Underwent Kasai Portoenterostomy Research Articles

Portal vein thrombosis complicating neonatal umbilical vein catheterization in a 3-month-old infant with coincidental extrahepatic biliary atresia: A case report.

Umbilical vein catheterization (UVC) is a common procedure in neonatal intensive care units (NICU) but carries risks of severe complications such as portal vein thrombosis (PVT). Extrahepatic biliary atresia (EHBA), a leading cause of neonatal cholestasis, often progresses to end-stage liver disease. This case report discusses the rare coexistence of PVT and EHBA in a 3-month-old infant, highlighting the critical need for timely diagnosis and intervention. A 3-month-old female presented with jaundice, dark-colored urine, and clay-colored stools. She had a history of NICU admission for neonatal sepsis during which a UVC was inserted. Physical examination revealed jaundice and hepatosplenomegaly. Abdominal ultrasonography identified hepatosplenomegaly, mild ascites, and portal cavernoma. A liver biopsy confirmed a diagnosis of EHBA. The patient underwent Kasai portoenterostomy. Postoperatively, she developed complications including ascites, systemic hypertension, and hyperammonemia. Initial improvements were observed with decreased bilirubin levels. Despite initial stabilization, the patient's condition deteriorated, and she succumbed on day 15 postoperation. This case underscores the significant risks of PVT associated with UVC and the importance of monitoring NICU graduates for early detection of complications. The early onset of portal hypertension and esophageal varices in this case challenges existing beliefs about EHBA's clinical progression. Greater awareness and routine follow-up imaging are essential to improve outcomes in similar scenarios.

Medium-chain triglyceride supplementation and the association with growth, nutritional status and clinical outcomes in infants with biliary atresia

Background and aimsInfants with biliary atresia experience gastrointestinal malabsorption of long-chain triglycerides and are commonly supplemented with medium-chain triglyceride (MCTs) that can be passively absorbed. The aim was to investigate the association of MCT supplementation with growth, nutritional status and clinical outcomes in infants with biliary atresia. MethodsInfants who underwent Kasai portoenterostomy and were followed up for at least two years or until death or transplantation were reviewed. Infants with comorbidities affecting growth or outcome were excluded. Data were extracted from medical records from more than a decade in relation to MCT supplementation, growth, nutritional status and clinical outcome at baseline, 6-weeks, 3-, 6-, 12- and 24-months. Mixed-effects modelling was used to test associations of MCT in the first six months with these outcomes. ResultsOf 200 infants (108 male), 108 (54%) were alive with native liver at two years, 84 (42%) underwent liver transplantation and eight (4%) died. MCT percentage prescribed was mean 57.3% (SD 11.2) while MCT intake was median 2.7 (IQR 2.2, 3.8) g/kg/d. For every g/kg/d MCT consumed, the rate of change in z-score for weight was -0.27 (95% CI -0.37 to -0.17) and length was -0.31 (-0.42 to -0.17) (both p<0.001). Compared to the low MCT group (<2.7 g/kg/d), the high group (≥2.7 g/kg/d) consumed more energy (118 vs. 108 kcal/kg; p<0.001), however, at 3-months they had lower weight (-1.7 (1.2) v. -1.0 (1.2) and length (-1.3 (1.1) v. -0.6 (1.4) z-scores (both p<0.001) but no differences in growth at later time points. There was no overall association between MCT and nutritional status or clinical outcomes. ConclusionsThis is the first study to investigate the association of MCT with growth, nutritional status and clinical outcomes in biliary atresia. No association was found between MCT with growth beyond 3-months, overall nutritional status or clinical outcomes. The association between MCT (g/kg/d) and poorer growth in the first 3-months may be explained by infants with poorer growth drinking more or being prescribed more MCT formula milk. A randomised controlled trial could help to better understand this association.

