PROMOTER ANALYSIS SUGGESTS THE IMPLICATION OF NF100001KAPPAB/C‐REL TRANSCRIPTION FACTORS IN BILIARY ATRESIA
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Introduction Biliary atresia is a severe liver disease in neonates, and the prognosis partially depends on the age at which infants undergo the Kasai procedure. Matrix metalloproteinase-7 (MMP-7) was confirmed to have significant value in the diagnosis of biliary atresia. However, so far, the reference range and its cut-off value for diagnosing biliary atresia have not been established yet. Methods and analysis DIagnosis Algorithm for Biliary Atresia (DIABA-7) is a prospective diagnostic test. Cholestatic infants and normal controls within 150 days of age are recruiting from the Chinese Biliary Atresia Collaborative Network. The serum samples and dried blood spot (DBS) samples are obtained to detect MMP-7 concentrations using an ELISA kit. The reference standard is the intraoperative exploration and subsequent histological examination of liver biopsies. Lambda-Mu-Sigma (LMS) method is used to calculate the normal range of serum MMP-7 of each age group. Receiver operating characteristics (ROC) curves are constructed to calculate the best cut-off point and area under the curve for the index test. The sensitivity, specificity, positive predictive value and negative predictive value are used to show the diagnostic accuracy. Pearson correlation coefficient test is applied to assess the correlation of serum MMP-7 and DBS MMP-7. Ethics and dissemination This study was reviewed and approved by the Ethics Committee of Children’s Hospital of Fudan University (Number 2020–296). Dissemination will be guided by investigators and patients. The aim is to publish the study results in a high-quality peer-reviewed journal and present the findings at international academic meetings. Trial registration number ChiCTR2000032983.
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Disadvantaged
Liver disease
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Stoma (medicine)
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In an earlier issue this year, the Kyoto group presented the long-term outcome of live donor liver transplantation (LDLT) for adult post-Kasai biliary atresia (BA) recipients (1Uchida Y Kasahara M Egawa H et al.Long-term outcome of adult-to-adult living donor liver transplantation for post-Kasai biliary atresia.Am J Transplant. 2006; 6: 2443-2448Abstract Full Text Full Text PDF PubMed Scopus (38) Google Scholar). Among the 464 patients with BA that underwent LDLT at their institution, 47 (10.1%) were considered adults (median age 20 years, range 16–34 years). The authors reported that the outcome of LDLT in adult BA patients was significantly poorer than that in pediatric patients: the overall 1-, 5- and 10-year survival rates in adult patients were 72.2%, 69.7% and 55.8%, while the corresponding rates in pediatric patients were 87.7%, 86.7% and 80.8%, respectively (p < 0.005). Multivariate analysis with a multiple logistic regression model revealed that post-transplant complications requiring repeat laparotomy and model for end-stage liver disease score over 20 were independent prognostic factors. The authors concluded that LDLT should be considered proactively at the earliest possible stage. The study is the largest single center series published to date, and the results are very valuable. We do not disagree with the importance of early liver transplantation in children with end-stage liver disease to avoid growth retardation and the development of other lethal complications. We are not, however, convinced of their comments that earlier performance of LDLT is recommended because of the poorer outcome of LDLT performed later in adulthood for post-Kasai BA. We are very concerned that some of the obvious important variables such as donor-recipient blood type incompatibility, presence of moderate to severe (>30%) intrapulmonary shunting, and presence of hepatocellular carcinoma were not included in their multivariate analysis, although the apparent influence of these factors was indicated by univariate analysis in their paper. These are all well-known factors contributing to poorer short-term survival rates following liver transplantation in adults. According to the data depicted by the Kaplan–Meier method presented in their paper, the majority of mortalities in the adult population occurred within the first year, most likely due to these complications. We strongly suspect that if such higher risk recipients—patients with donor-recipient blood type incompatibility, with moderate to severe intrapulmonary shunting, or with hepatocellular carcinoma—were excluded from the analysis, the adult series may have presented with results no different from their pediatric series, that is, overall 1- and 5-year survival rates above 80%. The study presented by Uchida et al. only confirms the current general understanding that adult patients with co-morbidity present with a more complicated post-operative course with higher mortality. The data presented do not suggest that BA treated by the Kasai procedure earlier in life is the cause of the poorer outcomes following LDLT. Further study is necessary to elucidate whether the long-term outcome of LDLT for post-Kasai BA in the adult population is inferior to that in the pediatric population and to determine the optimal conditions and timing for successful outcomes.
Liver disease
Single Center
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The survival of infants with biliary atresia has improved significantly during the past two decades as a result of modification of the Kasai hepatoportoenterostomy procedure complemented by advances in liver transplantation. Recent reports suggest that the long-term success rate of the Kasai procedure is 40%. Failures are salvaged by liver transplantation. Advances in organ preservation, the use of reduced-sized grafts, and newer immunosuppressive agents (cyclosporine, FK 506) have strongly influenced these improved results. Unfortunately, liver transplantation is associated with a high complication rate, the risk of opportunistic infection, and an increased rate of malignancy due to immunosuppression. Until immunotolerance can be achieved, the Kasai procedure remains the procedure of choice for infants with biliary atresia. Liver transplantation is a life-saving complementary procedure for patients who fail to drain bile following the Kasai procedure, who are older than 3 to 4 months of age at diagnosis, or who have advanced cirrhosis. In the current era, the overall survival should exceed 80%.
Immunosuppression
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Liver function
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Objective To investigate the diagnosis, treatment and postoperative outcome of biliary atresia, and discuss early diagnosis and new medical strategies of treatment.Methods The diagnosis, operative age, surgical procedures and postoperative survival rate of 28 children in our hospital with biliary atresia were reviewed retrospectively.Results The early diagnostic rate of biliary atresia was 14%. Operative age was from 13 to 270 days and that of 4 (14%) children was 60 days, There were 13 children with age ranging from 61 to 90 days (46%), 8 from 91 to 120 days (29%) and 3120 days (11%). No child with biliary atresia received liver transplantation. The 1 5 survival rate was 35% (9/25).Conclusion Lower early diagnostic rates were the main causes of lower survival rates. Close observation of clinical presentations and imaging features of children with biliary atresia and exploration under laparoscopy in time can enhance the early diagnostic rates of patients with biliary atresia. The long term survival rates can be increased through those methods such as modifying surgical procedures, more biliary atresia children with advanced disease accepting liver transplantation and developing new medical strategies for treating patients with biliary atresia.
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Most neonatal cholestatic disorders are rare diseases (RD), but they represent the leading indication for pediatric liver transplantation. From the pediatric surgical point of view, biliary atresia (BA) and choledochal cyst are from particular interest because diagnostic pathways and therapeutic algorithms are diverging as well as outcome parameters of follow-up studies. In addition, screening programs for BA are still at an early stage while transition of those patients with and without liver transplantation is not established yet. The newly created Web site www.bard-online.com focuses on BA and related diseases and includes decentralized online registries, information for patients and health workers, as well as a platform for interdisciplinary communication and service offerings. This initiative was started to compensate for disadvantages for patients with RD and to help unifying the community of clinicians and researchers in this field.
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