DIP Episode 367 - The Clutch Amyloidosis Podcast (for Step 1-3)

Okay, welcome. My name is Divine. This is episode 367 of the Divine Intervention podcasts and this podcast I'm going to be tightly in this as the Clotch Milloydosis Podcast. The Clotch Milloydosis Podcast. I'm a lot is a very high-ealtopic and before I jump right into it if you're taking your USMELY step two, CKO step three or complex level two or three exams within this month or I will see within the first three weeks of March. Then I do have an MBME Testikin Strategy's course, Tikin Place on the 21st from to the 430 PM Mountain Time which is photo 630 Eastern, basically two hours behind Eastern Time and then I have a 24 hour review course. It's going to be Tikin Place from the 22nd to the 25th of February and all of this is Thruzum. The 24 hour courses from 70 to 1 PM Mountain Time. That's like 9 to 3 PM Eastern Time every day. So if you're interested just shoot me an email through the website and I'll give you some more information. Again, I've had many people take the course and they've done extremely well on the exams. In fact, I got I've got in many emails from people that I've got in the 260's, the 270's and even the red 280's from taking my course. So again, I think it's something you're going to find to be helpful and then if you're taking your USMELY exams in the summer I have a Divine Intervention Step 2 CK School. It's going to be taking place within the first two weeks of May. It's going to be a 70, basically it's going to be 75 hours.

We're going to have like an expanded Tikin Strategy course. We're going to have adaptive learning. We're going to have like a ton of review. In fact, I would imagine that by the end of the course, the school, people should have about 400 to very likely 500 or more pages of notes. That does not include the slides, the tons and tons of slides they would have as well. So again, if you're interested, just shoot me an email through the website. I have a pretty comprehensive podcast where I talked about it's a colleague at the disk DISK school. Okay, so let's jump right into Ameloidosis. So this should be a short podcast. So what is Ameloid? Well, Ameloid is just pretty much a mis-forted protein. Ameloid for the most part is a mis-forted protein and you know, there are two ways you can get Ameloidosis. I will say probably the most common pathway is you start with a protein that is normal, but because your body just produces so much of it, so much of it, it then accumulates and begins to cause problems. Right? Or it can also be a protein that your body accumulates because you can not just get rid of it, right? Again, just think about it, right? Like if for example, something just keeps accumulating, accumulating, accumulating. Over time, it's going to become problematic. Your body does not like things like accumulating. That's why your body has many organs to help you get rid of stuff like your GI tract, your kidneys, your liver, right? Even your skin is an excretory organ, right?

But if all these things are accumulating with nowhere to go, then that becomes a problem. And we'll talk about some of these things really like if you understand what we talk about here today, it should be able to answer pretty much all your Ameloidosis questions, right? On the exam, right? And then another thing I want to mention is you can also get Ameloidosis from mutations in proteins that then causes them to fall in very weird ways, right? So again, it just kind of depends on, but that one is a lot rarer than just the regular Ameloid, right? And for purposes of USML exams, sometimes Ameloid is broken down into two parts, right? So we have the primary or E-E Ameloid. Sorry, the primary of the EL Ameloid. So primary or EL Ameloid. That's the one associated with multiple myeloma. And then we have the secondary or E-E Ameloid. That's the one that's associated with pretty much every other cause, right? Especially when you have like chronic infection or chronic inflammation in the body like rheumatoid arthritis, for example, right? Now some structural information that you need to know, this is probably more so for step one, but I want this podcast to be something that will help everyone taking step one through step three and their encounter in Ameloid questions. But a structural thing that's helpful to know about Ameloid is that Ameloid shows up as a bit of pleated sheet, right? Remember, bit of pleated sheets represent a special kind of protein structure.

More specifically, it's a secondary kind of protein structure, right? So it shows up as a bit of pleated sheet, especially when you look at it under an electron microscope. And then it's also high yield to know how to identify Ameloid. When a person has Ameloid doses and you take the Ameloid protein, the thing that's going to happen is if you look at it under, if you're stingy with a particular sting called congruared, and then you look at it on that polarized light, right? You're going to observe apple green by refringents, right? Again, these are just all basic things you kind of want to make sure you know about Ameloid, right? So now let's jump into just almost call it like vignettes that our friends at the NV Me scan can bring, right? So if they give you a question about a person that you know has a history of Down Syndrome that dies of a sub-rock noise hemorrhage. What's going on there? Well, I hope you're thinking about like Ameloid and Geopathy, right? Ameloid and Geopathy. We remember people that have Down Syndrome, right? That's tricellumy 21. They literally have three copies of chromosome 21. And on chromosome 21, we have Ameloid precursor protein, right? So normally if you think about it, if you're normally getting gene product from two chromosomes, but now you're getting from three, you're potentially making excess, right?

