Journal of Pediatric Article Review: June 2023, AAP Issue

Welcome back, everyone. We are back with another episode of the Stay Current podcast. I'm Cecilia Gigena and I'm M. Tom Bash. And we are research fellows from Cincinnati Children's Hospital, and along with Stay Current, we are sharing knowledge to improve child health around the globe. And so today we have another episode of the JPS podcast. We have June episode this week, and this is the AAP issue. AAP stands for American Academy of Pediatrics. Great. And for this issue, we talked to Dr. Colin Martin. He is the publication chair and the editor of this issue. Hello, my name is Colin Martin. I'm a pediatric surgeon and the division chief of pediatric Surgery at Washington University in St. Louis, and I'm also the chair of the Publications Committee for the AAP. And he selected 3 articles. The first one is about the disruption of the enterohepatic circulation in patients with short bowel. For the second one, we have the pursuit of no evidenced disease status in hepatoblastoma, and last but not least, the surveillance in esophageal atricia patients. As always, you can find the articles in the description below so you can follow along with us. So, let's get started. This first article for today is one from Basic Clinical, so it's a little bit more complicated of what we are used to, but let's give it a try. This paper is disruption of enterohepatic circulation of bile acids ameliorates small bowel receptor. associated hepatic injury, and this is from Barneshus Hospital from Saint Louis, Missouri. In this paper, they wanted to elucidate the driving force behind the hepatic injury in patients with short bowel syndrome. And for this, we talked with Doctor Brad Warner. He's the senior author of this paper. Yeah, I'm Brad Warner. Uh, I'm the chief of pediatric surgery at Saint Louis Children's Hospital. And professor of surgery at Washington University School of Medicine in St. Louis. They wanted to see what happened with the entire hepatic circulation and the bile acids in different short bowel resections. In looking for mechanisms as to how this occurs, we sought to determine whether bile acids or the site of intestinal resection would really make a difference in terms of the liver injury. And so for that they study a group of mice. Half of them had a proximal resection, half of them had a distal resection, and what they realized actually is that patients that had a distal resection that we thought was. Super prejudicial and bad for the patients, actually had a kind of protective effect on the liver injuries. In the latest study, they revealed that proximal small bowel resection results in a greater oxidative stress in the liver, as evidenced by elevated messenger RNA transcripts for tumor necrosis factor A. Additing denucleotide phosphate oxidase and glutathione synthase with a greater compensatory antioxidant response. And that was Doctor Colin Martin, the editor that helped us choose these articles. Two takeaways here, one of which is the ileocecal region. May not be important to preserve and may actually be injurious. And the second point is that administration of a specific bile acid that is more um uh lipophilic and, uh, and more hepatic, uh, I guess, uh, protective could be potential therapy for patients that have cholestatic liver disease after a massive intestinal resection. And here's what Todd had to say. This is a great study, but this is just in mice. So, obviously, the next thing that needs to be done is a, a clinical assessment. There are a lot of factors that are obviously in play here. Uh, we can choose which segment of intestine to remove. In patients, you can't choose that. You have to remove what's dead, and you intentionally would never remove anything that wasn't. You want to preserve as much and And I think that's really important to highlight. So, I think what we learned from here though, is what's the absolute physiology that occurs when you remove the ileocecal valve. If the preservation of the ileocecal region is not as beneficial as we thought, this is a really groundbreaking discovery. Like, it changed every pediatric surgery mindset. So now we're ready for the next paper, so kill it, um. The next paper is aggressive pursuit of no Evidence of Disease status and hepatoblastoma Improved Survival, and Observational Study. This study is from Saint Jude Children's Hospital in Memphis, Tennessee. And for this paper, we talked to the first author. My name is Andrew Fleming, and I'm a surgery resident with the University of Tennessee Health Science Center in Memphis, Tennessee. Um, I'm currently completing a two-year postdoctoral research fellowship at Saint Jude Children's Research Hospital, uh, under the mentorship of Doctor Andrew Murphy. This project, I was also fortunate enough to be mentored by the senior author, uh on this paper, Doctor Max Langham. So in this paper, authors examined the effect of aggressive pursuit of no evidence of disease status on like event-free and overall survivor in hepatoblastoma. They put together the records of patients with hepatoblastoma from 2005 to 2021. Their primary outcomes were, Overall survivor and event-free status and no evidence of disease status. There were 50 patients that were enrolled from 2005 to 2021. 46 of the 50 underwent resection with the use of adjuvant techniques including total hepatic vascular exclusion, vascular reconstruction. Biliary reconstruction or end-block resection and total hepatectomy with transplantation, and the remarkable thing is that of these patients, 82%, 41 out of 50, were able to achieve NED status. And he's Doctor Martin. He helped us to pick these articles for this issue. According to their data, 14 high-risk patients underwent a median of 2.5 pulmonary metastatectomies, 7 for unilateral disease. 