DIP Episode 169 - The Clutch Nephrotic/Nephritic Syndrome Podcast

Okay, welcome. My name is Divine, I'm a resident. This is episode 169 of the Divine Intervention Podcasts. In this podcast, I'll be talking about nephrodic and nephrodic syndromes. Kind of going in a lot of requests from people to please make a podcast on nephrodic nephrodic syndromes. So basically what I'm doing, I'm going to try to accomplish is I'm going to cover it from the step two, CK step three perspective and then I'll also cover it from the step one perspective. So basically if you listen to this podcast, you should know essentially everything, you do need to know about any of the nephrodic nephrodic syndromes, at least the high ear ones that are commonly tested on exams. So let's, let's, you know, just jump into it. So basically, right, these are glomerular diseases, the big thing, and again, I'll sort of talk about it in a later. So you may seem to talk about the same thing three times, but each time, I'll talk about something slightly different. So let's start from the clinical perspective first, let's get like the quick and dirty stuff out of the way. Right. So obviously, if a person has an nephrodic syndrome, right, the big thing you want to look at is the protein excretion in a 24 hour period, right. So if you excrete more than three and a half grams or 35, 100 milligrams of protein in a 24 hour period, that's nephrodic syndrome.

One thing you may see on the upper level exams, like two CK step three, internal medicine exams, the can tell you that the person's urine protein to creatine ratio is greater than three and like three and a half grams per gram or like greater than 35, 100 milligrams per gram. Those things are called a five four nephrodic syndrome. And again, what are the classic findings in the phrodic syndrome? The person will have like low levels of albumin because they essentially peed out in the urine, right? They'll have like a dima because remember, if you, if you have low levels of albumin, then your correct pressure in your blood is going to crap, right. So you have low levels of albumin, your correct pressure in the bloodstream will go down. So you have an extravacational fluid that will cause a dima. And then these people also get hyperlipidemia because again, when the body lever notices that your correct pressure is low, your liver is like what is going on. So it will try everything in its power to raise on correct pressure. So your liver will become tonal lipid. So these people tend to have like a hyperlipidemia. That is why in general when people have a nephrodic syndrome, you tend to treat them with like with a statins. And again, the thing is for the most part, you know, nephrodic syndrome, they can be like some primary causes of nephrodic syndrome. But on the other hand, you can also have some systemic diseases causing nephrodic syndrome, right?

So things like diabetes, amyloidosis, right? Those things can all cause a nephrodic syndrome. So what are the big things you want to keep at the back of your mind with nephrodic syndrome? The first maybe big one you want to understand is like FSGS, right? So like Focal, segmental, glomerulus, sclerosis, right? For the most part, you want to consider this if you see like a nephrodic syndrome question in an African-American, right? So in a black person. And this thing, there are certain high-yield risk factors that tend to pop up on tests, right? So for person is an IV drug user, like a heroin user, right? That's a classic presentation of FSGS. If a person has HIV, HIV in fact, you tend to have like the really bad prognosis FSGS is known as the colapse in FSGS. Okay, that's actually a variant. You definitely want to know for your exam. And then people that are okay, like morbidly obese people, they also tend to have FSGS, right? And really the way you make the diagnosis, so if they ask you for a diagnostic test, when you do a renal biopsy. And really the way you treat it is you can treat, you know, you can give steroids, you can give like local corticodes, or you can give like cyclosporine, right? Those are the treatments for FSGS. Problem with FSGS is about 50% of people with FSGS, they progress to chronic renal failure. And then the next big one I'll talk about right is like membranose nephropathy. Sometimes you may see the refer to an exam says, membranose glomeralopathy.

Okay, so either term actually works. And really again, if you're thinking about a white person with nephotic syndrome, this is what you should go after, right? And one again, one of not paid attention to a concept on the exams with a membranose nephropathy is that this has an association with antibodies against the forceful lipase ethy receptor. Okay, antibodies against the forceful lipase ethy receptor. The NBMA loves to test this stuff. And again, there are many things that can cause this, right? Like people that have like solid cancers, like colon cancer, and stuff like that, they tend to have membranose nephropathy contrasts these with minimal chain disease, where those people tend to have like more like liquid cancers, by liquid, I don't mean like the cancer is liquid. I mean more like hematologic malignancies, hematologic malignancies have a stronger association with with a minimal chain disease, right? So some other things can also cause membraneostephropathy, right? So like hebihepsi, right? If they give you a question about a person that has membraneostephropathy in like an African country or something, you want to think about malaria, believe it or not, malaria can actually be a cause of a membraneostephropathy, right? And the thing is of all the nephotic syndrome, so remember that in nephotic syndrome, you lose a lot of proteins in the urine.