Those literally, those beta Ameloid plaques from Ameloid precursor protein, they're going to build up and they're going to deposit in the brain and they're going to cause Alzheimer's, right? Most people that have Down Syndrome right around the age of 40, almost all of them as a rural university have Alzheimer's, right? But again, unfortunately that beta Ameloid are protein can deposit in the walls of blood vessels and make them weaker. When you deposit those things, then those vessels can blow out and the person getting to get into trouble with a sub-rock noise hemorrhage, right? And I guess one thing I should have maybe said in the introduction is Ameloid can affect many organs in the body. But the primary organ, Ameloid loves to go after, is the kidneys. The kidneys are the most commonly affected organ in an ameloidosis. But if you see a person that has ameloidosis die, most times they die from heart disease, right? Ameloidosis can cause the astolic dysfunction of the heart because it can deposit in the walls of the heart, causes like electrical problems in the heart, causes problems with the heart failure appropriately. So the person goes into the astolic heart failure. That is if I'm not mistaken, the most common cause of death in people that have ameloidosis, right? Or if they give you a question about a person, you know, like an old guy that has been having multiple faults, that's a pretty classic way they like to present this, right?

And they may tell you that, you know, he has these, he has these visual hallucinations. Then I really hope you're then about Louis body dementia, right? Remember, Louis body dementia is kind of related to Parkinson's disease, right? So really like these Parkinson's disease, slash Parkinson's disease related syndrome, like multiple systematrophy, for example, they are associated with mutations in a protein with ameloid, right? From a protein called alpha-synuclein. Alpha-synuclein is the protein that causes the problem, right? Or you can see people that have, you know, they give you a question about like a neuropathologist or a person that's an archaeologist and the person over like six weeks, they have this rapidly progressive dementia and then the pass away, right? I would really hope you're saying, oh, divine, and many things they'll have myoclonus, right? That's kertsfold eacop disease, right? kertsfeld eacop disease that's spelled as C-R-E-U-T-Z-F-E-L-D-T, right? kertsfeld eacop, eacop, some people say J-A-C-O-B-O-J-A-K-O-B, right? So kertsfeld eacop disease, it's a pre-on disease, right? Sometimes they call it a spongy form and say phallopathy, one exact, right? So again, remember our friends at the MBA meetings, one thing they've started doing in recent times is instead of using the name that you're used to, they just use a name that describes the pathology. Again, that's why you can not just onkey a little success on this example.

You should onkey, but yet, my wrong, onkey is amazing. It's one of the best tools ever invented, but you need to make sure you're understanding those things, you're just onkey, right? So when a person has kertsfold eacop disease, sometimes they can call it a spongy form and say phallopathy. It's just a description of how the brain looks grossly when those people die, because those people are invariably going to die, right? I remember the protein that causes the problem is the PRP superscript SC protein, right? So PRP superscript SC protein is a pre-on disease, right? It's a pre-on disease. And then if they give you a question about a person that has dementia, has all these inappropriate words, gestures, stuff like that, you want to think about like front to temporal dementia, right? Or even pix disease, sometimes they call it pix disease, right? Those things are actually associated with aminoidosis, right? Really many dementia are associated with aminoidosis, right? So like the protein varies, right? Like it's a tau protein, right? It's a protein that's associated with micro tubules. Remember, micro tubules are things that move along, they're very important for moving neurotransmitters in neurons, right? So it would make sense that it can cause a lot of neurologic disease, because guess what? Your brain is chock full of neurons, right? Even if they give you a question about a computer scientist, right?

That has, is like in his 40s, and you know, he has these choriform movements in appropriate words, right? That's going to be haunting tense, right? Or those are more dominant disease, chromosome 4 problems, C, G, trinoclothid repeats, right? Those are all associated with aminoid, right? Like really like the haunting protein can form aminoid, the haunting protein can form aminoid, right? And then if you give you a question about some old person with pathologic fractures, right? And you notice that the person's causing is like 15, like you know, some crazy high number, the person keeps getting these recurrent infections, right? That's going to be multiple myeloma, right? It's going to be pretty classic for primary or E L analog. Remember, it's a light chains that cause the problem, right? It's a light chains that cause the problem. Remember, in multiple myeloma, right? The homerca, those crop symptoms hypercalcemia, redophilia, anemia, bone pain, right? Again, because again, those plasma cells, they take over the bone marrow, so you're not able to produce red blood cells, so you get an anemia, right? And then you wonder why they're getting recurrent infections. Well, the thing is plasma cells are supposed to be antibody factories, but those plasma cells in multiple myeloma, those cells are super defective, they don't really work well at all, right? So the person is not going to have a good time, right? So they're going to have an annoying dosage, right?

