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- [00:01]
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Doctor/Physician
I guess I haven't really been myself lately. Like, I've been nauseous, swollen, feeling pretty run down, but it's just normal pregnancy stuff. It was a bit late for you to still be getting nauseous.
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We would like to get an ultrasound and run a few tests.
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Unfortunately, you have a condition called acute fatty liver of pregnancy. We need to do a C section.
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Dr. Chapa
Too soon.
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Doctor/Physician
The baby is viable. We don't have any other options.
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What's going on?
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Oh, they have to deliver the baby.
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Dr. Chapa
I'm scared. Where are you? So in the quite melodramatic and peripherally accurate Chicago Med, there was an episode where they highlighted acute fatty liver of pregnancy aflp. And I'm glad they did. I'm glad any of these shows call attention to maternal complications because at least it gets the word out. Of course, it was done very melodramatically for emphasis, and I'm okay with that. Anything that gets the word out about these issues, I'm all for. But we're talking about that clip, which, by the way, it's not a sponsor. We're talking about acute fat delivery of pregnancy because it relates directly to a real world case that we have on our case log right now. So let me just set this up very briefly and I'm going to get into, as you could guess, aflp, acute fatty liver pregnancy. So here it is. Short of it is 24 hours ago, yesterday, from our day of our recording, we readmitted a patient who was six days post op from her C section. She had her section because the kid was ginormous. You know, it was way off the growth charts. It met criteria for macrosomia meeting the cutoff for shoulder dystocia prevention. We offered her a section. She agreed. But by the way, she also had intrahepatic cholestasis with some slightly elevated transaminases. They weren't terrible. They were maybe just barely twice normal. But, you know, not unusual for icp. Intrahepatic cholestasis of pregnancy. It happens. So we sectioned her. She was fine. No hypertension. Went home. Well, she came back yesterday, which was six days post op, to take away her staples, do a wound check. Because her BMI was elevated, we wanted to make sure her wound look okay. And lo and behold, her pressure is above 140 over 90. And she just looks kind of bad and it doesn't look right. We said, so how's it going? You know, how do you feel? What's going on? She goes, well, you know, I've got this throbbing headache at the front of my head, just right above my eyes and it won't go away. And I kind of have this weird, just kind of. I thought it was heartburn. It just. It won't go away. Kind of, you know, mid chest. Yeah, I just don't feel well. Well, pretty straightforward, right? You got elevated pressures, unrelenting headache, maybe some rib quadrant slash, epigastric discomfort. We're like textbook preeclampsia with features in the postpartum interval straight to labor and delivery. Thankfully, she went because not all of our patients do, but she went. And she was started on Mag sulfate, as is protocol. Of course. We redid her labs and her transaminases were in about the 400 range. All right, they're elevated. That's another severe criteria. It still fits the picture of preeclampsia with severe features. None of that's outrageous. Now, she had no evidence of HELP syndrome. Her pressures were elevated, they were brought down and stable well today. So this is 24 hours now from the original readmission, her transaminases, which we checked again because we wanted to make sure she wasn't deteriorating into help. And her transaminases today are now about 900 range, severely elevated. What they were compared to baseline and even just 24 hours ago now, yeah, still fits the criteria of elevated liver enzymes, which fits with preeclampsia with severe features. But here's where the question came up within our faculty as we took care of this patient with the resident team, could this potentially be. And are we missing aflp? Acute fatty liver of pregnancy. Because the two conditions, guys, preeclampsia with severe features, aflp, they can look the same, they can have elevated liver enzymes, sometimes there's some high blood pressure that goes with it. So how do we figure this out and are we missing that now? We don't wanna miss either of them, that's for sure. But if we do miss aflp, it has the potential, the potential to be life threatening. I mean, this can lead to multi system organ dysfunction. This leads to a hepatic failure. AMMONIUM levels go through the roof. And it's a huge problem because coagulopathy becomes nearly impossible to control. Uncontrolled or ignored or overlooked, AFLP is potentially fatal. That's a bad one that you don't want to miss. Now, thankfully, it's much more rare as opposed to a preeclampsia with severe features, which is what we settled on, though we're putting the patient on serial labs to trend. And just FYI, she does not meet lab criteria for help syndrome. Platelets are fine, so automatically that's an exclusion for help. But isn't that a good real world scenario? This was a conversation we're having. Could she have aflp? We went through the criteria. Nope. This looks like preeclamps. You have severe, though we will continue to follow and trend labs. So I thought, you know what, it's a great episode. This is actually on our case log right now. Let's talk about acute fatty liver of pregnancy. And how do we distinguish this from other issues that jack up the liver function, like preeclampsia with severe features? There's great criteria. There's actually recent data on this. Something was published out of serious 2025 in June. The title of this was Management of acute fatty liver of pregnancy. This was a retrospective study of 12 cases. 12 cases compared with data in the literature. That's an end of 12, guys. That's how rare this is. But you've got to get this right, because again, if you miss this, it could be potentially really, really. Let's throw in one more really bad. So I think I've set it up enough. Let's talk about acute fatty liver of pregnancy, which is bad, and how to distinguish that from other things that jack up liver enzymes in pregnancy. Specifically things like help or preeclampsia with severe features. I think I've set it up enough. We'll be right back. This is Dr. Chapa's OBGYN no Spin podcast.