Living-Related Liver Retransplantation in a Child: When it Seems Impossible (A Clinical Case)

The objective: to show the possibility of anesthesia during liver retransplantation in a child in the absence of adequate vascular access.A clinical case of liver retransplantation in a 10-year-old patient with liver transplant dysfunction and acquired thrombophilia is considered. In 2011, the child underwent Kasai portoenterostomy, and in 2012, living-related transplantation of the left lateral liver bisegment from a related donor was performed due to liver cirrhosis as an outcome of biliary atresia. Also, the child had multiple surgical interventions due to perforations of the small intestine with underlying segmental venous mesenteric thrombosis. In the long term after the transplantation, irreversible transplant dysfunction developed with manifestations and worsening of hepatocellular insufficiency, encephalopathy, as well as recurrent bleeding from varicose veins of the esophagus and cardiac orifice. The clinical situation was complicated by the lack of adequate vascular access due to total thrombosis of the venous system, thrombosis of the superior and inferior vena cava. The only possible option for ensuring adequate venous access was the implantation of a tunneled catheter into the right atrium of the right atrium for prolonged standing in conditions of single-lung ventilation through right-sided thoracotomy.

The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000-2018).

BackgroundThe epidemiology and outcomes of biliary atresia (BA) have been well-documented in national cohorts from two main ethnicities, namely, the Asian Orientals and Caucasians, with incidence ranging from 1 in 5,000 to 1 in 9,000 live births in East Asia and 1 in 15,000 to 19,000 live births in Europe and North America.ObjectiveWe report the first nationwide BA study outside North America, Europe, and East Asia to describe the epidemiology and outcomes of BA in Saudi Arabia.MethodsA national database of BA cases diagnosed between 2000 and 2018 was analyzed. We assessed clearance of jaundice (bilirubin <20 μmol/L) in all cases that underwent Kasai portoenterostomy (KPE). We then estimated survival using the Kaplan–Meier method with endpoints of liver transplantation (LT), death, or survival with native liver (SNL).ResultsBA was diagnosed in 204 infants (106 females; 10% pre-term). The incidence of BA was 1 in 44,365, or 2.254 in 100,000 live births (range, 0.5–4 in 100,000). Polysplenia was diagnosed in 22 cases (11%). The median age at referral was 65 days. A total of 146 children (71.5%) underwent KPE at a median age of 70 days. Clearance of jaundice was achieved in 66 of the 146 (45%) infants. The 10-year SNL after KPE was 25.5%, and the overall 10-year estimated survival was 72.5%. The Kaplan–Meier survival curves for patients undergoing KPE at the age of <60, 61–90, and >90 days showed a SNL rate at 51.6, 33, and 12.5%, respectively, at 5 years (P < 0.001). The 2-, 5-, and 10-year post-LT survival rates were 92.5, 90.6, and 90%, respectively. Undergoing an initial KPE did not impact negatively on the overall LT survival rate when compared to BA cases that underwent primary LT (P = 0.88).ConclusionThe incidence rate of BA in Saudi Arabia is lower than the incidence reported elsewhere. Late referral of BA cases remains a problem in Saudi Arabia; as a result, the SNL rate was lower than reported by other national registries. Hence, national policies devoted to timely referral and earlier age at KPE are needed.

Association between mitochondrial and nuclear DNA damages and cellular senescence in the patients with biliary atresia undergoing Kasai portoenterostomy and liver transplantation.

Biliary atresia (BA) is a cholestatic disease with extrahepatic bile duct obstruction that requires early surgical intervention and occasionally liver transplantation (LT). Accumulation of toxic bile acids induces oxidative stress that results in cell damage, such as cell senescence, mitochondrial dysfunction and others. However, details of their reciprocal association and clinical significance are unexplored. Therefore, we used immuno-localization of markers for cell senescence (p16 and p21), nuclear double-strand DNA damage (γH2AX), autophagy (p62), and mtDNA damage (mtDNA copy number) in patients with BA who underwent Kasai portoenterostomy (KP) and LT. We studied liver biopsy specimens from 54 patients with BA, 14 who underwent LT and 11 from the livers of neonates and infants obtained at autopsy. In hepatocytes, p21 expression was significantly increased in KP. In cholangiocytes, p16 expression was significantly increased in LT, and p21 expression was significantly increased in KP. p62 expression was significantly increased in the KP hepatocytes and LT cholangiocytes. Furthermore, mtDNA copy number significantly decreased in KP and LT compared with the control. Cell senescence and mitochondrial DNA damage progression were dependent on the BA clinical stages and could possibly serve as the markers of indication of LT.

