Maple Syrup Urine Disease (MSUD) | USMLE Step 1 Biochemistry Mnemonic

Maple syrupe urine disease (MSUD) is an autosomal recessive metabolic disorder that leads to the accumulation of branched-chain amino acids. Infants with maple syrup urine disease have urine with a distinctive sweet odor that is reminiscent of maple syrup. This sweet-smelling urine is usually noticed prior to diagnosis and during acute disease. MSUD is caused by a defect in branched-chain ketoacid dehydrogenase, which leads to the accumulation of isoleucine, leucine, and valine. This accumulation of branched-chain amino acids leads to a number of CNS problems: developmental delay, lethargy, and posturing (decorticate or decerebrate). If left untreated, patients usually die in infancy. Since thiamine pyrophosphate (TPP) is a cofactor for branched-chain ketoacid dehydrogenase, some forms of maple syrup urine disease may improve and be treated with thiamine (vitamin B1).
Learn about maple syrup urine disease and other medical school topics with Pixorize's USMLE Step 1 mnemonics. Part of our Metabolic Disorders playlist for the USMLE Step 1 exam.
Subscribe for More: bit.ly/2yybxhm
Study Interactive Image: pixorize.com/view/4215

[@sharmishta8897]

Oh my god how did you know I was slumping in the chair while watching this video?🤣

[@sobster123]

Actually terrific vid! Everything is on point ☝️ 👌

[@dr.praveenkumarbansaloncol8308]

really good videos

[@tikokhachirashvili5520]

thank you so much! it's very helpful

[@khadijashaaban]

this channel is gold 3>

[@mbbsknowledge7066]

Wow, wonderful

[@its.chris4324]

excellent video

[@fatimahagh2110]

I'm impressed😂 good job

[@ziyacecile3494]

This is actually very good representation and it helps me to remember..easy pesy thanks!!

[@Pixorize]

Glad it was helpful for you!

[@drmadhurireddy9474]

Thank you so much

[@shubhamkushwaha8471]

Wow. Thanks a tonne!

[@gargikadu7071]

Simply wow

[@arjunharikumar9040]

Your video is just awesome and made my day as I prepare for my neet exam

[@Pixorize]

Happy to help!

[@raafatali4176]

Brother awesome

[@weirdlywild_]

Thanks man. 🙏

[@countcoupblessings979]

Stumbled on to this & I love it for 2 reasons. Years ago in nursing school I totally loved the word picture ways that they taught the Latin terms, this is that on steroids . I'm a total word nerd & love it. The 2nd reason is Sx I've been trying for years to figure out. I have decorticate posturing at times Among other health issues, some genetic , I often have times where I hit a shit storm of Sx & I just focus on getting thru it . I have decided today I am gonna look up one Sx that is Extremely maple syrup urine. (Just not all the time) So it's possible that my mystery decorticate positioning and the Maple urine (both intermittent together) are connected. Hhmm and the intermittent type of MSUD can be present in adults . Well it's worth a try to ask the Drs. Most of anything self & siblings have ever gotten Dx ...we had to research and bring the info to our Dr ourselves. Barely ever had a good results just presenting odd Sx alone .

[@jacquelinescourageousworld3848]

😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱😱
The sad thing is I was born 1994 and I was born at 24 weeks early and MSUD Maple 🍁 Syrup Urine Disease was something I was born with. My life is very very complicated.
In 1994 doctors 🥼 barley had any information on Cerebral Palsy.

[@pratik9841052968]

Thanks

[@koi_no_yoken]

That's so smart ❤

[@DrMalaK-lx4zg]

So good 😇thank you so much

[@hoshiyarsingh6051]

Are u also medical student??

[@user-te6qc9kw6v]

thank u so much that was awesome

[@Pixorize]

Thanks for watching!

[@Nirvana20202]

Thank u sir

[@zahra_hassan]

Nice

[@aravindanm9962]

Soooo soooooo gooodddddd👍.

[@tonto_kemosabe]

So now maplesyrup smells like urine disease instead of just anti-freeze.

[@user-iv6bp1uf5p]

فيديو باللغة العربية ابني عنده msud عمره ١٠ شهور

[@raafatali4176]

❤️

[@KerbalHub]

Press f to pay respects to the guy

[@brysongoodrich5726]

f

[@segamatthews5023]

I'm glad my urine doesn't smell like maple syrup I might end up putting it on my pancakes one day

[@KrosanBeast315]

You are a very disturbed individual. You should seek professional help. As someone who has MSUD, survived 3 comas from it, and hospitalized 18 times because of it... I can only say you have no right to comment on a disease that KILLS people. It killed me at 8 years old, I flatlined, i had my heart restarted and was in 21 day Coma with 9 wires in my body. You think that is funny???

[@user-iv6bp1uf5p]

Idont know englich

[@fqoffgoogleurcr33py]

This just confused me.

[@DivyaAgarwalDb105]

Why?

[@Cass_i]

@@DivyaAgarwalDb105 it's just all over the place and many of the things just don't... fit. It's not that it confused me it just doesn't make sense. Maybe I just don't learn like this. It probably isn't my thing or maybe it's just this video

[@syedzainulabideen1951]

Whats more delayed than a turtle?
*A snail*

[@85.noorazam74]

Your brain 🧠

[@antonstserba5377]

Did this tree just pissed?!

[@dirantoporu2647]

This is an insult to those like me who have MSUD. His video is sick and DISGUSTING on so many levels, I can't find a place to start. This a mockery to everyone who lives with and all children who have died of this disease. Shame on you.

[@geethabalasundaram3983]

sorry

[@Agtsmirnoff]

What is your problem?

[@elyseenger-fq8ye]

Then must I remind you that in the olden days this was often the case? Doctors didn’t have the knowledge to treat this disorder without the molecular information, and in extremely poor areas with limited resources it still causes death in affected newborns.

[@KrosanBeast315]

dirantoporu2647 is my other account. So, I HAVE MSUD, obviously. Why do you think I would be upset?? This disease put me in 3 comas, hospitalized me 18 times, and it killed me at 8 years old. This disease KILLS people, and mostly children. They are having illustrations that MOCK the disease. Do you really think that this disease is a joke, or some kind of medical prank?! I wish it wasn't real EVERYDAY! @@Agtsmirnoff

[@jacquelinescourageousworld3848]

The scary 😧 thing is I have been living with MSUD since the day I was born.
😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭😭
The Urine got into my mouth at least one time in my life. Because I was born in 1994 doctors didn’t have the amount of information that they do today.

[@KrosanBeast315]

I have had MSUD since I was born too. I wasn't diagnosed with it until I was 8, For a person with MSUD, urine full of Ketones doesn't taste sweet, it smells sweet. Hence the normal name of the disease. It's impossible to fit Branch Chain alpha-Ketoacid Dehydrogenase Deficiency on to a MedicAlert, so the common and embarrassing name is used instead. It's odd for me to talk about MSUD since I'm an Intermittent Variant with a 30g daily protein intake limit due to the unusual nature of my Codons. I basically changed the paradigm for much of what was known about MSUD or could be achieved with MSUD for decades.