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- [08:06]
Doctor/Physician
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- [08:10]
Dr. Chapa
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- [08:14]
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- [08:22]
Dr. Chapa
All right, podcast peeps, here we go. Here's your first clinical pearl. Aflp. Acute fatty liver of pregnancy. Bad. Bad. It's bad. Okay. We're pretty much done. No, no, no. There's a lot more. There's a lot more. But the point is, respect this. I think we had an episode in the past. The help. Didn't we fear the help. Help is bad. An acute fatty liver of pregnancy is bad. Having elevated liver function in pregnancy, you got to figure out what's going on. I mean, this could be a hepatitis issue. Is it something with a torch infection panel? Where's this coming from? Is it a metabolic steatohepatitis? You know, the old fatty liver that's not an acute fatty liver, just fatty liver because of poor nutrition and typically the kind of fatty liver that goes with obesity. That's a different issue here. We're talking about acute fatty liver of pregnancy. That tends to happen in the third trimester and is generally a genetic deal. Okay, we're going to talk about it now. The good news is it is rare. I mean, we're talking about 1 in about 10,000 based on who you read or 1 in 20,000. But it's out there. I have seen it on more than one occasion. Would let you know that I've done this for a while and we've taken care of a lot of sick patients, mainly because we were referral sites. But this is real. So whether it's 1 in 10,000 or 1 in 20,000, it seems like it's just. You're never gonna see it. Trust me, it is out there. And you've gotta keep your eyes open and looking for it and always entertain this possibility, like our faculty did for our patient that we readmitted six days after her C section. A couple of quinky things to keep in mind. Some little weird facts about this. Much more common in twins and much more common in male fetuses than in pregnancies with female fetuses. Yeah, guys, we just like to make problems. So male fetuses are much more likely contribute to acute fatty liver of pregnancy, whether that is during the pregnancy or postpartum. Now, I mentioned that this is a genetic deal. Okay, now there's. That's absolutely true. This seems to be a genetic. A kink in the mitochondrial metabolism of long chain and medium chain fatty acids that leads to this disorganized byproducts of fatty acid metabolism. And there's absolutely a genetic mutation tied to this. Okay, now here's the weird kind of convoluted and tricky part. You can search for this mutation. I'm going to give you the letters here in a minute. It's very quick, okay? You can search for this and this mutation can either be in the maternal compartment, meaning mom actually has it, or it can actually be in the fetal compartment, meaning mom is otherwise fine. But because the baby carries this mutation, which affects mitochondrial fatty acid metabolism, the effect can then affect the mother. Okay, so this isn't just an issue that mom can have the mutation. Yes, that is true, but mom may not have it. And this can be because the child who she is carrying has the mutation. So it's either fetal or maternal derived. But here's the catch. There are some that you test, you check the baby, you check mom, and like, wow, crap, we don't find this mutation. By the way, that mutation is the letter G1528C. Alright, so G1528C. That's the specific mutation that leads to the altered mitochondrial fatty acid metabolism, that leads then to the altered byproducts of this that then accumulates in tissues and it affects the liver. It can also affect the kidneys and causes chaos because of mitochondrial fatty acid metabolism gone away. Okay, so this is the catch. If you look for it, great. You can put a checkbox and go, wow, we have this. This is a big deal. Although recurrence rates are unclear, if you can't. If the mother carries a mutation, it seems to be higher, but it's so rare that we actually can. We can't predict that it's x percent more likely to happen in a future pregnancy. Although if it happened once and the mother has the mutation, obviously it's more likely to repeat itself, although it's not 100%. So here's the catch though. You can check for this mutation, but just because the patient or the child are negative doesn't put them in the clear. Okay, that's why I said it's complicated. So when somebody asks you, is there a genetic cause to this? Yeah, no question. It's G1528C. And we've known that for some time. However, not having this mutation doesn't put the patient in the clear and it doesn't excuse the patient from not developing this because the diagnosis doses of this is made on clinical criteria, which we're going to cover. All right? There's a specific set of criteria here where you, if you meet six of these 15 things, it's pretty darn likely that they have AFLP, and we're going to talk about that. And if they meet the six criteria or more, it should obliviate or eliminate the need for liver biopsy, which Was