Features of Cirrhotic Cardiomyopathy Early in the Lives of Infants With Biliary Atresia Correlate With Outcomes Following Kasai Portoenterostomy.

Cirrhotic cardiomyopathy (CCM), detected during two‐dimensional echocardiography (2DE), is prevalent in patients with biliary atresia (BA) awaiting transplant. Whether CCM occurs early in the lives of infants with BA is unknown. The aim of this study was to explore the incidence and consequence of CCM in patients with BA, focusing on the earliest ages when 2DE was performed. A cohort of 78 patients with BA at a single center underwent 2DE (median age = 132 days) during the first year of life. Left ventricular mass index (LVMI) to upper limit of normal (ULN) ratio ≥ 1.0 was present in 60% of patients who never underwent Kasai portoenterostomy (KPE; n = 15), 49% with nondraining KPE (n = 41), and 21% with draining KPE (n = 19). Patients with a draining KPE (median age at 2DE = 72 days) had a lower LVMI/ULN ratio (0.75 [interquartile range [IQR] 0.70, 0.91]) compared to those with a nondraining KPE (0.99 [IQR 0.78, 1.17] median age of 141 days; P = 0.012). In those whose 2DE was performed within 7 days of KPE (n = 19, median age of 61 days), the LVMI/ULN ratio was lower in those with a future draining KPE (0.73 [IQR 0.66, 0.75]) compared to the group with a future nondraining KPE (1.03 [IQR 0.88, 1.08], P = 0.002). Logistic regression modeling revealed LVMI/ULN ratio ≥ 1.0 as a predictor of KPE outcome, with an odds ratio of 16.7 (95% confidence interval 1.36‐204; P = 0.028) for a future nondraining KPE compared to those with a LVMI/ULN ratio < 1.0. Conclusion: 2DE early in the lives of patients with BA revealed features of CCM that correlated with future outcomes. If validated in a multicenter study, this could lead to 2DE as a useful clinical tool in the care of infants with BA.

The need for early Kasai portoenterostomy: a Western Pediatric Surgery Research Consortium study.

The purpose of this study was to investigate factors impacting transplant-free survival among infants with biliary atresia. A multi-institutional, retrospective cohort study was performed at nine tertiary-level children's hospitals in the United States. Infants who underwent Kasai portoenterostomy (KP) from January 2009 to May 2017 were identified. Clinical characteristics included age at time of KP, steroid use, surgical approach, liver pathology, and surgeon experience. Likelihood of transplant-free survival (TFS) was evaluated using logistic regression, adjusting for patient and surgeon-level factors. Secondary outcomes at 1 year included readmission, cholangitis, reoperation, mortality, and biliary clearance. Overall, 223 infants underwent KP, and 91 (40.8%) survived with their native liver. Mean age at surgery was 63.9 days (± 24.7 days). At 1 year, 78.5% experienced readmission, 56.9% developed cholangitis, 3.8% had a surgical revision, and 5 died. Biliary clearance at 3months was achieved in 76.6%. Controlling for patient and surgeon-level factors, each additional day of age toward operation was associated with a 2% decrease in likelihood of TFS (OR 0.98, 95% CI 0.97-0.99). Earlier surgical intervention by Kasai portoenterostomy at tertiary-level centers significantly increases likelihood for TFS. Policy-level interventions to facilitate early screening and surgical referral for infants with biliary atresia are warranted to improve outcomes.

＜Editors' Choice＞ Long-term outcomes of the partial splenectomy for hypersplenism after portoenterostomy of patients with biliary atresia.

