{"title":"Preoperative evaluation of biliary atresia.","authors":"Isabelle Lane Curran, Robert A Cowles","doi":"10.1016/j.sempedsurg.2025.151475","DOIUrl":"https://doi.org/10.1016/j.sempedsurg.2025.151475","url":null,"abstract":"<p><p>Biliary atresia can be a challenging diagnosis to make as there is no single definitive diagnostic laboratory or imaging study available and no single agreed upon diagnostic algorithm. The purpose of this article is to review the complex puzzle of clinical, laboratory, and imaging studies that aid in the evaluation of infants suspected of having biliary atresia. We have reviewed historical and current manuscripts and society guidelines, added our own experience in evaluating infants for biliary atresia, and then summarized the findings to provide a concise review of what we feel is the modern approach to diagnosis of biliary atresia.</p>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"151475"},"PeriodicalIF":1.4,"publicationDate":"2025-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143076232","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Predicting and managing liver fibrosis in biliary atresia.","authors":"Iiris Nyholm, Maria Hukkinen, Mikko P Pakarinen","doi":"10.1016/j.sempedsurg.2025.151473","DOIUrl":"https://doi.org/10.1016/j.sempedsurg.2025.151473","url":null,"abstract":"<p><p>Regardless of the underlying etiology and success of PE, progressive liver fibrosis and eventually cirrhosis represent the dominant pathology and the end-stage of BA. Ascending bile duct injury-induced cholestasis, inflammation and ductular reaction provide profibrogenic cytokine environment leading to myofibroblast activation and rapid progression of fibrosis especially after unsuccessful portoenterostomy. Although liver fibrosis and development of cirrhosis play a crucial role in determining BA outcomes, the exact prognostic significance and dynamics of mild to moderate liver fibrosis remain unclear. Manual scoring systems categorizing the degree of liver fibrosis are prone to intra- and interobserver variability, whereas novel combinations of digital pathology with artificial intelligence quantification can provide accurate information on fibrosis structure and dynamics at the level of individual collagen fibers. Although several studies have analyzed noninvasive assessment of fibrosis at time of PE, including imaging-based elastography and different serum biomarkers, current knowledge on their accuracy during the postoperative follow-up of BA is scarce. While therapeutic management of liver fibrosis in BA remains in its infancy, the resolution potential for liver fibrosis has been demonstrated after successful PE. Achievement of effective antifibrotic treatment may require combination of different therapies with complementary modes of action like anti-inflammatory medication, antioxidants and bile acid lowering agents.</p>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"151473"},"PeriodicalIF":1.4,"publicationDate":"2025-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143069169","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Immunology of Biliary Atresia.","authors":"Phoebe N Miller, Suruthi Baskaran, Amar Nijagal","doi":"10.1016/j.sempedsurg.2025.151474","DOIUrl":"10.1016/j.sempedsurg.2025.151474","url":null,"abstract":"<p><p>Biliary atresia is a progressive neonatal cholangiopathy that leads to liver failure. Characterized by inflammation-mediated liver injury, the immune system plays a critical role in the pathogenesis of this disease. Though several types of immune cells and mediators have been implicated in animal models of biliary atresia, emerging literature reflects the complex interplay of components of the immune response that contributes to disease progression in humans. Novel therapies targeting the immune system are needed to mitigate the devastating effects of biliary atresia. This review highlights the current literature on the components of the immune system that have been in implicated in biliary atresia and the rich interplay between the major arms of the immune system- innate and adaptive immunity- to cause the highly morbid consequences of this disease.</p>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"151474"},"PeriodicalIF":1.4,"publicationDate":"2025-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143043071","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Qiongfen Lin, Paul Kwong-Hang Tam, Clara Sze-Man Tang
{"title":"Genetics of biliary atresia: Approaches, pathological insights and challenges.","authors":"Qiongfen Lin, Paul Kwong-Hang Tam, Clara Sze-Man Tang","doi":"10.1016/j.sempedsurg.2025.151477","DOIUrl":"https://doi.org/10.1016/j.sempedsurg.2025.151477","url":null,"abstract":"<p><p>Biliary atresia (BA) is a severe neonatal cholestatic disorder marked by fibro-obliteration of the extrahepatic and intrahepatic bile ducts. It is the most common cause of pediatric end-stage liver disease and the leading indication for liver transplantation in children. There is significant heterogeneity in the etiology, involving various genetic and environmental factors such as viral infection, immune dysregulation and genetic predisposition to defective hepatobiliary development. In this review, we discuss the strategies to uncover the genetic factors underlying BA and highlight their associated molecular and pathological mechanisms, as well as the challenges faced in this area of research.