the old standard. Right. Looking for liver biopsy, seeing if there's fatty accumulation in certain locations. And that was the distinction that the definitive diagnosis. But now we can do it clinically. And that's also one of the criteria. We'll talk about here in a minute, and I'll let you know what that looks like on histology in just a bit when we talk about the criteria. Okay, so can this be genetic? Yes. Do we know what the mutation is? Yes. G1528C. But even if they test negative, doesn't mean that the patient is clear. Okay, so now that we've set that, I just wanted to give a quick idea that this isn't very common. This is potentially a genetic issue. So now let's talk about how this can look like other conditions and where it gets a little gray here, like in our patient. Is it just preeclampsia with severe, or could this potentially be aflp? And once you know the criteria, you're like, nope, this is pretty clearly either preeclampsia with severe and. Or something else like HELLP syndrome or, you know, traditional hepatitis or. Holy crap. I think this is aflp and you gotta act fast. Okay? Don't delay a delivery here if it's antepartum, because delivery is part of the massive win here for this condition before they spiral out of control. Okay, But I thought this was interesting. Since the clinical picture of preeclampsia with severe features can also have liver pathology. This is a difficulty here trying to distinguish one from the other. So let's talk about this. First of all is the presentation. Now, I'm going to give you the specific criteria here that we use to do this. This is called the Swansea or the Swansea criteria. I've heard both. Guys, it's like tomato, tomato. All right, so I've heard some say Swansea, whatever, that's fine. Others have said Swansea. Now, again, I've had different ways to pronounce this, heard different ways to pronounce this, both in the US and abroad, but I've always known it as Swansea. So that is S, W, A, N, S, E, A. And by the way, that's not just an OB thing. Swansea criteria is well recognized in the world of gi, smfm, acog. I mean, Swansea is known to be out there. And six or more of this is 15 total criteria, has pretty darn good sensitivity and specificity for acute fatty liver of pregnancy. And we're going to talk about this. But in general, these patients look a little bit different than just preeclampsia with severe. These patients typically have other things going on. They kind of have this weird malaise. They can have abdominal pain, nausea, they can have jaundice, which is a big deal, guys, because preeclampsia with severe features don't turn your patient yellow. All right, so if your patient is jaundiced, they may very well have high blood pressure and something else. But you, first of all, you go, holy crap. Could this potentially be AFLP and. Or another form of hepatitis or whatever? All right, so yes, these patients typically present with some kind of general malaise, kind of a non specific, almost like a viral syndrome, a lot of nausea, muscle aches, but the jaundice is a big deal. These also are sicker than preeclampsia with severe features. These patients tend to have hypoglycemia, they have coagulopathy, they have renal dysfunction, and their ammonium can also be. Serum ammonium is also abnormally elevated, whereas preeclampsia with severe features, not so much. So, okay, that doesn't happen with regularly old hypertensive disorders or pregnancy, even with severe criteria. So the catch is AFLP in general has really bad elevated transaminases. In general, over a thousand. But they can be hanging out, like in our patient, around 900, which is why we entertained the idea with our patient. These patients also have high bilirubin levels. Again, as we mentioned, they have hypoglycemia, they can have elevated ammonium, and they also have. And here's the catch, guys. They've got messed up pt, ptt, and their fibrinogen is jacked. Okay? They've got coagulopathy, and that's part of the main features of the Swansea criteria. Okay, so we're going to get into this. You need six or more of these 15 things to say. I'm pretty darn sure this is AFLP. And we're going to break this up because the Swansea criteria is divided into symptoms, of course, lab criteria, ultrasound findings, and if done, if done, specific histology findings on biopsy, which is microvesicular steatytosis on liver biopsy. All you need is six of the 15. Six of the 15. All right, so let's get into this real quick here, guys, and then we're gonna start wrapping this up. I want this to be relatively quickly. So first on the symptoms again, stuff we've already discussed, they tend to be sicker with symptoms that don't really fit. Preeclampsia, we have severe or help. They have vomiting, they've Got weird abdominal pain. They've got encephalopathy. They can have polydipsia, confusion. These are the symptoms that are part of Swansea criteria. Let me read them directly so I don't misquote anything. Vomiting, abdominal pain, polydipsia or polyuria, encephalopathy. Those are the main symptoms that can be part of the Swansea