ABSTRACTMassive splenomegaly and hypersplenism in patients with biliary atresia after Kasai portoenterostomy were treated with partial splenic embolization or total splenectomy. We performed partial splenectomy to reduce the complications of partial splenic embolization and avoid overwhelming post-splenectomy infection. This study aimed to evaluate the long-term effects of partial splenectomy for hypersplenism on postoperative liver and spleen function in patients with biliary atresia. Among jaundice-free patients with biliary atresia who underwent Kasai portoenterostomy between January 1992 and December 2012, 15 underwent partial splenectomy for massive splenomegaly and hypersplenism at our institution. Changes in the laboratory data 10 years post partial splenectomy were retrospectively investigated, and these along with the latest data were measured. A total of four patients (27%) required living-donor liver transplantation after partial splenectomy, a proportion similar to those who did not undergo partial splenectomy. Compared to the preoperative baseline, the platelet counts were significantly higher at 1 and 3 years after surgery (p < 0.05). Aspartic aminotransferase-to-platelet ratio index was significantly lower at 1, 7, and 10 years after partial splenectomy (p < 0.05). No further surgeries were required for hypersplenism after partial splenectomy over 10 years, and there were no cases of overwhelming post-splenectomy infection after partial splenectomy. Partial splenectomy is safe and effective for the treatment of hypersplenism with biliary atresia over a long time period. It could be considered as an alternative to partial splenic embolization as it can suppress hypersplenism for a long time and induces fewer postoperative complications.

Analysis of the odds ratio of developmental delay in children with biliary atresia 12 months after liver transplantation

Background. Liver cirrhosis occurring before 1 year of age can affect a child’s development. Liver transplantation is the only radical treatment for decompensated cirrhosis. In biliary atresia, cirrhosis develops during the first months of life. The duration of cirrhosis in biliary atresia may vary from palliative Kasai portoenterostomy (PE) to liver transplantation. Developmental abnormalities in children with biliary atresia have been shown to occur both before and after liver transplantation. Association between duration of liver cirrhosis and psychomotor development of children has been underestimated.Objective: to determine the chances of developmental delay in children depending on the cirrhosis persistence duration.Materials and methods. The study enrolled 83 children with biliary atresia (47 children underwent palliative Kasai PE, 36 children with liver transplantation did not undergo Kasai PE). All children had their psychomotor development assessed before PE and 12 months after PE using the Griffiths psychomotor developmental scale (translation and adaptation by E.S. Keshishian) for children up to 24 months of age. Statistical analysis was performed by calculating odds ratios with 95% confidence intervals.Results. Comparative analysis showed that in the subgroup of children who underwent Kasai PE, cirrhosis persistence before transplantation was 2.6 months longer than in children without Kasai PE (p = 0.011). The odds of developmental delay in preparation for liver transplantation were 3.3 times higher in the subgroup of children who underwent Kasai palliative PE compared to children without palliative (95%, CI 1.35–8.31). The odds of developmental delay 12 months after liver transplantation were 4.4 times higher in the subgroup of children who underwent palliative Kasai PE than in children without the palliative care (95% CI 1.54–12.5).Conclusion. Children who underwent liver transplantation after palliative surgical treatment had lower levels of psychomotor development than children without palliative Kasai PE both before and 12 months after liver transplantation (p = 0.0018, p = 0.01 respectively).

Elevation of microRNA-214 is associated with progression of liver fibrosis in patients with biliary atresia.

MicroRNAs (miRNAs) play an important role in regulating fibrogenesis in the liver. The current study examined the ability of microRNA-214 (miR-214) level in liver and serum samples obtained from patients with BA to predict progressive liver fibrosis in patients with biliary atresia (BA). We examined miR-214 level in relation to conventional markers of liver fibrosis, with liver and serum samples from BA patients. Fifty-two patients with BA who underwent Kasai portoenterostomy and four control patients underwent liver biopsy. In 28 patients with BA, blood samples were collected to analyze circulating serum miR-214. MiR-214 levels in liver tissue were significantly upregulated in patients with BA who had severe liver fibrosis (F3-4) compared to those with none to mild fibrosis (F0-2), whereas suppressors-of-fused homolog (Sufu) mRNA levels were significantly suppressed in F3-4. Serum miR-214 levels were significantly higher in patients with F3-4 compared with F0-2. Area under the curve analysis showed that the serum miR-214 cut-off level for predicting F3-4 was 0.805 (p = 0.0046). Hepatic overexpression of miR-214 is associated with progression of liver fibrosis in patients with BA, and the circulating miR-214 level may serve as a non-invasive predictor of liver fibrosis.