One of the big proteins you lose in the urine in people that have nephotic syndrome, especially like membraneostephropathy is antithrombein three, remember antithrombein three, is an inhibitor factor 10 and factor two. So when people have nephotic syndrome, they lose antithrombein three, there's less inhibition of factor 10 and factor two, so those people become hyperquaglubal. Okay, so one of the hyperquaglubal syndromes, you want to associate with membraneostephropathy on your exam is a renoven thrombosis. So if the describe a person that has a history of membraneostephropathy, or some history of some weird nephotic syndrome, and then the present would like sodium onset, severe flank pain, and fevers, you really want to think about renoven thrombosis. And again, membraneostephropathy, you do a biopsy, right? And for the most part, you give steroids, right? Or you can actually give, so typically you can do one of two things, you can give cyclosporine as monotherapy, or you can combine steroids and cyclophosphamate as the treatment, right? And the thing is if a person has like hebi at the same time as the membraneostephropathy, you also need to treat the hebi at the same time, right? And remember, hebi, right? The treatment is, I mean, you can give interferon, but really on MBME exams, probably the most common treatment for hebi is the combination of entrecidabin, lamiviodin, and tenofovir. Okay, so I'll say that again, entrecidabin, lamiviodin, and tenofovir.

The mmonika, always giving people to remember that is hep belt, so the b is for hebi, and then the e is for entrecidabin, the l is for lamiviodin, the t is for tenofovir. And then the next big nephotic syndrome, right? Like, minimal chain disease, again, if you see a kid with minimal chain disease on your test, I mean with nephotic syndrome on your test, that's minimal chain disease, right? Although you can also get minimal chain disease, believe it or not, in adults, right? So again, just something to keep in mind is actually relatively common in adults, believe it or not, but classically on the USML is the guapter, these in kids, and the reasons common emotional chain disease is when you do like like microscopy, you literally see minimal change, but in electron microscopy, you want to remember that you see like put aside food process effacement, right? So the thing is when you have a put aside food process effacement, you'll lose the negative charges, that you typically find around those put aside food processes, so that can then cause like certain proteins right to leak, leaking to the, leaking to the nephotic, right? And again, remember the association, like liquid malignancies, association with NSAI Ds, that's a big thing to know, right? And it also has an association with like, upper respiratory infections, okay?

And again, the way we made the diagnosis, you go biopsy, you give steroids for treatment, really for the most part, most people that have membranol, I mean minimal chain disease, they are very, very perceived to chronic retinofilia, contrast it with like FSGS, about half of the people that have FSGS will ultimately progress to an endstable disease. And then the next big one I think I will talk about is like diabetic nephropathy, right? If you're thinking about like secondary causes of the nephotic syndrome diabetic nephropathy is like right up there. And one thing you want to keep at the back of your mind with diabetic nephropathy, you don't need to do a biopsy for this, right? For the most part, it's a clinical diagnosis, you see like, you know, like microabuminuria in a person that has like a prolonged history of diabetes, and obviously right, the way you want to treat this, you want to give like an ACE inhibitor and ARB, right? To slow down the progression of a diabetic and a frappathy, right? And remember that for the most part, you want to, you know, try to control the person's glucose, control the person's blood pressure. Now one bizarre thing that your friends at the MBME have started going after on these exams with diabetic nephropathy is like when you should start like, you know, like checking kidney function in a diabetic. The thing is if you have a type 2 diabetic at the time of diagnosis, you need to measure the argument to creatine ratio, right?

So again, just check the kidney function. But if you're dealing with a patient that has type 1 diabetes, you start five years after the diagnosis has been made. Believe it or not, this is one of those bizarre things that essentially everyone gets wrong on exams, but hopefully you don't get it wrong because you paid attention on this podcast. Now, so those are again all big things you want to keep at the back of your mind. And again, right, just again, I've kind of talked about like the specific treatments for these are different nephotic syndromes, but just in general, right? Again, if a person has nephotic syndrome, they should get like, you know, get statins, right? For the hypercholestrolemia, again, you, you want to put them on anti-coagulation if they get like the renoven thrombosis or anything like that, right? If you have like really bad edema, you can put them on like a lube diuretic like ferozamide. And one thing, right? And I'll probably mention this again later, right? But remember that some of the classic findings on your analysis in people that have nephotic syndrome, you'll see like the motis cross pattern, especially from like the oval fat bodies, okay? Those are all classic things that present with an nephotic syndrome. Now, quick drive-by of the nephotic syndrome, right? So I think if the nephotic syndrome is called, I think of nephotic syndrome as a heart, right? So in the nephotic syndrome, you tend to have more like inflammation of the glomerulus, right?

So, you know, these people tend to have like hematuria, they tend to have like what is called nephotic ring protein area, right? So like 24 hour urine protein collection of less than 300 half grams, okay? And these people tend to have again like this, if you look, if you do your analysis, you tend to find like red blood cell casts, but the thing is, you know, friends at the MDME, they don't necessarily put the term red blood cell casts, they may put the term dysmorphic erythrocytes on your test, okay? They all mean one on the same thing. And these people, whatever is in the thing, to have like periobital edema, I don't know why they like they get the edema in those places, but periobital edema, hypertension, those are pretty classic findings in the nephotic syndrome, right? And again, like I said, dysmorphic erythrocytes red blood cell casts one on the same thing. Now, what are some big things you want to keep at the back of your mind with nephotic syndromes? Remember, like, so again, let's just do a quick drive by, right? So like a big one is like good pastures, right? So good pastures, you essentially make autoantibodies against like the alpha-3 chain of type 4 collagen, right? So it's a type 2 hypersensitivity reaction, the classic presentation will be like a young male, and this young guy will have like hematuria, right?

