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Biliatresone:progress in Biliary atresia study

World Journal of Pediatrics 2023年第5期19卷 417-424页

作者： Jia‑Jie Zhu Yi‑Fan Yang Rui Dong Shan Zheng Department of Pediatric Surgery Shanghai Key Laboratory of Birth Defectand Key Laboratory of Neonatal DiseaseMinistry of HealthChildren’s Hospital of Fudan University399 Wan Yuan RoadShanghai 201102China

Background Biliary atresia(BA)is one of the main causes of neonatal end-stage liver disease.Without timely diagnosis and treatment,most children with BA will develop irreversible liver fibrosis within the first two months.While current theorized causes of BA include viral infection,immune disorders,and genetic defects,the comprehensive etiology is still largely unknown.Recently,biliatresone attracted much interest for its ability to induce BA in both zebrafish and mice,so we summarized the latest progress of biliatresone research in BA and tried to answer the question of whether it could provide further clues to the etiology of human BA.Data sources We conducted a PubMed search for any published articles related to the topic using search terms including“Biliary atresia”,“biliatresone”,“GSH”,and“HSP90”.Relevant data were extracted from the original text or supplementary materials of the corresponding articles.Results Biliatresone had shown its unique toxicity in multiple species such as zebrafish and mice,and pathogenic factors involved included glutathione(GSH),heat shock protein 90(HSP90)and the related pathways.In combination with epidemiological evidence and recent studies on the intestinal flora in Biliary atresia,a new pathogenic hypothesis that the occurrence of Biliary atresia is partly due to biliatresone or its structure-like compounds depositing in human body via vegetables or/and the altered intestinal flora structure can be tentatively established.Conclusions Based on the existing evidence,we emphasized that GSH and HSP90 are involved in the development of BA,and the maternal diet,especially higher vegetable intake of Asian women of childbearing age,accompanied by the altered intestinal flora structure,may contribute to the occurrence of Biliary atresia and the higher incidence in the Asia group.However,the evidence from large sample epidemiological research is necessary.

关键词： Biliary atresia Biliatresone Glutathione Gut flora

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SOX9 in Biliary atresia: New insight for fibrosis progression

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Hepatobiliary & Pancreatic Diseases International 2021年第2期20卷 154-162页

作者： Hanaa Ahmed El-Araby Magdy Anwar Saber Noha Mohamed Radwan Doha Maher Taie Nermin Mohamed Adawy Ahmad Mohamed Sira Department of Pediatric Hepatology Gastroenterologyand NutritionNational Liver InstituteMenoufia University32511 Shebin El-koomMenoufiaEgypt Department of Pathology National Liver InstituteMenoufia University32511 Shebin El-koomMenoufiaEgypt

Background:Liver fibrosis is a hallmark determinant of morbidity in Biliary atresia(BA)even in successfully operated cases.Responsible factors for this rapid progression of fibrosis are not completely defined.Aberrant expression of the transcription factor SOX9 and hepatic progenitor cells(HPCs)proliferation have roles in fibrogenesis in cholestatic disorders.However,they were not investigated sufficiently in BA.We aimed to delineate the relation of SOX9 and HPCs to fibrosis and its progression in BA.Methods:Forty-eight patients with BA who underwent an initial diagnostic liver biopsy(LB)and consequent intraoperative LB were recruited and compared to 28 cases with non-BA cholestasis that had an LB in their diagnostic workup.Liver fibrosis,tissue SOX9 and HPC expressions were studied in both BA and non-BA-cholestasis cases.Liver fibrosis,SOX9,and HPCs’dynamic changes in BA cases were assessed.Relation of fibrosis and its progression to SOX9 and HPCs in BA was assessed.Results:SOX9 and HPCs in ductular reaction(DR)form were expressed in 100%of BA and their grades increased significantly in the second biopsy.The rapidly progressive fibrosis in BA,represented by fibrosis grade of the intraoperative LB,correlated significantly to SOX9-DR and HPC-DR at the diagnostic(r=0.420,P=0.003 and r=0.405,P=0.004,respectively)and the intraoperative(r=0.460,P=0.001 and r=0.467,P=0.001,respectively)biopsy.On the other hand,fibrosis,SOX9-DR,and HPC-DR were significantly lower in non-BA cases at a comparable age(P<0.001,P=0.006,and P=0.014,respectively).Conclusions:Fibrosis in BA is rapidly progressive within a short time and is significantly correlated to SOX9 and HPCs.Assessment of targeting SOX9 and HPCs on fibrosis progression is warranted.

