welcome back to dirty medicines dirty biochemistry series in today's video we're going to be talking about familial dis lipedema now admittedly the different dis lip además are really a pain in the butt to memorize the reason is is because if you flip through any of your review textbooks or your question banks you've probably seen that really daunting chart and it looks something like this so you've got all these different types of dislocating eoz and they're all due to different deficiencies they all have different build ups of different lipid intermediates and they're all associated with different high yield findings and because there's 1 2 3 4 5 6 different types of disabilities note that one of them's not shown on this chart we'll get into that later and each of these six different types has deficiencies buildups and findings it makes for a huge pain to memorize but that is the purpose of today's video in this video i'm going to simplify everything for you and i'll give you a one sentence literally one sentence mnemonic that will help you get all of the questions right about familial dis lipedema will close today's lecture at the very end by talking about some other high-yield things that are not dis lipedema as per se but that are oftentimes associated with this topic so to begin I'm gonna fill in this chart and I don't want you to get overwhelmed because I'm gonna go through everything individually and break it all down and give you some awesome dirty medicine mnemonics but this is what you've probably seen before and it's the same reason that you probably have crazy overwhelming anxiety when it comes to learning the familial dis lipedema --is so we've got type 1 type 2 a type 2 B type 3 type 4 and not shown on this slide there's also a type 5 now again each of these different types has deficiencies buildups and high yield findings and I'm going to simplify everything and help you master this content so that you never miss a question on USMLE or complex so in order to get all this information right I have to give you my one sentence mnemonic and we'll start with the mnemonic and then I'll break it down for you and relate it back to this chart so you can see how awesome the mnemonic truly is so repeat after me 1 LP 2 LD be ads v3 is e4 gets more we're gonna do it again I'm gonna do this a few times just to really get this stuck in your head okay one LP - LD be ads v3 is e4 gets more repeat after me we're gonna keep going one LP - LD be ads v3 is e4 gets more one LP - LD be ads v3 is e4 gets more you absolutely have to memorize this because I guarantee you that if you learn this one sentence you'll get every single question right now let me prove it to you so one LP so each of the numbers right one to be is four to be three four these all correspond to the different types of familial dis lipedema so let's start with one LP so one obviously tells us that we're talking about type 1 familial dis lipedema LP is telling us the deficiency that causes type 1 familial dis lipedema so one LP LP stands for LPL deficiency so lipoprotein lipase deficiency now because you've watched my videos on lipid transport you already know what happens if LPL doesn't work right so if you memorize one LP and you know that type 1 causes an LPL deficiency than in your head you should already have some understanding that without LPL you're gonna get triglyceride accumulation right if LPL isn't working then you can't cut and cleave remember that was from the video you can't cut and cleave triglycerides so triglycerides are going to accumulate so one LP LP for LPL deficiency and that will of course cause a triglyceride accumulation now if you haven't started studying GI yet it's incredibly important and high yield to know that anything that markedly increases triglycerides can cause pancreatitis so type 1 familial dyslipidemia the high-yield finding is pancreatitis that's because of a triglyceride accumulation the triglyceride accumulation is due to LPL deficiency and the way that you memorize LPL deficiency is by saying one LP okay so now let's look at our chart and make sure that this makes sense in the context of what you need to know for USMLE and comlex so type 1 is either LPL or a possi - but in most of the questions are going after LPL so one LP is going to tell you that it's an LPL deficiency the build up is going to be chylomicrons triglycerides and cholesterol the cholesterol should make sense because it's a dis lipedema but specifically the high-yield finding is triglycerides and that's the reason that you see pancreatitis okay so this is an autosomal recessive familial dis lipid II BIA type one one LP LP as LPL the LPL deficiency raises triglycerides and causes pancreatitis okay I hope that that makes sense it should be pretty straightforward but here you are let's repeat after me one LP - LD be adds v3 is e4 gets more so one LP for the type 1 is the LPL deficiency now just remember triglyceride accumulation causes pancreatitis we're gonna see that here in type 1 familial this lipid emia but we're also gonna see it in another type so just keep that in mind if you have a crazy amount of triglycerides you get pancreatitis so now let's move on to 2 LD so we've got 1 LT now we're going to 2 LD so type 2 is due to LDL receptor deficiency so 2 LD LD stands for LDL receptor deficiency okay now this is both type 2 a and type 2 B okay there's two different types of type 2 familial dis lipedema there's 2a and 2b in both 2a and 2b it's due to an LDL receptor deficiency okay so if you have an LDL receptor deficiency then you can't internalize the LDL so you're gonna get a buildup of LDL this causes a tendon xanthomas or like a little fat ball on the back of your Achilles tendon shown here in this picture now how do you remember all this stuff well 2ld obviously tells you that type 2a and type 2b familial dis lipedema is due to an LDL receptor deficiency it also tells you that you have a buildup of LDL it's important to understand that tendon xantho must only occur in type 2a and type 2b so the way that you should remember this is that you have two achilles