And this young guy will also have hemoptysis because he so happens that type 4 collagen is found in the basement membrane of the glomerular light, but it's also found in the basement membrane of pulmonary of the pulmonary vasculature, especially like the pulmonary capillaries, right? And the thing is one high-ealthine you want to keep at the back of your mind with a good pastures syndrome. Sometimes it's called anti-gBM disease, so anti-lomerular basement membrane disease. The thing is if you're performing in the new floor, since you have like a linear pattern, okay? A linear pattern. Again, those are all high-ealthines to know. And then one of the class of nephotic syndromes you want to know about like your posse immune glomerular nephritis, like your wegners, your microscopic polyurethane. Remember, wegners, the name has changed to GPA, right? So like granolomatosis with polyangitis, and then there's also microscopic polyangitis, and then there's truck strows. But again, truck strows, the name has changed to EGPA, right? So like Eucenophilic granolomatosis with polyangitis. The thing is these posse immune glomerular nephritis, the reason they are called posse immune is that when you do like immunofluorescence, you'll not really see like much in the way of deposition of like antibodies are complement, right?

But the thing is these things for whatever reason, not all of them, but for whatever reason, the generalian NV Me exams tend to cause something called a rapidly progressive glomerular nephritis. I'll define that again, a rapidly progressive glomerular nephritis. Basically, the thing that defines an RPG and is that you just progress from no more renal function to like bad, bad, bad renal function over a few days, two weeks. That's why it's called rapidly progressive glomerular nephritis. And the classic histologic finding in people that have RPG and is like a crescent, okay? Basically, the thing that happens is that some of your mononuclear cells, like your monocytes and whatnot, they leak through the glomerular, they leak through the glomerular basement membrane into woman's space and then they proliferate and that's what gives the chrysentic appearance on a light microscopy, right? So sometimes rapidly progressive glomerular nephritis is also known as chrysentic glomerular nephritis. Again, those are all high yield things you want to keep at the back of your mind doing on exams. And again, I'll talk about like again, some of the other nephritis and drums, like these ones are for the most piloted three hypersensitivity reactions where you have like an antigen antibody complex, like IGN ethyropathy, for example. So that'll be the one that shows up like, you know, like one to two days after an operator's buttocksion or like a GI infection.

And then he'll not show line preparers, like the systemic manifestation of IGN ethyropathy, right? Where those people have like, you know, they'll have like the hematuria, they'll have like abdominal pain and they'll have palpable proper below the buttocks, right? And then I'll talk about like lupus nephritis where like they have like the wire nupa appearance or light microscopy. And then at some point I'll also talk about like membrane proliferative or glomerular nephritis where those people tend to have like the subendothelial trum tracks, right? And then I'll talk about post-infectious glomerular nephritis again, where they tend to have like the subepithelial humps. So in just terms of these like weird and electromyroscopy findings, let me just summarize them here real quick, right? So if a person has minimal chain disease on electromyroscopy, expect to see the putocyte-foot process effacement. That's the boss phrase you want to remember. And a person that has post-infectious or post-traptococcal glomerular nephritis, those people tend to have subepithelial humps, right? And a person that has membranostephrapathy, the classic finding on electromyroscopy is a subepithelial spike and doom, okay? In a person that has membrane proliferative glomerular nephritis, those people tend to have subendothelial trum tracks on electromyroscopy. People that have lupus nephritis, they tend to have a wire lupus appearance of the glomerular basement membrane, okay?

So those are again just unique electromyroscopy slash like microscopy features. You want to start the back of your mind for NV Me exams. Okay, so good posture syndrome again, right? I kind of talked about it. Alpha-3 chain type 4 collagen, your micro-antibodies against it. It's kind of unique in the nephritic syndrome that it is actually a type 2 hypersensitivity reaction. Believe it or not, your friends at the NV Me love this stuff, right? Like, try to just see if you can differentiate 1 hypersensitivity reaction from the other, right? And really for the most part, again, like I said, for good pastures, you give stairs and cyclophosphamide, you can also do plasma for recess to kind of get rid of the nasty antibodies. And again, if you're doing mineral fluorescence, you'll see like a linear deposition of like the of the mineral globulins. And then the posse immunoglobulin of fridities, right? So again, these people have hematuria, but typically they tend to get like RPGN or the chrysentica glomerular nephritis, right? And the big ones you want to keep at the back of your mind, right? You want to think of like, you know, like, wegners, right? Again, remember, wegners is also known as GPA, granulomatosis with polyangitis, right? But the classic presentation of wegners is a triad of like hematuria from the renal failure, right? Hemoptysis from like lower respiratory tract disease. And then sinusitis from upper respiratory tract disease.

But the thing is your friends at the NV MeV realize that every human being has memorized the term sinusitis. So they are beginning to throwing exotic, uh, upper respiratory infections as the third part of the triad of granulomatosis with polyangitis, right? So they're beginning to throwing things like mastoiditis, otitis media, sadunus deformity, right? You station tube dysfunction as all, those things all count as sinusitis in the preview of the NV Me for, um, uh, wegners, right? I remember that wegners is associated with antibodies against the protein is three, okay? Which is also known as Cianca. Essentially, wegners is the only Cianca related pathology you probably ever learned in your test taking career as a medicine physician. Well, unless someone discovers something or completely new that is Cianca positive, right? And then you want to think about like again, microscopic polyangitis. That one is very non-specific, but it's like Pianca and usually shows up in people that are over the age of 60, okay? And they will have like fever, right? So you see like a person that has hematuria has fever, the over the age of 60 and they have like Pianca positivity. They will literally need to tell you the answer to the question in the question because microscopic polyangitis is very non-specific, but remember that it is Pianca positive. Now contrast this with trox-trails. In trox-trails, remember, another name for trox-trails is Eosenophilic granulomatosis with polyangitis.