Because those light chains are going to accumulate, right? So that's something that's pretty high to know for, for example, right? And then really if you have any chronic inflammatory state, rheumatoid arthritis, or like a chronic infection, like chronic osteomyelitis, for example, right? Your form and acute phase reactant, that of acute phase reactant is called a serum aminoid A, that serum aminoid A can literally accumulate again and form aminoid, right? Now, what if they give you a question about an old guy, right? Usually, I mean, this condition I'm going to discuss now is certainly happens in old women, but it's something that almost exclusively on NBM Es and also just in the real world happens in old men, right? They give you a question about an old man and he incidiously develops these heart failure symptoms, you know, like he has like pulmonary dima, has druglavenous distension, has low extremity of dima, right? And then they tell you that the money is pretty cardic, right? That's a very specific signature. It's going to be an old man. Usually it's going to be a person over the age of 80 on exam. Sometimes they can give you in the 70s, but usually it's going to be a person over the age of 80, right? Old man with heart failure symptoms and Brady Cardia. If you see this, I really want you to think about something that we call senile systemic amyloidosis, right? Senile systemic amyloidosis, right?

The thing is it's a normal protein, it's a protein called a transphyritin, T-R-E-N-S, T-H-Y, R-E-T-I-N, right? Transphyritin. It can deposit in the heart, right? And again, when amyloid deposits, right? Cosy electrical problems, so you can get all these heart blocks, right? You can get really bad Brady Cardia, right? But they also have heart failure symptoms. That's the classic way. This is one of those things that you learn by a world of high-yod history on inbinding exams, right? And how do you diagnose this senile amyloidosis? So back in the day, and actually to this day, some people still do biopsies, right? Again, biopsie, the heart, you do an endomyocardiobyopsy, guided by echocardiography, usually like a T-E-E, and you get a sample, check it under, you know, polarized light stink on regret, right? You're going to see a blue-green buyer of fringes. I've actually, I think, a moment's taking I've seen this in the real world, but actually, it's not like I think I have seen this in the real world, right? But these days, the way it's diagnosed, you can actually use a nucleobaticine study, right? So there's this thing called the Technician 99 Empire of Forsyth's Gun, right? So Technician 99 M-Synthigraphy, you can use that to diagnose senile systemic amyloidosis, right? Or if they give you a question about a person that has been on dialysis for a long time, right?

And then you start noticing that they have like a couple tonal syndrome, they're having their stolic-cardia dysfunction, they're having like an enlarged tongue. When you see stuff like that, I want you to think about dialysis-related amyloidosis, right? Remember, in dialysis, normally your kidneys can get rid of the protein beta-2 microglobulin, but when you have dialysis, you cannot, right? The dialysis machine cannot get rid of beta-2 microglobulin. So beta-2 microglobulin, right, is one of the things you find on receptors. In many of your immune system cells, it's going to be old up, right? And it's going to form amyloid. Then also remember, for a person who has diabetes, right? Especially with type 2 diabetes, over time, because if you've probably learned this that open a half-type 2 diabetes, you know, over time the aparkis is going to burn out. One of the thoughts as to why the aparkis burns out is because they're making a ton of amyloid, right? That amyloid then deposits in the pancreas and destroys it. So where does that amyloid come from? Well remember, whenever people that have type 2 diabetes, the problem is they have like insulin resistance, right? So people that have type 2 diabetes actually have pretty crazy high amounts of insulin, right? So the thing is, insulin is being made. There's an opportunity that's made at the same time called amylin, AMY, like Amy, right? AMY, LIN, right? Sometimes they call it IEPP, right?

So that amylin can accumulate and form amyloid, right? In fact, you can also see a similar amyloidosis in the presence of an insulin normal. Remember, both are having insulin normal, they're going to have with post-triad, right? It's one of the most obvious trials in all of medicine, right? They're going to be hypogeolicymic, then they're going to have signs of hypogeolicymia, and then said hypogeolicymia will resolve and give them glucose, kind of makes sense. I don't know why that's a tried in the first place, but anyhow, I'm going to get off my soup box, right? So that's another thing that can cause amyloidosis, right? That amylin can form polymers that can then become amyloid, right? Or you see a person that has like an egg mass, right? And again, is that having these antisydents of amyloidosis, like capotono syndrome? Remember, amyloid lost to the positive in the capoton in the capotono, right? And it can cause capotono syndrome, right? It can cause enlargement of the tongue, it can cause cardiac arrhythmias, right? That's not like this function, but again, I said the most one organising involves is the kidneys, right? Those people can absolutely form amyloid, right? So remember, in medallary thyroid cancer, right? Calsytunin, that calcium tonin can, you know, come together and form amyloid, right? So again, those are just all things you want to keep at the back of your mind, for example.