Anatomical patterns of biliary atresia including hepatic radicles at the porta hepatis influence short- and long-term prognoses.

The biliary atresia (BA) inflammatory process leads to various obstructive patterns of extrahepatic biliary trees. The significance of the various BA obstructive patterns is unclear. This study aimed to determine the relationship between the anatomical patterns of the biliary tract and short- and long-term prognoses in BA. Between 1989 and 2018, 3483 patients were registered in the Japanese Biliary Atresia Registry. For this study, we selected 2649 patients who underwent Kasai portoenterostomy (KP) between the ages of 31 and 90days to eliminate the influence of age at KP as much as possible. Regarding the main type, there were significant differences in the jaundice clearance rate (JCR; Type I: 67.9%, Type I-cyst: 79.4%, Type II: 74.5%, Type III: 60.9%; P<.0001) and the native liver survival rate (NLSR; P<.0001). In subgroups with hepatic radicles in Types I, II, and I-cyst, there was a significant difference in JCR (P=.0004) and NLSR (P=.0026). In subgroups with hepatic radicles in Type III, there was a significant difference in JCR (P=.0148) and NLSR (P=.0421). Anatomical patterns of obstruction influenced short- and long-term prognoses in BA. These patterns were suggested to be prognostic factors following KP.

Centralization of Biliary Atresia: Has Germany Learned Its Lessons?

The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade. In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany. Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001-2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001-2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001-2005 [p = 0.5]). Compared with the 2001-2005 analysis, all criteria showed improvement but the differences are statistically not significant. Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction.

Temporal histopathological changes in biliary atresia: A perspective for rapid fibrosis progression

Introduction and ObjectivesBiliary atresia (BA) is characterized by rapid progression of fibrosis with no definite causes. Histopathological findings have been extensively described, but very few studies have assessed temporal changes in BA. Understanding these short-term changes and their relationship with fibrosis progression could have an impact on ameliorating rapid fibrogenesis. We aimed to study the relationship between temporal histopathological changes and fibrosis progression in BA within a short time interval. Patients and MethodsForty-nine infants with BA who underwent Kasai portoenterostomy, a diagnostic liver biopsy, and an intraoperative liver biopsy were recruited. Histopathological characteristics of the two biopsies were examined. Temporal histopathological changes were assessed by comparing the two types of biopsies. Correlation of temporal changes in fibrosis with age, interval between biopsies, laboratory profiles, and temporal histopathological changes were studied. ResultsIn the univariate analysis, bile ductular proliferation (BDP), portal infiltrate, giant cells, hepatocellular swelling, and fibrosis showed significant temporal changes within a short interval (5–31 days). BDP and fibrosis showed the most frequent increase in their grades (32/49 and 31/49 cases, respectively). In the multivariate analysis, BDP was the only independent pathological feature showing a significant temporal increase (p = 0.021, 95% confidence interval: 1.249–16.017). Fibrosis progression was correlated with temporal changes in BDP (r = 0.456, p = 0.001), but not with age (p = 0.283) or the interval between the biopsies (p = 0.309). ConclusionsFibrosis in BA progresses rapidly and is significantly correlated with BDP. Assessment of targeting BDP as an adjuvant medical therapy is recommended.

Evaluation of Perioperative Complications in the Management of Biliary Atresia.