Trox-trails tends to present, um, in people that have like a pretty storied like asthma history, right? So these people may have like a history of asthma or like really severe allergies and then they will give you like a CBC and you see that these people have like just your crap ton of Eosenophils, right? If you see that in the setting of hematuria, think about Eosenophilic granulomatosis with polyangitis. I remember that it's Pianca positive. And if you remember earlier, I said that Cianca is associated with antibodies against protein is three. Pianca is associated with antibodies against myeloperoxidics, right? So sometimes your friends at the MBME because they know every human being has memorized the terms Cianca and Pianca. They may do things to kind of mess with your head, right? So they may put instead of putting Cianca on your test as an answer choice, in me put anti-protonise three antibodies, right? Like anti-protonise three, Anca, or instead of putting Pianca on your example, we put like anti-MPU Anca, okay? So like anti-myeloperoxidics, Anca. So you definitely want to know what's the terms Cianca and the term Pianca mean, okay? So that's again a big thing when you keep out the back of your mind. And then like I said, right? People that have these posse immune glomerular and afraid of these, again on the amino fluorescence, you wouldn't really see much in the way of antibodies.

And one classic thing that I feel like really helps on MBME exams with these three diseases like the microscopic polyangitis, webinars and drug stresses that the complement levels are typically normal in these diseases. So again, that's something to keep in mind. And again, for the most part, you're tripping on exams, you can do like steroids and cyclophosphamide. Sometimes you can do like retoximap that also does help on that, those certain circumstances. And then IGN Fropathy, right? Again, it will be a person that has like a bacteria like you know, one to two days after like like an upper respiratory infection or GIA infection, right? And the thing is again, the way you make the diagnosis, you do like kidney biopsy, again, complement levels are normal, right? So one thing that I've really found to be helpful for these like nephritic symptoms is just knowing which ones have normal complement, which ones have low complement. And re-IGN Fropathy, most people tend to recover pretty well, although some people may have like multiple recurrences. And really for the most part, you really don't need to treat IGN Fropathy on MBME exams. And I mean sometimes you can you know, maybe toss in like an is in Hebrew and Arabic, but for the most part, you really don't need to treat IGN Fropathy. And then the close causing of IGN Fropathy, right? Like the systemic manifestation of IGN Fropathy, like the the vasculitis associated with IGN Fropathy essentially is like Hinoxion line paper, right?

So again, they essentially have like the same fine names on like my cross copy as you have in IGN Fropathy. But these people again typically have like a poppable paper, but you know the BOTOX, again, you don't treat Hinoxion line paper, again, you know, friends at the MBME realize that most people have memorized that, oh, nephritic, nephritic syndrome always treat, always treat, no, not so for like IGNF ropathy and HSP. IGN Fropathy and HSP are really treated on MBME exams. So again, that's something kind of unique. You want to keep at the back of your mind on tests. And then, right, if they describe a person like a Hishov like Miller-Rash or like positive in a, right? Or do you mean say that, oh, the person has like positive anti-Smith or anti-double stranded DNA and T-Badis, right? And you need to tell you that all the complement is low, like C1 is low, C2, C3, C4, everything is low. Sometimes the pathologist call this the full house pattern. Whenever you see that, you want to think a lot about Lupos and nephritis. And Lupos and nephritis, right? Like some of them, like there's like five or six different types if I'm not mistaken. Really like knowing those different types probably doesn't make much sense from an MBME perspective. But you want to recognize this term. Sometimes on the MBME, they may put something known as diffuse proliferative glomerulina phritis, okay? Diffuse proliferative glomerulina phritis or DPG.

And that's actually one of the most common, that's one of the most common, it tends to show up as more like an ephratic syndrome in people that have, in people that have a Lupos, right? And again, remember the association, like positive anti-double-stranded DNA antibodies, one Thai Smith antibodies. The anti-double-stranded DNA antibodies tend to be, you know, like when a person has those things positive, it tends to like correlate with like the presence of a Lupos and nephritis. And then, remember, you're post-infectious glomerulina phritis, right? So I'll actually explain the mechanism behind this in a bit. But again, I want to cover like the high level, step 2, CK, step 3, step 1 stuff, and then I'll start going into the weeds a little. But basically, people that have post-infectious glomerulina phritis, the big thing that happens is that, you know, they'll have like a strep like upper respiratory infection, right? So like, I don't know, like a strep throat or something like that, or they can have a strep skin infection, right? So like air sypulas or in patego. And then afterwards, like one to six weeks after, right? You know, they'll have like hematuria, they'll have low complement, so complement is low here. Okay, remember, I've essentially said two things that are so low complement, come on, divine think. So post-infectious glomerulina phritis is one. And then, what was the second one I just mentioned? Lupos and nephritis, right?