Or you can even give you a question about a person that develops amyloidosis, like this one is not systemic, it tends to be like localized, like it's an injection site's problem, right? Like I mentioned, it has been on chronic injections for HIV, right? This is going to be the drug and fulver type, right? For those of you studying for step one, I'll really hope you know what fulver type is or does, right? It's a fusion inhibitor, right? Remember, GP41 is a protein that enables HIV to fuse and then infect itself, right? So the thing is, fulver tide spelled as ENFU, VIRTID, ENFU Lver tide, right? It can form amyloidosis, right? At the site of injection, right? That's something to just keep in mind, for example. It's just one of these weird things, you see all your exams and you're like, whoa, where did this come from? Right? So again, if you know these things that have described about amyloidosis, you should be in pretty, pretty good shape. So I think I'm going to go ahead and wrap up here. Again, as I do at the end of every podcast, I don't for one or one two day from all the USML exams, step one, step two, CK step three, preclinical medical exams, 30-ish-off exams, and I also offer tutoring for, you know, I offer these courses for step two, CK and step three, right? I have the divine intervention step two, CK school. It's limited to 40 people, again, it's taking place in the first weeks of May. But then I also have 24-hour review course that I host.

That's going to be taking place at the end of this month, again, from the 22nd to the 25th, and an MBME test thinking strategy course on the 21st. And then I do have these podcasts on Apple podcasts, Google podcasts, Spotify, right? So if you're interested in any of these podcasts, just go ahead and find it, you know, you're going to find the most recent 150. If you're on everything from a episode one to episode 367, you have to go on the website, the vinytravensionpodcast.com. If you have a Word Press account and you subscribe, then you get an email notification whenever I make a new podcast. And then I have a You Tube channel, the vinytravension, you have some really podcasts and videos. That's where I post the videos that I make. And I do make quite a few videos every now and then. And then finally, I also have a new website called the vinytravension, lifelessens.com. It's a Bible Bee's website. It's a lot of podcasts and most of them are about 10 minutes long, 10, 12 minutes long. And I just describe a classic problem that's facing humanity. And then just describe how the Bible tacos that problem. I actually also have the podcasts on Apple podcasts, called the Divine Intervention, Lifelessens Podcast. So thank you for listening to me. One quick thing I just want to say here is this is a new year, right? Stop looking backwards, right? Stop looking forwards. I feel like that's kind of like a good life lesson for today, right?

Many people, they just keep looking backwards, keep looking backwards. The thing is, you know, you may have meetings fixing your past. Everyone has meetings at some point. You know, meetings fixing your past, checkered past, you know, bad relationships, bad things you've done, people you've heard, right? But again, you can turn your life around, right? The way you turn around, right? Think about it if you're, if you want to turn around, you should stop moving in the direction we're moving before, right? So if you're moving backwards before, just start moving forwards, right? Start looking forwards. I like this Bible character like a lot. He's wife, you know, they were being delivered from Suttaman Gomorrah, but they were like, a lot of wife was like, you know, what I really like Suttaman Gomorrah a lot, right? They should keep looking at Suttaman Gomorrah and then in the end, she trying to appeal off salt, right? No, just keep moving forward, keep moving forward, right? So I'm telling you, if you've messed up in the past, the fact that you have a bad background, it doesn't, again, you've heard this statement many times, right? Your bad ground should not keep your back to the ground, right? Just keep looking forward, keep pressing forward, right? And all be well with you. So thank you for listening to this podcast. Have a wonderful rest of your day. God bless you and have a great weekend. Thank you.

It is a condition caused by the misfolding and accumulation of proteins that deposit extracellularly in various organs.

1) Overproduction of a normal protein, or 2) Impaired clearance/removal of a protein.

Congo Red stain, which exhibits apple green birefringence.

Primary AL (light chain) amyloidosis.

Serum Amyloid A (SAA).

Heart failure symptoms and marked bradycardia/cardiac conduction issues.

Primary is associated with plasma cell dyscrasias (e.g., Multiple Myeloma, light chains). Secondary is associated with chronic inflammation (e.g., SAA from RA or infection).

$\beta_2$-microglobulin.

Heart failure symptoms and marked bradycardia, often diagnosed via Technetium-99m scintigraphy.

Amyloid precursor protein (due to Trisomy 21).

Hypoglycemia, signs of hypoglycemia, and subsequent resolution/rebound glucose.

Creutzfeldt-Jakob Disease (CJD), caused by $\text{PrP}^{Sc}$ protein.

Primary is associated with plasma cell dyscrasias (e.g., Multiple Myeloma, light chains). Secondary is associated with chronic inflammation (e.g., SAA from RA or infection).

$\beta_2$-microglobulin.

Heart failure symptoms and marked bradycardia, often diagnosed via Technetium-99m scintigraphy.

Amyloid precursor protein (due to Trisomy 21).

Hypoglycemia, signs of hypoglycemia, and subsequent resolution/rebound glucose.

Creutzfeldt-Jakob Disease (CJD), caused by $\text{PrP}^{Sc}$ protein.