Purpose: To analyze the influence of perioperative complications in the management of biliary atresia (BA).Methods: A retrospective study was performed using a total of 422 BA patients who underwent Kasai portoenterostomy (KPE) in a single institution between February 2016 and May 2017. Data on patients' clinical characteristics, laboratory examinations, perioperative complications, and outcomes were collected. Unpaired two-tailed t-test and χ2 test were employed for the comparison between BA patients with and without perioperative complications. Cox regression analysis was used to screen the risk factors for 2-years NLS in BA, and their influence on the 2-years NLS was analyzed using Kaplan–Meier survival analysis as well as the log-rank test.Results: The incidence of perioperative complications, 6-months jaundice clearance (JC) and 2-years native liver survival (NLS) rate were 60.4, 59.5, and 56.6%, respectively. Patients with perioperative complications had lower serum albumin (ALB) level, but higher aspartate aminotransferase-to-platelet ratio index (APRI) and international normalized ratio (INR) levels when compared with those without perioperative complications (ALB, P < 0.05; APRI, P < 0.01; INR, P < 0.05). Moreover, perioperative complications were correlated with glucocorticoid administration (P = 0.002). Univariate Cox regression analysis showed no relationship between perioperative complications and 2-years NLS (P > 0.05). However, multivariate Cox regression analysis indicated 6-months JC was an independent protective factor for 2-years NLS [P < 0.0001, hazard ratio (HR) = 0.074, 95% confidence interval = 0.05–0.11], and concordance index of this prediction model including age, weight, APRI, glucocorticoid, and 6-months JC was 0.811.Conclusion: Although perioperative complication is common during and after KPE, it had no influence on the prognosis of BA. However, assessment of the serum level of total bilirubin after KPE may serve as an important predictor for the outcome in BA.

Comparison of the outcomes of biliary atresia with cystic degeneration and isolated biliary atresia: A matched-pair analysis

BackgroundPatients with biliary atresia (BA) with extrahepatic cystic degeneration (BACD) have a unique pathophysiology; however, clinical outcomes and progression of perinatal degeneration are not well-defined. We aimed to investigate the differences in clinical characteristics and outcomes between BACD and isolated BA (IBA). MethodsWe performed a retrospective analysis of patients with BA who underwent Kasai portoenterostomy (KPE) from August 1997 to January 2018 and compared the clinical features and outcomes between BACD (n = 21) and IBA (n = 237). Matched-pair analysis for age and sex was performed between BACD and IBA groups to reduce confounding. ResultsBefore matched-pair analysis, we found that BACD patients were younger at KPE (45 vs. 64 days, p = 0.008), showed lower total bilirubin at the 3-month follow-up (0.5 vs. 1.4 mg/dL, p = 0.002), and higher 5-year native liver survival rate (95.2% vs. 61.4%, p = 0.006) than IBA patients. After matching, the BACD group showed significantly lower total bilirubin levels at the 3-month follow-up (0.5 vs. 1.5 mg/dL, p = 0.036) and higher 5-year native liver survival rate (95.2% vs. 57.5%, p = 0.006) than the IBA group. ConclusionBACD demonstrated higher bilirubin clearance and native liver survival rates than IBA. Levels of EvidenceTreatment Study, Level III.

Predictors of demand for liver transplantation in children undergoing Kasai portoenterostomy for biliary atresia

Context Biliary atresia (BA) remains the most common indication for pediatric liver transplantation worldwide. Ultimately, 50–80% of BA patients will require a liver transplant Thus, much of the research in the field has focused on identifying predictors for transplant and optimizing the efficacy of Kasai portoenterostomy (KPE), in order to decrease the need for transplantation and avoid the risks of lifelong immunosuppression. Aim The aim of this study was to identify perioperative risk factors for the need of liver transplantation following KPE operation for BA. Patients and methods A retrospective analysis of 150 patients undergoing KPE for BA at Hepatobiliary and Pancreatic Surgery Department, National Liver Institute, Menoufia University, from May 2013 to May 2018, was carried out. Patients were divided into two groups: group one included 73 (48.7%) patients who survived without the need for liver transplantation and group two included 77 (51.3%) patients with mortality, listed for liver transplantation or transplanted; thereafter, logistic regression analysis was used to identify the independent predictors of cases needing liver transplantation. Statistical analysis used Continuous variables were expressed as mean±SD and categorical variables as proportions. Univariate analysis for the two groups were carried out using the independent samples t test for continuous variables and χ2 test for categorical variables. Logistic regression analysis was used to identify the independent predictors of cases needing liver transplantation. Receiver operating characteristic analysis was used for the cut-off determination of predictive parameters using MedCalc application, version 18.2.1. Results The mean age at the time of operation was 74.9±14.6 days after birth (range, 31–111 days); there were 70 (46.7%) male babies and 80 (53.3%) female babies. The mean preoperative weight was 4.6±0.6 kg, while the mean preoperative height was 57.3±3.58 cm. Total bilirubin and albumin levels at 1 and 3 months following KPE were associated with death or the need for liver transplantation; however, only a total bilirubin level of more than 6 mg/dl at 3 months is an independent predictor of the need for liver transplantation. Age at the time of KPE and postoperative steroid use were not associated with improved transplant-free survival. Conclusion Total bilirubin level of more than 7.3 mg/dl at 1 month and a level of more than 6 mg/dl at 3 months after KPE are predictive of the need for liver transplantation (P