tendons right one on each leg one on each foot so you got two of them so it only occurs in type two that's type 2 and type 2b okay so now let me prove it to you that this will get you all your questions right let's look at our chart type 2a and type 2b are both due to LDL receptor deficiency it can be caused by April be 100 problems which you should understand if you know the you know the physiology of lipid transport which we've already talked about in previous videos but this is classically associated with LDL receptor deficiency and there autosomal dominant this causes a buildup of LDL among some other things but on the test they're gonna go after LDL okay so 2ld tells you LDL receptor deficiency and LDL build-up you have two Achilles tendons so you're gonna see the Achilles tendon xanthoma you also could see corneal arcus now I didn't put that on the previous slide but it ties right into my pneumonic okay just like you've got two Achilles tendons you have two eyeballs so corneal arcus will be in type two and Achilles tendon xanthomas will also be in type two now the only difference literally the only difference between 2a and 2b is what the next part of our mnemonic tells us so that's B adds V so in type 2 B it's everything that you see in type 2a plus VLDL so look at our chart the build-up is the same in 2a and 2b with the exception being that in 2b you add V right so you have VLDL added into there so in our mnemonic one LP 2 LD be adds V the B type 2b adds VLDL so it's everything that you see in 2a plus a buildup of the LDL so let's keep this stupid let's keep this straightforward right we're just here four points free points guys so now you know type one you know type two and you know type 2b just adds V LDL let's keep going so the next part of our mnemonic 3 is e so we've got 1 LP 2 LD be ats-v 3 is e so type 3 familial dis lipedema is caused by a deficiency of a PO e so 3 is e this is a beautiful beautiful mnemonic it rhymes for your studying pleasure so this is an ape oe deficiency now if you've watched the videos in this section on lipid transport you should know that a po e is important for remnant reuptake so if you can't if you don't have a po even you can't take up the remnants which means things that turn into remnants won't be taken up which means they're gonna accumulate so what turns into remnants chylomicrons and VLDL so with an April II deficiency in type three because three is e you have an increase of chylomicrons and VLDL because you can't take them up as remnants so they just build up now the high-yield finding for type 3 is a Palmer xanthoma so just like we saw xanthomas on the akiles in type 2 and type 3 we see them in the palm so look at this picture I mean little fat deposits in the palm of the hand so how do you remember this right you already know that three is e so you know it's an April a deficiency building up chylomicrons and VLDL because of problems with remnant reuptake but how do you memorize the Palmer xanthomas right how do you know the high-yield finding well because 3 is e I want you to think of a hand giving the number 3 and the reason that it's a hand is because you get Palmer xanthomas right you get xanthomas on the hand so remember the number 3 someone giving you the number 3 that should remind you of the palm for Palmer xanthomas ok so now let's look at the chart I will prove to you that in the context of USMLE and comlex i've taught you everything you need to know so type 3 this is autosomal recessive this is due to an a po e deficiency right 3 is e if 3 is e a po e isn't working you're gonna build up remnants right you're gonna build up Carla microns and VLDL remember that 3 someone giving you the number 3 with their hand for the Palmer xanthoma that's the high-yield finding associated with type 3 familial dis lipedema so guys we're absolutely crushing it right now you already know for different types of familial dis lipedema --is meanwhile the guy or the girl studying next to you in the library is making flashcards and failing miserably because they don't have dirty medicine so tell them to sign up so here we are we've got 1 LP 2 LD bx v 3 is e the last part is 4 gets more so obviously we're talking about type 4 familial dis lipedema now what what this is due to is an overproduction of VLDL so when something gets more it gets more of it so there's an overproduction of it 4 gets more so we're over producing VLDL now if followed the lipid transport videos in the dirty biochemistry section you should already know what the consequences of too much VLDL will be right you'll get a lot of triglycerides and if you get a lot of triglycerides you get pancreatitis we already talked about this and we talked about type 1 to many triglycerides equals pancreatitis so the high-yield finding associated with type 4 just like the high-yield finding associate of a type 1 is also pancreatitis now in addition to 4 gets more type 4 is due to more the LDL or an overproduction of the LDL you can also memorize that VLDL has more letters right so it's got more letters than LDL more letters than IDL more letters than TG so that can also help you remember that this is due to V LDL okay so let's look at the chart type 4 gets more so it's due to V LDL overproduction right more VLDL so you're building up VLDL but you're also building up triglycerides because that's a consequence of too much VLDL and if there's too much triglycerides too many triglycerides of course you get pancreatitis okay so let's look at some common themes here too many triglycerides in type 1 and type 4 so pancreatitis and type 1 and type 4 okay very very important and high yield to know that association so here's the beautiful mnemonic and you've now learned them all right 1 LP 2 LD bx v 3 gets e 4 gets more if you guys aren't sick enough of hearing me say this yet then when you're just hanging out at home when you're using the bathroom showering say this to yourself because it's gonna save you points now that's all the familial disability means that are incredibly high yield that you need to know but there is more wait there's more