关键词： Biliary atresia Fibrosis Hepatic progenitor cells Immunohistochemistry Neonatal cholestasis SOX9

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Evidence for Viral Induction of Biliary atresia: A Review

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Journal of Clinical and Translational Hepatology 2018年第4期6卷 410-419页

作者： Leon D.Averbukh George Y.Wu Department of Medicine Division of Gastroenterology-HepatologyUniversity of Connecticut Health CenterFarmingtonCTUSA

Biliary atresia(BA)is a childhood disease which manifests with abnormal narrowing,blockage or complete absence of bile ducts within the liver.Many possible etiologies have been reported for the development of BA,including congenital,perinatal and acquired conditions.Since the 1970's,there has been increasing evidence linking BA development to viral perinatal infections.The viral vectors most commonly implicated include members of the herpesviridae family(cytomegalovirus and Epstein-Barr virus)as well as those of the reoviridae family(reovirus and rotavirus).While extensive work has been done on a murine model of disease,the current review focuses primarily on evidence from human studies of viral vectors in children afflicted with BA.

关键词： Biliary atresia Cytomegalovirus Epstein-Barr virus Reovirus Rotavirus

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Clinical Assessment of Differential Diagnostic Methods in Infants with Cholestasis due to Biliary atresia or Non-Biliary atresia

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Journal of Huazhong University of Science and Technology(Medical Sciences) 2018年第1期38卷 137-143页

作者：董琛朱慧云陈云超罗小平黄志华 Department of Pediatrics Tongji HospitalTongji Medical CollegeHuazhong University of Science and Technology Department of Ultrasound Tongji HospitalTongji Medical CollegeHuazhong University of Science and Technology

The different methods in differentiating Biliary atresia（BA） from non-BA-related cholestasis were evaluated in order to provide a practical basis for a rapid,early and accurate differential diagnosis of the diseases.396 infants with cholestatic jaundice were studied prospectively during the period of May 2007 to June 2011.The liver function in all subjects was tested.All cases underwent abdominal ultrasonography and duodenal fluid examination.Most cases were subjected to hepatobiliary scintigraphy,magnetic resonance cholangiopancreatography（MRCP） and a percutaneous liver biopsy.The diagnosis of BA was finally made by cholangiography or histopathologic examination.The accuracy,sensitivity,specificity and predictive values of these various methods were compared.178 patients（108 males and 70 females with a mean age of 58±30 days） were diagnosed as having BA.218 patients（136 males and 82 females with a mean age of 61±24 days） were diagnosed as having non-BA etiologies of cholestasis jaundice during the follow-up period in which jaundice faded after treatment with medical therapy.For diagnosis of BA,clinical evaluation,hepatomegaly,stool color,serum gamma-glutamyltranspeptidase（GGT）,duodenal juice color,bile acid in duodenal juice,ultrasonography（gallbladder）,ultrasonography（griangular cord or strip-apparent hyperechoic foci）,hepatobiliary scintigraphy,MRCP,liver biopsy had an accuracy of 76.0%,51.8%,84.3%,70.0%,92.4%,98.0%,90.4%,67.2%,85.3%,83.2% and 96.6%,a sensitivity of 83.1%,87.6%,96.1%,73.7%,90.4%,100%,92.7%,27.5%,100%,89.0% and 97.4%,a specificity of 70.2%,77.5%,74.8%,67.0%,94.0%,96.3%,88.5%,99.5%,73.3%,75.4% and 94.3%,a positive predictive value of 69.0%,72.6%,75.7%,64.6%,92.5%,95.7%,86.8%,98.0%,75.4%,82.6% and 98.0%,and a negative predictive value of 83.6%,8.5%,95.9%,75.7%,92.3%,100%,84.2%,93.7%,100%,84.0% and 92.6%,respectively.It was concluded that all the differential diagnosis methods are useful.The test for duodenal drainage and elements is fast and accurate.It is helpful in the differential diagnosis of

关键词： neonatal cholestasis Biliary atresia non-Biliary atresia etiologies of cholestasis jaundice

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Novel NIR-Ⅱfluorescent probes for Biliary atresia imaging

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Acta Pharmaceutica Sinica B 2023年第11期13卷 4578-4590页