Those things will have a low complement. So again, very high up to know that. And again, one to six weeks after an upper respiratory infection, or strep skin infection. And you know, you check some key antibodies like ASL-tiders, so anti-structuralizing, no-tiders. You check antibodies against the anti-DN As, B and T bodies, right? Remember, those are actually more specific for like strep skin infections. Okay? So again, those are big things to keep at the back of your mind. And then, I think probably the last big one I'll talk about is like membrane proliferative or glomerulina phritis, right? So remember, again, this is actually one of those ones where complement is low, okay? And typically, they may give you like a weird situation where the persons see one and see for a normal, but the see-three is low. Like the only thing that is generally low in membrane proliferative glomerulina phritis on NBMXM is see-three. The reason that the see-three specifically is low is right. But many of these MPG Ns, at least there's a specific one. I think it's known as like dense deposit disease, so basically like triple D, where they have this thing called like see-three nephritic factor. See-three nephritic factor is see-three convertives, right? So, you know, always break down see-three. So these will see-three levels are like dramatically low. Whatever other thing will be normal, okay?

And remember that this membrane proliferative glomerulina phritis, again, it has like the subendothelial tram tracks on an electron microscopy and you know, kind of has like certain associations like HB, HC, Lupus, right? Membrano proliferative glomerulina phritis, believe it or not, can be actually be a presentation of Lupus. And again, like I said, complement is very low, especially see-three, right? So again, those are big things you want to keep in mind. So, I think maybe this is maybe like, you know, not the worst time in the world to kind of go into the weeds a little, not necessarily the weeds, but let's say maybe like more the step one stuff that classically shows up on tests with regards to these nephritic nephritic syndrome. So again, I've kind of talked about the high-level stuff, but let me sort of give you like some understanding of pathophysiology. I think this will probably especially useful for people taking step one, people that are taking step one, they're just like, okay, divine, I don't know, you know, just memorize like random crap. I kind of want to actually understand what's going on. And again, I'll review some of the high-yield things I already mentioned again, again, just to kind of help you out here, right? But post-infectious glomerulina phritis, right? Again, typically happens after like, after like a strep infection, right? So you can happen after like strep to coca-coffiring gidesys.

And one thing that you may see on step one is that they may try to trick you on, like, they may make like, you know, very big, very weird question, asking you about like, oh, that, there are the strains of groupase strep that cause a, like post-infectious glomerulina phritis, kind of like the same as the strains that cause rheumatic fever. That's actually not true. They actually totally different, right? So it's very different strains of groupase strep that cause post-infectious glomerulina phritis. They are not, they are called like the nephrodogenic strains of a groupase strep. They are very different from the ones that cause acute rheumatic fever. That is actually one of the primary reasons why if you take antibiotics for, like, strep throat, it will decrease your incidence of rheumatic fever, but it actually does not increase your incidence of post-infectious glomerulina phritis for that reason, because it's just different strains of the bulk that cause those problems, right? And again, the thing is, the pathophys here is that essentially when your body makes, although your body makes antibodies against groupase strep, the thing is that those antibodies can cross-react with antigens that you find in the glomerulus, right? So you essentially form an antigen antibody complex and then complement comes and destroys everything in its week and then you have like inflammation of the glomerulus.

And again, don't forget the association with having this like one to six weeks after an upper respiratory infection. Okay, and again, remember that you will see like your classic against subipithelial humps. And again, the key antibodies you want to keep at the back of your mind, you want to keep like your ASO titers at the back of your mind, you want to think about anti-DN Es B titers, right? And then there is this thing called, the location where it pops up on Indian is like anti-higher monoronides, okay? Anti-higher monoronides, that's like a classic presentation on NV Me exams. So again, all big things you want to keep at the back of your mind and then for people taking step one, right? Like when people have post-infectious glomerulus arthritis, if you perform immunofluorescence, you'll see something called a granola pattern. Basically, most of these antigen antibody complex nephotic nephrodite syndrome on immunofluorescence, you see like a granola pattern with one very good exception, right? Which is a good posture syndrome. Good posture syndrome, remember, it has a linear pattern on immunofluorescence, okay? So again, those are all big things you want to keep at the back of your mind on exams. Now, the thing about there is one weird bio-stats, so let's see like epidemiology, like epidemiology. I don't know, this was just an hard to pronounce some time. So weird, like I'll just call it epi. If you see me here, see the word epi, I mean epi-demiology, right?

So one weird epi concept that you may see with post-infectious glomerulus arthritis is that when people have a PSGN, if it's a kid that has it, like it's like 1% or less that end up progressing to like RPGN or getting to like you know like chronic renal failure, contrast is with an adult that gets PSGN, they tend to progress to like chronic renal failure in like about 40% of adults that get a post-infectious glomerulus arthritis. So that's like a difference in incidents of chronic renal failure in people that have PSGN between like adult, like kids versus adults, that you want to keep at the back of your mind for epidemiology exams. And then again, like I said, if you ever hear the term RPGN, again, it just means that this people progress from normal kidney function to just badness, like just super badness within like these two weeks. And again, the classic finding on histology with rapidly progressive glomerular nephritis is the present of a crescent, right? It's the present of a crescent. And again, remember that it tends to have an association with the posse immune of glomerular nephritis, right? So like microscopic polyangitis, troughs which is known as EGPA or Eucenophilic granulomatosis with polyangitis or Wagner's which is known as GPA, right? Granulomatosis with polyangitis. But again, the formation of crescent is the big, big primary pathophysiology. You want to keep at the back of your mind. And basically, like I said, right?