I've got more high yields for you now the information that comes after this slide is still important right don't get me wrong it's still high yield but it's just one step down from everything we've already talked about so far so if you're finding that your brain is too crams that you can't learn any more information and maybe stop the video now because these are these are still important don't get me wrong but they're a little bit lower yield but I'm just to be complete I want to give you everything that you need to know so there is a fifth type right there is a type 5 but it's really easy because it's basically a combination of type 1 and type 4 which is super easy to remember because 1 plus 4 is 5 so type 5 is the same as 1 plus tag 4 + 1 + 4 is 5 so this is like a no-brainer I'm not even gonna waste any more of my breath if if you got a question about it think about the mnemonic for 1 & 4 & then put them together and that's what you're gonna see for type 5 what I do want to spend just a couple minutes on is something called a beta lipoprotein emia okay this is a disease that's not a dis lipedema right it has nothing to do with what we just talked about but it gets lumped into this section in a lot of study sources and question banks because it has to do with a problem with lipoproteins okay so before we even get into this I want to break down the word for you because I'm a big believer in understanding language and like prefixes and suffixes in order to help you answer questions right so if you don't know what this disease means and you're taking a total guess look at the frickin word guys right a beta lipoprotein emia so a means not having beta lipoprotein means not having beta lipoproteins and amia means blood so the whole thing is a beta lipoprotein emia or not having beta lipoproteins in the blood okay so if you don't have beta lipoproteins in the blood the symptoms should make perfect sense to you so this is due to a deficiency of a PO b48 and a PO b100 right so not having the beta lipoproteins right the B lipoproteins not having B 48 and B 100 so if you don't have those then you already know what's gonna happen right because we talked about what these do in the lipid transport videos so it should come as no surprise to you if you've been studying that if you don't have April B 48 and you don't have April be 100 you're not gonna have VLDL you're not gonna have LDL and you're not gonna have chylomicrons right now this is a problem major problem because if you don't have these components then you can't process fats that's a problem now this is an autosomal recessive disease and let's talk about a couple of the associated findings that you should keep in mind just in case they ask you about this so if you don't have the LDL and you don't have LDL and you don't have Carla microns then you can't shuffle lipids right you can't do lipid transport if you can't do lipid transport you just can't absorb fats period so a decrease in fat absorption means you're gonna see what's called steatorrhea right disgusting fat in your poop it's greasy it's smelly it's got a characteristic appearance and smell if they say greasy smelly stools they're hinting at steatorrhea okay so again a beta lipoprotein idea not having the B lipoproteins in the blood if you don't have them you can't make VLDL LDL or chylomicrons if you can't make those things then you can't absorb fats if you can't absorb fats you get steatorrhea so fat in the poop now what else happens well you're gonna get a decrease in your fat soluble vitamins right because you can't do fat absorption so you can't absorb the fat soluble vitamins and specifically there's two important vitamins vitamins a and vitamins E vitamin A and vitamin E and if you can't absorb these guys especially vitamin E you're gonna manifest certain symptoms so most of the symptoms of a beta lipoprotein emia come from the inability to absorb vitamin E the vitamin A is also important so if you memorize that a and E are the problems the symptoms should make sense you can get retinitis pigmentosa and you get spinocerebellar degeneration okay so take a step back and think about what this means not having B proteins in the blood which means no VLDL LDL or chylomicrons that means no fat absorption which means to a Duryea and if you have no fat absorption you can't get vitamin A you can't get vitamin E which means you get retinitis pigmentosa and spinocerebellar degeneration so guys look if you take this step back and you think about what this word means it kind of explains everything right you know a little bit about what the B lipoproteins do so you know what can't work and then you know what you can't absorb a couple other things I just want to point out one is the treatment and two is the microscopic histologic findings so you're gonna see a canvas sites right and campus sites are just red blood cells that have a spiny appearance of them and you can see them in this picture very very classically associated with a beta lipoprotein emia so if you see that then consider this disease is the possible answer the treatment for a beta lipoprotein emia is giving vitamin E right so if you can't absorb vitamin E you got to give really really high doses get as much of it into the body as you possibly can even if it's just a little amount because having spinocerebellar degeneration is gonna cause a lot of problems right if you're degenerating part of your cerebellum you're gonna have things like eight Axia balance issues etc etc so you give them massive amounts vitamin E the other option is just to restrict triglycerides because if you can't absorb and process them then you just restrict them in the diet so high you'll give vitamin E try to restrict triglycerides that's a bit of lipoprotein emia again not a dis lipedema per se but you know classically included in the discussion of this topic so I put it here just to be complete but guys this is it right this is what you need to know 1 LP 2 LD B X 3 3 is e 4 gets more if you know this you will get most if not all of the questions on familial disappea demeo's right on test day