作者： Xiaodong Zeng Yuqin Liao Xue Qiao Ke Liang Qiusi Luo Mingbo Deng Yishen Liu Weijing Zhang Xuechuan Hong Yuling Xiao State Key Laboratory of Virology Department of CardiologyZhongnan Hospital of Wuhan UniversitySchool of Pharmaceutical SciencesWuhan UniversityWuhan 430071China State Key Laboratory of Drug Research&Center of Pharmaceutics Shanghai Institute of Materia MedicaChinese Academy of SciencesShanghai 201203China Shandong Laboratory of Yantai Drug Discovery Bohai Rim Advanced Research Institute for Drug DiscoveryYantai 264117China Key Laboratory of Biodiversity and Environment on the Qinghai-Tibetan Plateau Ministry of EducationSchool of Ecology and EnvironmentTibet UniversityLhasa 850000China Shenzhen Institute of Wuhan University Shenzhen 518057China School of Pharmacy Yantai UniversityYantai 264005China School of Biomedical Engineering Shanghai Jiao Tong UniversityShanghai 200240China

Biliary atresia is a rare infant disease that predisposes patients to liver transplantation and death if not treated in time.However,early diagnosis is challenging because the clinical manifestations and laboratory tests of Biliary atresia overlap with other cholestatic diseases.Therefore,it is very important to develop a simple,safe and reliable method for the early diagnosis of Biliary atresia.Herein,a novel NIR-Ⅱfluorescence probe,HZL2,with high quantum yield,excellent biocompatibility,low cytotoxicity and rapid excretion through the liver and gallbladder was developed based on the oil/water partition coefficient and permeability.A simple fecal sample after injection of HZL2 can be used to efficiently identify the success of the mouse model of Biliary atresia for the first time,allowing for an early diagnosis of the disease.This study not only developed a simple and safe method for the early diagnosis of Biliary atresia with great potential in clinical translation but also provides a research tool for the development of pathogenesis and therapeutic medicines for Biliary atresia.

关键词： Biliary atresia Fluorescence imaging Second near-infrared window Early diagnosis Hepatobiliary excretion

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Post-hepatoportoenterostomy Acoustic Radiation Force Impulse Elastography to Predict Two-year Outcome of Biliary atresia

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Journal of Clinical and Translational Hepatology 2023年第3期11卷 595-604页

作者： Dongying Zhao Yahui Li Wei Xie Wenjie Wu Yan Chen Yongjun Zhang Department of Neonatology Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghaiChina Department of Pediatric Surgery Intensive Care Unit Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghaiChina Department of Pediatric Surgery Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghaiChina

Background and Aims: Early identification of prognostic factors to predict transplant/death outcome of Biliary atresia (BA) is challenging. We aimed to investigate the longitudinal changes and predictive value of dynamic changes in acoustic radiation force impulse elastography with shear wave speed (SWS) quantification and other parameters within three months after hepatoportoenterostomy (HPE) for 2-year BA outcomes. Methods: Seventy-four patients who underwent HPE between July 2016 and June 2019 were prospectively enrolled. Outcomes were classified into native liver survival and transplant/death groups. Acoustic radiation force im-pulse elastography was performed sequentially at 3 months intervals post-HPE. Cox regression analysis was used to de-termine the superior SWS values and other predictors of liver transplantation or death. Results: Among patients 2 years of age, 36 survived with a native liver, nine died, and 29 underwent liver transplantation. The trend in SWS levels in the transplant/death group was significantly different from that in the native liver survival group. ΔSWS at 1-3 months post-HPE and total bilirubin at 1 month post-HPE were se-lected as superior predictors of liver transplantation or death using multivariate Cox regression models: hazard ratio (HR)=1.927;95% confidence interval (CI): 1.475-2.661;p<0.001 and HR=1.010;95% CI: 1.003-1.017;p=0.007, respectively. The combination of the selected ΔSWS and total bilirubin had good predictive power, with an area under the receiver operating characteristics curve of 0.89, specificity 94.44% and sensitivity 73.68%. Conclusions: Our results suggest that early postoperative bilirubin levels and SWS changes were reliable predictors of 2-year BA outcomes.

关键词： Biliary atresia Hepatoportoenterostomy Acoustic radiation force impulse elastography Native liver survival Liver transplantation.

