute pancreatitis. Maybe because overproduction of VLDL isn't an answer? +



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