In people that have RPGN, the reason they form crescent is that they have like just super terrible damage of the glomerulus. And when that happens, you'll essentially have like fibrenogen and like your mononuclear cells leaking into a woman's space. And then those mononuclear cells will proliferate like a ton, right? So, you know, kind of like almost like, because they are participating in the inflammation, and you will essentially form a crescent, okay? So people that get like these are RPG Ns, they can literally progress from like normal renal function to like complete renal failure within like a few days, right? And again, like I said, the posse immune, whatever tends to be like the classic, tends to be like the classic, classic causes of RPGN on NV Me exams. And then, like I said, microscopic polyangitis, right? Big things you want to keep at the back of your mind. It's a PNK positive, right? So like anti-MPO, so anti-myloperoxidysa, NK. The reason again, you see the virus repeating certain things over and over again. I'm not repeating these things for fanzis. I'm repeating them because they're actually high-youtu-no, for example. And again, I'm just trying to like, because again, right? Nephotic nephotic syndrome, nebulos subject for many people, right? So I feel like if I describe it, because notice I'm repeating the same thing, but I'm attacking it from different angles.

Like before, I give you like, you know, the high-leveled keyboard watch to remember, but now I'm kind of beginning to like jump into pathophysiology a little. I feel like if you understand this from all these angles, you'll probably never get an afraid of an ephotic syndrome question wrong on any USML exam, all the way from step one to step two, see key to step three, or the internal medicine board exams even. So again, microscopic polyanjitis will be a personal mode on 60 years old, has fever, has weak loss, and then they tell you that they have like hematurial dysmorphic erythrocytes in the oblot. I mean, they are urine on your analysis. If you see that, think about a microscopic polyanjitis, right? And then, remember that's the frappathy, right? Remember, it is actually, in fact, I'll maybe put it this way. Remember, not the frappathy, the literature keeps changing every year, but it's one of the most common causes of nephotic syndrome in adults, right? And again, remember, it tends to occur like, you know, in white people, and again, because it's a nephotic syndrome where you're losing more than three and a half grams of protein in the year in the 24-hour period. And really many cases of membranous nephropathy, there are some key associations you want to remember, right? So like, for present has like a B, hip C, right? For present has like a solid cancer, right? If they have, if they take 13 drugs like NSAIDS, right?

And also, lupus can be a presentation of membranous nephropathy. In fact, if you see a person that has lupus and has an ephotic, not an ephotic, nephotic syndrome, think about nephropathy. If you're present, you see a person who lupus and they have nephritic syndrome, think about DPG and diffuse proliferative glomerular nephritis. Okay? And then, don't forget that. Again, this thing is, I promise you, this is one of those things you very likely see on an exam. Membranous nephropathy has an association with antibodies to the phospholipase etur receptor. Okay? The phospholipase etur receptor. And then, remember the classic finding on histology, right? Is the subipithylial spike in dom. Okay? The subipithylial spike in dom. And again, remember, you lose antithromythrine in three-year-in, you get renoven thrombosis because you have less inhibition of factor 10 and factor 2, right? Renovine, like the nephotic syndrome that has the strongest association with renoven thrombosis is membranous nephropathy. Okay? And then, um, uh, yeah, I think those are probably, you know, like the big things I want to mention with, uh, with membranous nephropathy. Again, it's an antigen antibody complex disease, right? So like the complement binding region of the antibody in the antigen antibody complex, you know, activates the complement cascade and then everything goes haywire and then you inflame and destroy, you inflame and destroy the kidney.

And then, remember, in minimal change disease, right? Again, most common cause of nephropathy syndrome in kids, um, um, remember, right? That normally, right? You know, you have your glorular basement membrane and, uh, that, you know, it's kind of aligned by like, finished, shredded endothelium. And then, you know, you'll find your, you'll find your put aside foot processes. What I think is those put aside foot processes, they get effaced. So what does it mean by effaced? If effaced just means that they essentially like fused together, right? When they fused together, they essentially lose like, they are, like, the negative charge. And when they lose the negative charge, all you mean begins to leak, um, through those, uh, foot processes, right? And then the person ultimately gets a proteinuria with that. And again, for the most part, if you see minimal change disease, you can give steroids, most people that get minimal change disease, you know, they recover pretty well, right? And then, um, effaced, yes, right? So like focosegmental or glomerular sclerosis, right? Remember that it's called, uh, um, focose because it's less than 50% of the glomerular that are affected. And then it's called segmental because it's not the entire, so if you're looking at one glomerulus, it's not the entire glomerulus, that's affected. It's a small part of the glomerulus that is affected. And this on electro microscopy, you'll also see again the classic put aside foot process effacement.