criteria. Again, vomiting, abdominal pain, polydipsia, polyuria, encephalopathy. On labs, as you would guess, there's elevated bilirubin, there's hypoglycemia, there's elevated uric acid. There can be leukocytosis. There's of course, elevated transaminases. Hello. There's an elevated ammonium level. There can be renal impairment and coagulopathy. That's both PT or PTT that can be affected. So we've talked about symptoms, we've talked about labs. Ultrasound is another part of the Swansea criteria. On ultrasound, there can be ascites or the liver that shows up pretty bright on ultrasound. Okay. So it has a different homogeneous texture. It appears very hyperechoic. That is a soft marker. And that's one of the criter on Swansea using ultrasound. And then, of course, the 15th marker or thing on the list on the criteria is that there is microvesicular steatosis on liver biopsy. But again, I haven't done liver biopsy or ordered one of these liver biopsies in like 20 years because the criteria is pretty darn close, right? It's pretty darn tight. And if they have six or more of this, it makes the diagnosis pretty darn clear. Okay, so now that we've covered that, why don't we take a quick break? So let that sit there. Six out of the 15 of the Swansea criteria, which is endorsed by multiple professional societies and disciplines for acute fatty liver pregnancy. When we come back very quickly, just going to start wrapping this up. We're going to talk about the management here. What do we do with this? And it's very directed. And then here's the other question we're going to ask as we wrap this up. Is plasmapheresis a thing? The answer may surprise you. We'll be right back.
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Doctor/Physician
We know this is, excuse me, a damn fine cup of coffee I've had. I can't tell you how many cups of coffee in my life.
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Dr. Chapa
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- [22:01]
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Dr. Chapa
I think I've said this before, but if I did it, I'm gonna tell you now about how you know funny ways that we pronounce things. You know, Swansea. Swansea. Whatever, man. Just know what it is and know how to reference it. I don't care how you pronounce it. I just want you to know it. I told you I went through my first two years of medical school at a good school. I mean, I'm proud of, I'm proud of my medical school. It's UT Southwestern. I thought it was very well prepared, prepared for the real world. Tough school. Tough school. But I learned the condition post viral of progressive neuropathy as Gillian Barr. Okay, Gillian Barr. Because that's what my professor did, who was a very well published, very well respected immunologist. It was Gillian. We got Gillian Barr, of course, because that's how we pronounce things in Texas. And then it wasn't until later on when I figured out, oh my gosh, it's actually Guillaume Barre. Guillaume Barre sounds nothing like Gillian Barr. All right, so it tells you how in Texas, because we got our own way of saying things. So whether you want to call it Swansea, which I've heard, or Swansea, I don't care. Just know the criteria. You need 6 of 15 and that's the important thing. Gillian Barr. Can you believe it? Guillain Barre. Guillain Barre. Okay, so management of acute fatty liver pregnancy. Yes, this can happen late in the third trimester. It can also happen postpartum, which is why again, we entertain this thought, this possibility with our patient who has a bounce back admission. And we're still trending. Our goal is every day we're going to do a set of labs to make sure that she's doing fine and that we don't deteriorate into help, because this is much more likely to be a Preeclampsia with severe features than acute fatty liver. Now, the big discriminator here, outside of the Swansea. Swansea criteria, is that if the patient has high blood pressure, if she has typical symptoms of preeclampsia and she has protein in her urine, that's pretty much preeclampsia. We have severe features. The presence of hypertension, classic findings of preeclampsia and urine protein makes a diagnosis of preeclampsia with severe. We get that. That's pretty straightforward. But always on the back of your mind, as our team did today, always have the possibility, could this be somehow underlying acute fatty liver of pregnancy? And that's where the Swansea or Swansea criteria come into play. Six of the 15, pretty darn good at making the distinction. Okay? And remember, those things don't. Some things don't live in isolation. I'm not saying that you have to have one or the other. It's possible to have both. But that's why you have to keep your eyes open to these possibilities. Hypoglycemia is not normal with preeclampsia with severe features unless you took too much insulin or metformin or something else. Having high ammonium is not part of preeclampsia with severe features. So these are the things that distinguish one from the other. Confusion. Encephalopathy is not really part of preeclampsia with severe features unless