Prognostic markers at adolescence in patients requiring liver transplantation for biliary atresia in adulthood

In patients with biliary atresia (BA), the rate of native liver survival (NLS) to adulthood has been reported as 14-44% worldwide. Complications related to portal hypertension (PHT) and cholangitis are common in adulthood. For those requiring liver transplantation (LT), the timing can be challenging. The aim of this study was to identify variables that could predict whether young people with BA would require LT when they are >16 years of age. This study was a single-centre retrospective analysis of 397 patients who underwent Kasai portoenterostomy (KP) between 1980-96 in the UK. After KP, 111/397 (28%) demonstrated NLS until 16 years of age. At final follow-up, 67 showed NLS when >16 years old (Group 1) and 22 required LT when >16 years old (Group 2). Laboratory, clinical and radiological parameters were collected for both groups at a median age of 16.06 years (13.6-17.4 years). The need for LT when >16 years old was associated with higher total bilirubin (hazard ratio 1.03, p = 0.019) and lower creatinine (hazard ratio 0.95, p = 0.040), at 16 years, on multivariate analysis. Receiver-operating characteristic curve analysis demonstrated that a total bilirubin level of ≥21 µmol/L at 16 years old (AUROC = 0.848) predicted the need for LT when >16 years old, with 85% sensitivity and 74% specificity. Cholangitis episode(s) during adolescence were associated with a 5-fold increased risk of needing LT when >16 years old. The presence of PHT or gastro-oesophageal varices in patients <16 years old was associated with a 7-fold and 8.6-fold increase in the risk of needing LT, respectively. BA in adulthood requires specialised management. Adult liver disease scoring models are not appropriate for this cohort. Bilirubin ≥21 µmol/L, PHT or gastro-oesophageal varices at 16 years, and cholangitis in adolescence, can predict the need for future LT in young people with BA. Low creatinine at 16 years also has potential prognostic value. Patients with biliary atresia commonly require liver transplantation before reaching adulthood. Those who reach adulthood with their own liver are still at risk of needing a transplant. This study aimed to identify tests that could help clinicians predict which patients with biliary atresia who reach the age of 16 without a transplant will require one in later life. The study found that the presence of bilirubin ≥21 µmol/L, lower creatinine levels, and a history of portal hypertension or gastro-oesophageal varices at 16 years, as well as cholangitis in adolescence, could predict the future likelihood of needing a liver transplant for young people with biliary atresia.

Efficacy and safety of Yinchen Sini decoction in treating biliary atresia patients after Kasai portoenterostomy: A systematic review and meta-analysis protocol.

Background:Biliary atresia (BA) is a neonatal obstructive biliary tract disease in which the intrahepatic and extrahepatic bile ducts are obstructed and can lead to congenital biliary atresia of cholestatic cirrhosis and eventually liver failure. It has been confirmed that the Kasai portoenterostomy is an effective treatment for BA. But most patients still face complications, such as cholangitis and liver fibrosis. Yinchen Sini decoction (YCSND), a traditional herbal formula, is used as a treatment for BA after Kasai portoenterostomy. And it is supported that YCSND can improve jaundice and liver fibrosis through multiple targets and pathways. Based on the published literature, this study aims to evaluate the current situation in the treatment of BA in children with YCSND.Methods:The following databases will be searched until October 2018: PubMed, The Cochrane Library, Embase, Web of Science, China National Knowledge Infrastructure (CNKI), Chinese biomedical literature database (CBM), Wan Fang Database, Chinese Scientific Journals Database (VIP) and other sources such as Hand searching, Conference proceeding, International Clinical Trials Registry Platform and Chinese Clinical Trials Registry. All randomized controlled trials (RCTs) of YCSND or related formula as a treatment for postoperative patients of Kasai portoenterostomy for BA will be collected. Data extraction and risk of bias assessments will be carried out by 2 verifiers independently. The risk of bias will be evaluated through the Cochrane risk of bias tool. Review Manager software (RevMan V.5.3.0) and STATA 15 will be used for statistical analyses.Results:This study will provide a high-quality synthesis of current evidence of YCSND in treating children undergoing Kasai portoenterostomy for BA from several aspects.Conclusion:The conclusion of the meta-analysis will offer evidence for deciding whether YCSND is an effective measure for children undergoing Kasai portoenterostomy for BA.Ethics and dissemination:Not only will this systematic review be published in a peer-reviewed journal, but it will also be propagated electronically and in print. The review will bring patients benefit and provide practitioners reference in the fields of conventional medicine.PROSPERO registration number:PROSPERO CRD 42018111321.