7 for bilateral disease with a median of 4.5 nodules resected and 5 high risk patients in total relapsed and 3 of them were saved and this this natives of disease or NED status were inversely correlated with 5 year mortality. Once no evidence of disease status was achieved, the 10 year overall survival. Among patients that were high risk from the get-go was actually similar to patients that were not high risk, which I think again kind of emphasizes that if you are able to get children to that point, they can have long-term survival despite being stratified into a very high-risk group from the beginning. How hard to reach that no evidence of disease status? Because you need to get chemotherapy, radiotherapy, so it's not easy to get that disease status, but once you get them, it seems like it affects the long-term survival in a very like positive way, right? So, I think that the outcome of high-risk patients to have a better long-term survival and also the status, like event-free status. It's really important. I think again, just kind of the biggest takeaway is there is no threshold for disease burden that precludes you from aggressively trying to achieve no evidence of disease uh in children with hepatblastoma. And that was Doctor Fleming. He's the first author of this paper. Yes, again, I found it super interesting cause it's really hard when you're doing like super aggressive surgeries to Young children to decide whether that it's worth it or not, and seeing that this is worth it, I think it's huge. We always try to balance aggressiveness with With outcomes, and cause the, the pendulum keeps swinging. We, Halstead was incredibly aggressive, and, and the Halsteadian approach was go big, and then we got minimally invasive, and now we're trying to find this balance of aggressiveness and survival, and sometimes you unfortunately do have to be aggressive. So, it's an important paper. Are you ready for the 3rd paper? Yeah, let's jump. So for the last paper of the day, we have esophageal surveillance practices in esophageal attricia patients, a survey by the Eastern Pediatric Surgery Network. This paper coming out of the Eastern Pediatric Surgery Network. First author is Malcolm Hamilton Hall, the 3rd, senior author is Doctor J. Leslie Nodd, and that was Doctor. Colin Martin, and what they did is they wanted to see the guidelines after Nestofael Patricia repair and how the the experts did it. So they did a survey, a 23 question survey for 181 physicians at 19 institutions. There was a 77% response rate, and the interesting thing is that many institutions, um. Um, although they had air digestive teams, um, only 36% claimed to perform routine upper GI endoscopy regardless of symptoms, so a small number, despite the evidence showing that these patients should be surveilled. And in many institutions, the digestive clinic was the one who followed them. And we, we can see in this paper that physicians agreed with the current guidelines, and they believe that yes, we should do this. But how many of them are actually doing it? And so the paper just kind of points to lack of either lack of consensus or lack of adherence to a consensus guideline about these patients, and these patients, because of our advanced techniques, they're surviving, but now these patients are growing into adulthood. And without proper surveillance, they could be a risk for other problems, esophageal cancer, chronic dys motility, etc. This survey actually brought us a like a main problem and every pediatric surgeon probably knows that um we need to follow up. be more strict with the guidelines, but we're not, and I don't know if the survey can tell us what is the reason with that weak adherence or a weak commitment to the guideline, maybe. What do you think is the problem with this like lack of commitment to a guideline with the surveillance? I think that maybe not having something that's really proved to be working and more like Like as if they're not evidence-based. And they are more like. Institutional guidelines, maybe that's why they don't follow it as much. Um, I mean, I'm just guessing, it's not a real thing. But I think that, that maybe if we have some well-designed guidelines, they will follow it more. Let's not think about just pediatric surgeons who are not like uh committing enough to it like a protocol, but like how hospital is supporting them to commit that protocol because it's all, at the end of the day, it's all like a system problem, sounds like it. And here's what Todd had to say. I think this is great. So, the, the surgeons believe it should be done. But they're not doing it, and the way to get it done is to have a formal aerodigestive team. OK. So, that was our June JPS podcast. We talked about first, the disruption of the enterohepatic circulation in patients with short bowel, and we actually discovered that keeping the ocecal valve may be not as beneficial as we thought. And then we talk about the pursuit of no evidence disease in hepatoblastoma and we realized that actually in this, it's really important to achieve that for the long survival. In these patients. And last, we talked about the surveillance in esophageal attricia patients and how we really need an evidence-based guidelines to be more Aware of it and, and to follow the guidelines that we have. So, I think it was an awesome issue. If you like this episode, give us a rating on wherever you listen to your podcasts. Don't forget to subscribe to our YouTube channel, follow us on social media, and download the Stay Current app for hundreds of pieces of content. I'm I'm Tom Bash and I'm Cecilia Jigena, and we are research fellows at Cincinnati Children's Hospital, and along with Stay Current, we are sharing knowledge to improve child health around the globe.