she's postictal from her eclampsia. Okay, so you see how the two, they can be confusing. Guys, this is why I thought this was a good episode, because we've got to keep your eyes open for this. Acute fatty liver pregnancy is much less common than the more typical preeclampsia with severe. But keep that in the back of your mind. So now that we've said that, what do we do with this? Well, if you really do suspect acute guy delivered pregnancy and he's antepartum, you got to separate the two, okay? Now, ideally, of course, Holy Child is very viable. Viable like in the little Chicago Med clip that we played at the beginning. But prompt delivery is key here. You're not going to put this patient through a prolonged induction. Okay? So if you find this and she just happens to be crowning, that's one thing. But in general, these are delivered by cesarean section. Once you have ruled out active acute coagulopathy. And if there is coagulopathy, you've got to correct that and work together with your OB anesthesiologist or your crna, whatever, okay? This needs multidisciplinary attention. Typically, this is, you can have a GI or hepatologist, mfm, perinatology, neonatology based on if the baby's delivered or not, and of course, anesthesia. But here's the catch. Prompt delivery is game changing here. For some reason, removal of the placenta from the mama helps the recovery immensely. However, it's not that quick. Now they stabilize, but they may not have complete reversal of the condition for days to a couple of weeks after. Okay, so this is something you got to keep trending because they can still decompensate once you deliver. As long as they are not deteriorating. We keep these for a prolonged time. Now, we're not going to keep them for a month, but we keep them quicker than just, hey, you're good, you're 24 hours off mag or whatever. You got to keep an eye on these because you got to make sure that they're stable and hopefully turning the corner. Although it can take some weeks to do that, rarely, rarely does this lead to true hepatic failure. It has happened, but that's rare. Or liver rupture. And rarely, rarely do these patients need liver transplant. All right, they mentioned that in the Chicago Med episodes, she needs a liver transplant. Really, Bruh? Really? That's extremely rare. There is some data that plasmapheresis in those who have already had delivery and are just not getting better and. Or specifically if they're decomposing, compensating, in other words, refractory cases. There is some evidence that plasmapheresis can help remove those oxidative byproducts of this fatty acid metabolism and can help stabilize the patient. But that is not the first line. So if you're asked, what's the treatment for acute fatty liver, the first thing is, is she still pregnant? Because if she's still pregnant, she needs to not be. Okay, so she has to have prompt delivery to separate the two because that's game changer. If she is, then you gotta watch her because that's even harder. And potentially approach this by a multidisciplinary team. Correct any coagulopathy. And if she's not getting better and or deteriorating, consider plasmapheresis. There is systematic reviews that have looked at this and said, yeah, it's definitely not first line. It's got its own risks, but it can definitely improve survival and help biochemical changes reduce the stress on the body from the byproducts of the altered fatty acid metabolism. Okay, so potentially, yes, you could do plasmapheresis in these patients, but it's not the norm. So podcast family, we've covered this pretty quickly. There's a lot of data on this. Both the Preeclampsia foundation has this SMFM has stuff on it. I think it's mentioned briefly in the preeclampsia guidance from the college. And of course we'll leave some good references here in our show notes for this episode. So anyway, I thought this was super interesting because this goes to show how, you know, we get podcast ideas from real world issues and we applied this hey, when we had this conversation. Could this be acute fat deliverable? It's very easy. Is she hypoglycemic? Is she coagulopathic? Because coagulopathy is not typical preeclampsia with severe and neither is altered glucose metabolism. And then check her ammonia. How does the ammonium look? And in this case, the biggest factor here that pointed towards hypertensive disorder was the obvious she was hypertensive frontal headache. It looked like preeclampsia. It smelled like preeclampsia with severe features. It tasted like preeclampsia with severe features. All right, the tasted part was kind of weird, but you get what I'm saying. But AFLP versus preeclampsia with severe features or help can be a clinical conundrum. And I hope we've done it justice. Podcast family, as always, we're thankful for you. Thanks for being part of our podcast community. And now that we've done all that, let's take it home. Foreign this has been Dr. Chapa Zobetyn, no Spin Podcast Podcast Family. Thank you for your support. Thank you for listening. And as always, we'll see you on another episode of the no Spin Podcast. Sa.