To be perfectly honest with you, the two, uh, same like nephrodite syndrome that you want to associate with, like, put aside foot process effacement is, um, is, uh, effaced, yes. So focosegmental or glomerulus sclerosis, and also a minimal, uh, minimal change disease. Those generally associated with, um, with the put aside foot process effacement. And again, remember that effaced, yes, right? Like more than half, uh, like really more than half of patients with effaced, yes, it progressed like chronic renal failure, usually within like five to 10 years. And, um, one weird genetic mutation that your friends at the NBNME want you to kind of keep track of, uh, with regards to effaced, yes, is the MPHS one. I just think that like it's kind of easy to remember. It's like almost like recurring, recurring letters is like nephrodite syndrome one and nephrodite syndrome two mutations. Although I think they are called like nephrine, if I'm not mistaken, like nephrine gene mutations. So MPHS one and MPHS two mutations associated with, um, with, um, with, uh, effaced, yes. Okay. And again, remember, uh, HIV, obese person, IV drug user, right? And in HIV detain to get a collapsing effaced, yes, there's a kind of effaced, yes, that has a pretty good prognosis is called like the, I think tip link, uh, form, right? It has a pretty good prognosis. And then on the other end of the spectrum is where you have the collapsing kind that is just terrible, terrible, terrible and off.

And then, uh, MPGN, right? So membranose proliferative glomerular nephritis. Again, remember the associated with like a B, hep C, um, it can be a little lupus, right? And the thing is, um, when a person has an MPGN, right? You have like the tram tracks, but they are not sub epithelial. They are sub endothelial, right? So people will have like the sub endothelial tram tracks in a membranose proliferative of glomerular nephritis. And then there is almost like a, it's almost like a subset of MPGN. It's known as dense deposit disease. Just think of it as triple D. Basically, the thing that happens is, you know, you have like an electron microscopy, you have this person that has essentially like a very, or let me say clinically, you have this person that has like, you know, like an MPGN's, MPGN style presentation, but instead of seeing like the sub endothelial tram tracks on electron microscopy, you may see these things called like dense deposits. They're almost like ribbing shaped, so I don't know if I pronounced it right, some little pronounced ribbing, some little pronounced ribbing. So like R, I, W, B, O, N. Basically, you have like this ribbing shaped masses around the basement membrane of the glomerulus and they sort of like come together. And again, the big thing you want to keep at the back of your mind with dense deposit disease is the presence of something called a C3 nephritic factor, right? So in C3 nephritic factor, C3 nephritic factor is literally a C3 convertible.

So it will clearly break down a lot of C3. So these people tend to have like no more levels of C1 and C4, but you have like a profound decrease in the C3. If you see that, I really want you to think about dense deposit disease, which is like a weird variant of membrane proliferative glomerular nephritis. And then IGN nephropathy, right? So again, remember this is also known as like sent firing gyitic nephropathy. The pathophysiology always like bogged me for a while. But really, if you kind of think like, again, from like kind of like digging through the literature and kind of reading like a bunch of books, the thing is IGN nephropathy just typically starts as it's called IGN nephropathy because you have like certain subtypes of IGN, like IGN1, it kind of gets like a weird like like oscillation and when you have that weird glycosylation pattern, remember that I think I may have mentioned this in my intro, immunology podcast where I talked about like pamps, so like pathogen associated molecular patterns. The thing is weird patterns of glycosylation tend to kind of like attract the immune system a lot, right? So the thing is you may have like these people may have like IGN1 with like a weird glycosylation pattern and then the thing is we actually form antibodies against those like glycosylation patterns. So that forms an antigen antibody complex that then kind of like deposits within like the mesangium of the of the glomerulus.

And then you know, you have all this inflammation and you ultimately destroy the kidneys. So the thing is you may say okay, divine, you are saying that it's the antibody that gets glycated and then your form antibody is like, yeah, it's like IGN, like IG that gets like glycated and your form antibody is against it and then everything becomes terrible. So why is this associated with having like an upper respiratory infection or like a GIA infection? Well, here is the thing. Again, coming back to that thing I talked about earlier about pathogen associated molecular patterns. The thing is like viruses and bacteria, right? These some of these bugs on like the surfaces of their cells, they can express this thing that's called like an acetylgalactosamine, okay? So notice the name an acetylgalactosamine. So that means it's something that has again like glycosylation pattern, right? The thing is that glycosylation pattern on those bugs, the body can make antibodies against those and again, now from an antigen antibody complex and then that thing deposits in the kidneys, especially like the mesangium of the kidneys and everything becomes, everything becomes super bad, okay? So hopefully that kind of gives you an idea as to why like the presence of an upper respiratory infection or GIA infection tends to be associated with the development of IGN and frappathy. And then I think maybe the last thing I'll talk about out-port syndrome, right? So out-port syndrome, right?