Biliary atresia: 20–40-year follow-up with native liver in an Italian centre

IntroductionBiliary atresia (BA) represents the leading indication for liver transplantation in childhood. Only few studies reported the outcome of patients who survived more than 20 years on their native liver, and up to date there are no Italian data available. We reported our 40-year single centre experience with long-term follow-up of BA patients. Materials and methodsAll consecutive patients who underwent Kasai portoenterostomy (KPE) for BA managed at our Institution between 1975 and 1996 were retrospectively reviewed. Native liver (NLS) and overall survival (OS) were analyzed with Kaplan-Meyer curves and LogRank test. A p value of <.05 was regarded as significant. Quality of life of patients currently surviving with their native liver was assessed through a quality of life questionnaire. ResultsDuring the 22-year period of the study 174 patients underwent surgery (median age 60 days). Clearance of jaundice at 6 months from surgery was achieved in 90 patients (51.7%). NLS was 41% at 5 years, 32% at 10 years, 17.8% at 20 years and 14.9% at 40 years. Cholangitis was recorded in 32%, hepatocellular carcinoma in 0.5%. Twenty-six patients (14.9%) survived with their liver more than 20 years; 84.6% had normal serum bilirubin level and 23% had esophageal varices. Quality of life was comparable with the healthy Italian population in all but one patient. ConclusionsOur Italian experience confirms KPE represents the cornerstone of treatment for children with BA. Multidisciplinary and meticulous lifelong post-operative follow-up should be guaranteed for these patients because of the possibility of late-onset cholangitis, portal hypertension, hepatic deterioration and liver malignant tumors. Type of the studyretrospective case series. Level of evidenceIV.

Using 99mTc-DTPA galactosyl human serum albumin liver scintigraphy as a prognostic indicator in jaundice-free patients with biliary atresia

BackgroundWe evaluated the clinical significance of follow-up data, including 99mTc-DTPA galactosyl human serum albumin (99mTc-GSA) liver scintigraphy data, as prognostic indicators for jaundice-free patients with biliary atresia (BA). MethodsOf 87 patients who underwent Kasai portoenterostomy (KP) between 1991 and 2012, 45 jaundice-free patients aged 1–2 years underwent 99mTc-GSA scintigraphy and were classified into 2 groups: those who survived with a native liver (Group A, n = 34) and those who required liver transplantation (LTx) (Group B, n = 11). We compared 99mTc-GSA scintigraphy data (HH15, LHL15, and HH15/LHL15 [H/L15]) and liver function test (LFT) results between the groups. The patients underwent a second 99mTc-GSA scintigraphy at approximately 5 years of age. ResultsAll patients survived. HH15, H/L15, total bilirubin, direct bilirubin, gamma-glutamyl transpeptidase, and alanine transaminase levels were higher in Group B than in Group A (p<0.05). Total and direct bilirubin levels were associated with H/L15 (p<0.05). There were no significant changes in results between the first and second 99mTc-GSA scintigraphy in Group A. ConclusionsMid- and long-term prognoses may be predicted using 99mTc-GSA scintigraphy data and LFTs in patients aged 1–2 years. We recommend regular monitoring of postoperative data following KP, even in jaundice-free patients. Level of evidenceIII.