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Prognostic Factors Related to In-hospital Death in Children with Biliary atresia:Analysis of a Nationwide Inpatient Database

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Journal of Clinical and Translational Hepatology 2023年第2期11卷 416-424页

作者： Yu Shi Yi-Zhou Jiang Guang-Peng Zhou Ying Shi Lan-Xia Gan Yuan-Yuan Kong Hai-Bo Wang Zhi-Jun Zhu Li-Ying Sun Liver Transplantation Center Beijing Friendship HospitalCapital Medical UniversityBeijingChina National Clinical Research Center for Digestive Diseases Beijing Friendship HospitalCapital Medical UniversityBeijingChina Department of Critical Liver Diseases Liver Research CenterBeijing Friendship HospitalCapital Medical UniversityBeijingChina Clinical Center for Pediatric Liver Transplantation Capital Medical UniversityBeijingChina National Clinical Research Center for Cardiovascular Diseases Fuwai Hospital Chinese Academy of Medical SciencesShenzhenGuangdong ProvinceChina China Standard Medical Information Research Center ShenzhenGuangdongChina Clinical Trial Unit Precision Medicine InstituteFirst Affiliated Hospital of Sun Yat-Sen UniversityGuangzhouChina

Background and Aims:Patients with Biliary atresia(BA)are prone to hepatic decompensation,which might eventually lead to death.This study aimed to identify the possible risk factors affecting in-hospital death in BA patients in China.Methods:We collected data from the Hospital Quality Monitoring System,a national inpatient database.All patients aged up to 2 years old with a diagnosis of BA were included.The subjects were divided to three groups,including Kasai portoenterostomy(KP),liver transplantation(LT),and no surgery.Logistic regression with Firth’s method was performed to identify potential influencing variables associated with in-hospital death.Results:During the year 2013 to 2017,there were 14,038 pediatric admissions with a diagnosis of BA.The proportion of in-hospital death in pediatric BA admissions was 1.08%.Compared with patients under six months,there was a higher risk of in-hospital death for children aged six months to 1 year and 1–2 years old.Clinical signs,including cirrhosis,variceal bleeding,and hepatic encephalopathy,were significantly associated with the risk of in-hospital death.In no surgery group,compared to those in Beijing and Shanghai,BA patients admitted in other districts had a lower risk of in-hospital death(OR=0.39,95%CI:0.21,0.70).However,in the LT group,patients admitted in other districts had a higher risk of in-hospital death(OR=9.13,95%CI:3.99,20.87).Conclusions:In-hospital survival remains unsatisfactory for pediatric BA patients with severe complications.Furthermore,more resources and training for BA treatment,especially LT,are essential for districts with poor medical care in the future.

关键词： Biliary atresia In-hospital death Liver transplantation Kasai portoenterostomy

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The diagnostic value of high-frequency ultrasonography in Biliary atresia

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Hepatobiliary & Pancreatic Diseases International 2013年第4期12卷 415-422页

作者： Li-Ping Jiang Yun-Chao Chen Lu Ding Xiao-Ling Liu Kai-Yan Li Dao-Zhong Huang Ai-Yun Zhou Qing-Ping Zhang Department of Ultrasound Tongji Hospital Tongji Medical College Huazhong University of Science and Technology Department of Ultrasound the First Affiliated Hospital of Nanchang University

BACKGROUND: It is a globally challenging problem to differentially diagnose Biliary atresia (BA) from other disease processes causing infantile cholestatic jaundice. The high frequency ultrasonography (HUS) yields much improved spatial resolution and therefore, might show better image in BA diagnostic examination. The present study was to evaluate the HUS on the diagnosis of BA in infants with jaundice. METHODS: Fifty-one infants with neonatal jaundice were scanned with ultrasonography. Images included gallbladder, bile duct, right hepatic artery (RHA), portal vein (PV) and triangular cord (TC) sign, magnetic resonance imaging and additionally laboratory tests and histopathology reports were assessed. RESULTS: Twenty-three BA and 28 non-BA cases were con firmed. The sensitivity, specificity, and accuracy of HUS were 91.3%, 92.9%, and 92.2%, respectively. All of these indices were significantly higher than those of conventional ultrasonography (P<0.01) and MR cholangiopancreatography (P<0.05). The HUS features, included a positive TC sign, an increased RHA diameter and RHA-diameter to portal-vein-diameter ratio (RHA/PV) and abnormal gallbladder, were important in the diagnosis of BA. CONCLUSION: HUS provided better imaging of BA and should be considered as a primary modality in the differential diagnosis of infantile jaundice.