It has a classic triad. I believe it's like can't see, can't see, can't hear a high C, right? So can't see, can't hear a high C. So typically these people tend to, you know, it's in here, it's in an x-link dominant fashion, right? And FYI, there are two x-link dominant diseases you want to know for the US Emily exams. The first one is out-port syndrome, okay? The second one is, I think, the second one is a fragile x-sendron, remember, fragile x-sendron has like a weird association like CGG-Trenucruthy repeats. So out-port syndrome, you know, x-link dominant. So again, the classic presentation can't see, right? So those people have like eye problems like cataracts or like dislocation of the lens so they can have like weird development of the cornea. And then can't see, can't see, can't see, so the piece like, you know, the nephrodite syndrome they have. And then can't hear a high C, right? So they can have like, you know, like sensorineural hearing loss. So you usually see, you know, like multiple and the family have like these problems like, oh, like that couldn't hear, the uncle couldn't hear, just you know, like weird cluster. Once you see like diseases and multiple family members on in-beam exams, they essentially tell you that it's a genetic disease. So the big thing you want to keep at the back of your mind again, like I said, x-link dominant inheritance. And remember that the mutation is in the alpha, the alpha two chain of type 4 collagen, right?

So don't confuse the alpha two chain of type 4 collagen that screwed up in the outward syndrome with the alpha three chain of type 4 collagen that you make antibodies against in good pasture syndrome. Okay? So alpha three for good pastures, alpha two for our protein, you say, oh, divine. How to remember which is, which remember that a comes before g in the alphabet. So two comes before three when you're counting numbers. Okay? So alpha, alpha two for our ports, alpha three for good pastures. Okay? And you remember that you want to remember that it's mutation in the gene. So again, type 4 we want to remember the specific boss free, C-O-L4-A5. Okay? And C-O-L4-A5 mutation is what gives rise to, is what gives rise to our, our port syndrome. Okay? So I think I'm going to go ahead and stop here. I kind of feel like this is going on for a long, but really like if you're not everything in this podcast, then you will be like a master of like I'll be stunned if you got an euphoric, on euphoric question wrong, on literary inexam. Because I've intentionally tried to like talk about it from like the basic science perspective, the clinical presentation perspective, the diagnostic perspective, the treatment perspective, the histology perspective. So like I just can not think of any other thing, be contest that I really did not talk about. So as I do at the end of every podcast, I go for one or one to learn from any exams, right?

So step one, step two CK, step three, step two CS, pre clinical med school exams, 30-ish-off exams. And then I also offer tutoring for the internal medicine, IBI import exams, the internal medicine, intraneting exam. And then I do this thing called longitudinal tutoring, where if you're a first second or 30-amid student, a tutor for like your block exams or like your 30-ish-off exams. But at the same time, I tutor you for your upcoming USMLA exams. Again, I've done this with tons of people that have all been wildly successful. And then I offer like these USMLA booster courses is like 10 hours for step two, CK and step three, but it's 20 hours for step one. And basically it's something you do at the end of your dedicated period of way when you feel like you know your knowledge base is up to snuff. And then in a Q&E fashion, I put everything together for you basically. I review like most of the high-yield concepts from all the various disciplines that are tested on those respective USMLA exams. And then if you're you know like a med school in a plant to residency, so like an ERS application, or a college student applying to med school, so like an AMCA application, I do offer like one and one I guess you can call it Advice, you know coaching for those.

So like you know rec letters, personal statements, application editing, mock interviews, I've done that with tons of people and again the vast majority of people I've worked with have all matched in the first choice of of residency. And then if you're an international medical graduate, I actually work with a ton of IM Gs. I have worked with IM Gs that have you know been through like very difficult like residency applications circumstances. And again like pretty much every IMG I've worked with has matched, right. And most of them are, I mean I've worked with people that have filled the USMLA's multiple times and they have come back and done extremely well, right. So again if you're in any of those circumstances or you know like a US medical student in any of those circumstances, I feel free to have them reach out to me. And then finally I do offer like one or one tutoring like following the MCAT and like for like many college like pre-med courses, so like Gen CAM, O-CAM physics, bio-CAM, histology, physiology, offer tutoring for all those things. So if any of those things are feel free to reach out. So hopefully you've gotten something from this podcast. Have a wonderful rest of your day. I'll see you in the next podcast which will I believe will be episode 170. God bless you. Thank you.

Proteinuria > 3.5 grams/24 hours (or Urine protein/creatinine ratio > 3.5).

Hypoalbuminemia (due to loss), decreased oncotic pressure leading to edema, and hyperlipidemia (due to compensatory hepatic synthesis).

Antibodies against phospholipase A2 receptor (PLA2 R); also associated with solid cancers or HIV.

Granulomatosis with Polyangiitis (GPA/Wegener's).

Both can show low complement, but MPGN often shows a profound decrease specifically in C3.

Membranoproliferative Glomerulonephritis (MPGN).

Protein excretion > 3.5 grams/24 hours, or Urine protein/creatinine ratio > 3.5.

Anti-phospholipase A2 receptor (PLA2 R).

Diffuse effacement of podocyte foot processes.

Alpha-3 chain of Type IV collagen.

Anti-GBM disease (Goodpasture Syndrome).

Both are variants of MPGN, but DDD specifically involves C3 nephritic factor, leading to a profound decrease in C3 levels.

Outport Syndrome.

Protein excretion > 3.5 grams/24 hours, or Urine protein/creatinine ratio > 3.5.

Anti-phospholipase A2 receptor (PLA2 R).

Diffuse effacement of podocyte foot processes.

Alpha-3 chain of Type IV collagen.

Anti-GBM disease (Goodpasture Syndrome).

Both are variants of MPGN, but DDD specifically involves C3 nephritic factor, leading to a profound decrease in C3 levels.

Outport Syndrome.