Search

Objectives: Laparoscopic Kasai portoenterostomy (LKPE) has been shown to be a safe and feasible procedure in patients with biliary atresia (BA). The purpose of this study was to investigate the efficacy of modified LKPE (MLKPE) in the treatment of BA., Methods: Data of 58 BA patients undertaken MLPKE from July 2014 to December 2015 were retrospectively analyzed (group B), and compared with that of 43 BA patients received open Kasai portoenterostomy (OKPE) during the same period (group C). In addition, 195 BA patients who had undergone LKPE during May 2009 to June 2014 were also included (group A)., Results: All 296 patients enrolled in this study were non-syndromic type III BA. Compared with group A, group B had shorter operative time (ORT) (P < 0.01) and fewer intraoperative blood transfusion (IOBT) (P < 0.05). The conversion rate and cholangitis rate were also significantly lower in group B than that in group A (P < 0.05). The postoperative oral intake resumed (POOR), any postoperative complications (APOC), clearance of jaundice (CJ), 1-year and 3-year survival rate with native liver (SNL) were not significantly different between group A and group B (P > 0.05). The ORT, IOBT, POOR, APOC, CJ, 1-year and 3-year SNL in group B were much better than that of group C (P < 0.05)., Conclusion: The MLKPE was associated with good perioperative with ORT, IOBT, POOR, and APOC. The short-term outcomes in MLKPE were no worse than that of OKPE and LKPE. MLKPE can be regarded as a treatment option for BA.

Background/aims: The outcome of the Kasai operation remains unsatisfying. This study aims to evaluate the feasibility and effectiveness of primary LDLT on biliary atresia (BA) treatment for infant patients., Methodology: Fifty infant patients with BA who were treated in our center between Jan 2008-Jan 2011 were included. Seventeen of them received LDLT. Another 33 patients received Kasai operation as the first line treatment. All patients had no history of previous abdominal surgery. Records of these patients and the donors for LDLT were retrospectively reviewed., Results: The follow-up ranged from 12-48.5 months; 30 postoperative-day survival of the LDLT patients and Kasai patients were 82.4% and 81.8%, respectively (p=1). One-year survival of the two groups was 82.4% and 60.6% (p=0.200). Jaundice free rate of the patients who survived at least one month after the surgery was 100% vs. 70.4% of the LDLT group and Kasai group, respectively (p=0.035). Main cause of death in LDLT group was hepatic artery thrombosis and multiple organ dysfunction syndrome in the Kasai group., Conclusions: Primary LDLT is a safe and efficient management for infant patients with BA. It may serve as an alternative first line treatment for BA treatment with specific indications.

ObjectiveTo study the regulatory role of GLI1/GLI2, a nuclear transcription factor of the Sonic hedgehog (Shh) signaling pathway, in epithelial–mesenchymal transition (EMT) related to hepatic fibrosis in patients with biliary atresia (BA).MethodsThe messenger RNA (mRNA) and protein expression levels of GLI1/GLI2, Snail/Slug, and other Shh- and EMT-related cytokines were tested in the liver tissues of BA patients and animals. Then, GLI1/GLI2 was silenced and overexpressed in mouse intrahepatic bile duct epithelial cells (mIBECs) and BA animals to investigate changes in the mRNA and protein expression of EMT key factors and liver fibrosis indicators. After silencing and overexpression of GLI1/GLI2, immunofluorescence was used to detect the expression of cytokeratin-19 (CK19) and α-smooth muscle actin (α-SMA) in mIBECs, and hematoxylin and eosin (HE) staining and Masson staining were used to observe the degree of liver fibrosis in the BA animals.ResultsCompared with the control, the mRNA and protein expression levels of GLI2, Snail, vimentin, and α-SMA were significantly increased and those of E-cadherin were significantly decreased in liver tissue from BA patients and animals. Overexpression of GLI2 increased the mRNA and protein expression levels of Snail, vimentin, and α-SMA and that of E-cadherin was significantly decreased in mIBECs and BA animals. After GLI2 silencing, the opposite pattern was observed. Immunofluorescence detection showed enhanced expression of the bile duct epithelial cell marker CK19 in mIBECs after GLI2 silencing and enhanced expression of the mesenchymal cell marker α-SMA after GLI2 overexpression. HE and Masson staining suggested that the GLI2-overexpressing group had a significantly higher degree of fibrosis.ConclusionThe Shh signaling pathway plays an important role in fibrogenesis in BA. GLI2 can significantly regulate EMT in mIBECs and livers of BA mice.

This retrospective study was conducted to examine the development and current status of pediatric liver transplantation (LT) in western China. Clinical, demographic, morbidity, and mortality data were collected to analyze. It included 260 consecutive pediatric LTs performed at three centers in western China between January 2000 and May 2019. Kaplan‐Meier graft survival rates at 1, 3, 5, and 10 years were 82.1%, 77.2%, 76.6%, and 76.6%, respectively; corresponding patient survival rates were 84.7%, 80.7%, 80.0%, and 80.0%, respectively. More patients underwent living donor liver transplantation (LDLT; n = 188 (73.4%)) than deceased‐donor liver transplantation (DDLT; n = 68 (26.6%)). Survival was better after LDLT (91.5%, 86.6%, and 80.6% at 1, 3, and 5 years, respectively) than after DDLT (80.9%, 72.4%, and 63.9%, respectively; P <.05). Biliary atresia was the leading LT indication (n = 141 (55.1%)), followed by metabolic disease (n = 36 (14.1%)), which was associated with the best recipient survival (88.5% at 5 years). The transplant era and graft‐to‐recipient body weight ratio (GRWR) also significantly predicted overall survival. Survival rates at 5 years were worst in 2000‐2005 (54.5%) and best for GRWRs of 0.8%‐4% (80.4%). The development of pediatric LT in western China began slowly, but the quantity and quality of pediatric LT has progressed in recent years. This procedure is now a promising and reliable treatment for children with end‐stage liver disease in western China. [ABSTRACT FROM AUTHOR]

The matrix metalloproteinase-7 (MMP-7) is a promising marker for identification of biliary atresia. We assessed the accuracy and clinical value of MMP-7 for diagnosis of biliary atresia in infants. We searched MEDLINE, Embase, ISI Web of Knowledge, and the Cochrane Library, from inception to December 30, 2020. We included articles written in English, Chinese, or French that investigated MMP-7 for differentiation of biliary atresia infants from those with other form of cholestasis. Two review authors independently assessed eligibility for inclusion, evaluated the methodological quality of included studies, and extracted data to estimate diagnostic accuracy. We calculated individual and pooled sensitivities and specificities. We used I2 to test heterogeneity and investigated the source of heterogeneity. Our search identified 4 studies (593 infants). Risk of bias in the included studies was generally low. Bivariate analysis yielded a mean sensitivity of 0.96 (95% CI: 0.93–0.98) and specificity of 0.91 (95% CI: 0.85–0.95). The area under the receiver operating characteristic curve was 0.97 (95% CI: 0.95–0.98). A moderate heterogeneity was found among the included studies as the I2 values for sensitivity and specificity were 8.6% (95% CI: 1.5–19.04) and 56.7% (95% CI: 28.91–70.07), respectively. After a sensitivity analysis, there was no change in sensitivity and MMP-7 specificity ranged from 0.91 to 0.93. The serum MMP-7 is a helpful biomarker for diagnosis of biliary atresia in cholestasis infants. Nevertheless, it cannot be recommended as the single definitive test for biliary atresia diagnosis; further primary clinical research is mandatory.Trial registration: prospero230839 [ABSTRACT FROM AUTHOR]