关键词： neonatal jaundice Biliary atresia high-frequency ultrasonography

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Preoperative hepatic hemodynamics in the prediction of early portal vein thrombosis after liver transplantation in pediatric patients with Biliary atresia

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Hepatobiliary & Pancreatic Diseases International 2015年第4期14卷 380-385页

作者： Li-Hong Gu Hua Fang Feng-Hua Li Shi-Jun Zhang Long-Zhi Han Qi-Gen Li Department of Ultrasound Renji Hospital School of Medicine Shanghai Jiaotong University Department of Liver Surgery Renji Hospital School of Medicine Shanghai Jiaotong University

BACKGROUND： Portal vein thrombosis （PVT） is one of the main vascular complications after liver transplantation （LT）, especially in pediatric patients with Biliary atresia （BA）. This study aimed to assess the preoperative hepatic hemodynamics in pediatric patients with BA using Doppler ultrasound and determine whether ultrasonographic parameters may predict early PVT after LT.METHODS： One hundred and twenty-eight pediatric patients with BA younger than 3 years of age underwent Doppler ultra- sound within seven days before LT, between October 2006 and June 2013. The preoperative hepatic hemodynamic parameters were then compared between patients with early PVT （within 1 month following LT） and those without PVT. Receiver oper- ating characteristic analysis was performed to determine the optimal cutoff value for predicting early PVT. RESULTS： Of the 128 transplant recipients, 41 （32.03%） had a hypoplastic portal vein （PV）, 52 （40.63%） had hepatofugal PV flow and 40 （31.25%） had a high hepatic artery resistance index （HARI） of 〉1. Nine cases （7.03%） experienced early PVT. A PV diameter 〈4 mm （sensitivity 88.89%, specificity 72.27%）, and a hepatofugal PV flow （sensitivity 77.78%, specificity 62.18%） with a high HARI ≥1 （sensitivity 77.78%, specificity 72.27%） were hepatic hemodynamic risk factors for early PVT.（ CONCLUSIONS： Hepatic hemodynamic disturbances in pediatric recipients with BA were more common. Small PV diameter （≤4 mm） and hepatofugal PV flow combined with high HARI （≥1） are strong warning signs of early PVT after LT in pediatric patients with BA. Intense monitoring of vascular patency and prophylactic thrombolytic therapy should be considered in pediatric patients undergoing LT for BA.

关键词： liver transplantation Doppler ultrasound hemodynamics portal vein thrombosis Biliary atresia

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Use of shear wave elastography for the diagnosis and follow-up of Biliary atresia: A meta-analysis

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World Journal of Gastroenterology 2022年第32期28卷 4725-4739页

作者： Ellen S Wagner Hussien Ahmed H Abdelgawad Meghan Landry Belal Asfour Mark B Slidell Ruba Azzam Pediatric Gastroenterology Hepatologyand NutritionThe University of Chicago MedicineComer Children’s HospitalChicagoIL 60637United States Department of Medicine Zagazig UniversityZagazig 44511EgyptEgypt Department of Pediatrics The University of Chicago MedicineComer Children’s HospitalChicagoIL 60637United States Graduate School of Professional Education-Biomedical Informatics University of ChicagoChicagoIL 60637United States Department of Surgery The University of Chicago MedicineComer Children’s HospitalChicagoIL 60637United States

BACKGROUND Timely differentiation of Biliary atresia(BA)from other infantile cholestatic diseases can impact patient outcomes.Additionally,non-invasive staging of fibrosis after Kasai hepatoportoenterostomy has not been widely standardized.Shear wave elastography is an ultrasound modality that detects changes in tissue stiffness.The authors propose that the utility of elastography in BA can be elucidated through meta-analysis of existing studies.AIM To assess the utility of elastography in:(1)BA diagnosis,and(2)post-Kasai fibrosis surveillance.METHODS A literature search identified articles that evaluated elastography for BA diagnosis and for post-Kasai follow-up.Twenty studies met criteria for meta-analysis:Eleven for diagnosis and nine for follow-up post-Kasai.Estimated diagnostic odds ratio(DOR),sensitivity,and specificity of elastography were calculated through a random-effects model using Meta-DiSc software.RESULTS Mean liver stiffness in BA infants at diagnosis was significantly higher than in non-BA,with overall DOR 24.61,sensitivity 83%,and specificity 79%.Post-Kasai,mean liver stiffness was significantly higher in BA patients with varices than in patients without,with DOR 16.36,sensitivity 85%,and specificity 76%.Elastography differentiated stage F4 fibrosis from F0-F3 with DOR of 70.03,sensitivity 96%,and specificity 89%.Elastography also differentiated F3-F4 fibrosis from F0-F2 with DOR of 24.68,sensitivity 85%,and specificity 81%.CONCLUSION Elastography has potential as a non-invasive modality for BA diagnosis and surveillance post-Kasai.This paper’s limitations include inter-study method heterogeneity and small sample sizes.Future,standardized,multi-center studies are recommended.

关键词： Biliary atresia Cholestasis Hepatic portoenterostomy Fibrosis Esophageal and gastric varices Elasticity imaging techniques

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