</p>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"151477"},"PeriodicalIF":1.4,"publicationDate":"2025-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143043070","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Post-Kasai cholangitis evaluation and management strategies: Review of the literature with insights from the Swiss Biliary Atresia Registry.","authors":"Ana M Calinescu, Barbara E Wildhaber","doi":"10.1016/j.sempedsurg.2025.151471","DOIUrl":"https://doi.org/10.1016/j.sempedsurg.2025.151471","url":null,"abstract":"<p><p>Cholangitis, defined as the inflammation of the bile ducts, is the most frequent complication after Kasai hepatoportoenterostomy in patients with biliary atresia (BA). This review seeks to provide a comprehensive synthesis of current knowledge on diagnosing and managing BA-associated cholangitis while identifying gaps in the existing literature. A scoping literature review was conducted to gather global insights into the definition, evaluation, and management of post-Kasai cholangitis, illustrated through data from the Swiss Biliary Atresia Registry (SBAR). In the literature many different decision algorithms exist for BA-associated cholangitis underlining the clear need for standardizing diagnostic criteria. As an illustration, in 2015 the Swiss center introduced structured guidelines to diagnose BA-associated cholangitis. Upon retrospective analysis of SBAR data (2010-2022) we noted significant differences in disease management before and after implementation: The number of multiple diagnosed cholangitis episodes was reduced and we observed a reduced transplantation rate. Building on this, the Biliary Atresia and Related Diseases consensus has developed key diagnostic and management guidelines with objective criteria that have yet to undergo prospective validation. Indeed, prospective multicenter assessment is clearly needed to enhance acceptance and improve outcomes. Further, rigorous evaluation of prophylactic and therapeutic interventions, including antibiotics and immunomodulatory treatments, seems essential, with randomized trials required to establish their effectiveness and explore microbiota modulation in cholangitis management. The BA-community should strive to establish and adopt a widely accepted protocol for the diagnosis, management and prevention of BA-associated cholangitis. The implementation of such protocols is expected to significantly improve clinical outcomes for these patients.</p>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"151471"},"PeriodicalIF":1.4,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143043072","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Predicting and optimising outcome for biliary atresia.","authors":"Renos M Jeropoulos, Jorge Arroyo, Mark Davenport","doi":"10.1016/j.sempedsurg.2025.151479","DOIUrl":"https://doi.org/10.1016/j.sempedsurg.2025.151479","url":null,"abstract":"<p><p>Biliary atresia (BA) remains a disease of significant morbidity and mortality world-wide. Early and accurate diagnosis facilitates early intervention and improves outcomes. The gold standard in diagnosing BA is a liver biopsy followed by cholangiography, usually performed intra-operatively. Serum markers, like the aspartate aminotransferase-to-platelet ratio, matrix metalloproteinase-7 and several inflammatory cytokines have been recently investigated as non-invasive alternatives with varying degrees of success. Newer immunohistochemical analysis of liver biopsies, such as the expression of secretin receptors and Ki-67, from infants with BA have improved our understanding of the disease process and has shed a little light in predicting post-operative outcomes. There is little standardisation in the care of BA post operatively, though administration of steroids, prevention and treatment of cholangitis with antibiotics and anti-viral therapy for CMV+ve infants are becoming universally accepted as treatment. Experimental stem cell treatments show promise although remain in the out-of-reach future for now in routine clinical practice. This chapter aims to comprehensively describe recent knowledge on predicting the clinical outcomes of infants with BA, as well as optimising their care post operatively.</p>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"151479"},"PeriodicalIF":1.4,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143069170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Revision Kasai portoenterostomy: A review of indications and outcomes.","authors":"Alyssa Stetson, Alexander Bondoc, Greg Tiao","doi":"10.1016/j.sempedsurg.2025.151476","DOIUrl":"https://doi.org/10.1016/j.sempedsurg.2025.151476","url":null,"abstract":"<p><p>The Kasai portoenterostomy (KPE) can provide a surgical cure for children with biliary atresia (BA), without the need for a liver transplant (OLTxp). Revision KPE can be attempted following a failed initial KPE where biliary clearance is not achieved. The most common indications for revision KPE are recurrent jaundice or recurrent cholangitis, although it has also been performed for persistent jaundice or bile lakes. Outcomes are heterogenous but the best results appear to be with recurrent jaundice or limited episodes of recurrent cholangitis. In the setting of a failed KPE, providers must make a patient-specific decision about whether to attempt revision KPE versus proceed with OLTxp. While the choice is multifactorial, patients who undergo revision KPE likely do not have worse long-term outcomes than patients who undergo a single KPE.</p>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"151476"},"PeriodicalIF":1.4,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143069171","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Brynn FitzGerald , Krishna Jagannathan , Nicholas Burjek , Matthew Rowland
{"title":"The development and benefits of a pediatric airway response team in a children's hospital","authors":"Brynn FitzGerald , Krishna Jagannathan , Nicholas Burjek , Matthew Rowland","doi":"10.1016/j.sempedsurg.2024.151453","DOIUrl":"10.1016/j.sempedsurg.2024.151453","url":null,"abstract":"","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 5","pages":"Article 151453"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142441509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Eric C. Cheon , Mehul V. Raval , Alexander Froyshteter , Hubert A. Benzon
{"title":"Utilizing national surgical quality improvement program-pediatric for assessing anesthesia outcomes","authors":"Eric C. Cheon , Mehul V. Raval , Alexander Froyshteter , Hubert A. Benzon","doi":"10.1016/j.sempedsurg.2024.151454","DOIUrl":"10.1016/j.sempedsurg.2024.151454","url":null,"abstract":"","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 5","pages":"Article 151454"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142434371","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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