Search

Abstract Background The study aims to investigate Shear Wave Elastography's accuracy in assessing pediatric cholestasis and differentiating biliary atresia from other causes. The early diagnosis of cholestasis in newborns, when caused by true liver disease and not physiological or transient jaundice is usually eluded. To maximize the diagnostic reliability of ultrasound as an initial imaging modality to diagnose biliary atresia, ultrasound elastography has been introduced as a quantitative non-invasive sonographic technique to measure liver stiffness which is presumed to be different in biliary atresia from other cholestasis as well as follow-up liver stiffness post-Kasai operation. Results Ten patients (34.48%) were diagnosed with cholestasis with normal/low GGT. Five patients (17.24%) were diagnosed with hepatitis, and 2 patients had syndromes (10.34%). Other causes were found in 11 patients (37.93%). Major bile duct obstruction with fibrosis/cirrhosis was the majority of findings found in biliary atresia patients (100%), most of them with mild fibrosis (40%), followed by moderate (36.67%), then marked fibrosis (13.33%) and secondary cirrhosis (10%). ROC curve analysis shows that the best cut-off value for SWE = 1.97 with a sensitivity of 75.0% and specificity of 83.5%, with positive and negative predictive values were 82.8% and 74.1%, respectively. Mean shear wave elastography was significantly higher among the biliary atresia group (median 2.86) than in non-biliary atresia (median 1.81) and control groups (median 1.64) (P < 0.001) The best cut-off value of shear wear elastography for predicting outcome in pediatric patients post-Kasai procedure was 3.55, with a sensitivity of 66.7%, specificity of 64.7%, positive and negative predictive values were 25% and 91.7% respectively, with accuracy 65%. Conclusion The results of this study suggest that shear-wave elastography is a useful diagnostic tool for differentiating biliary atresia from other causes of cholestasis in pediatric patients, with a high sensitivity and specificity. Further studies are needed to validate the findings and determine the optimal cut-off value for shear wear elastography in clinical practice.

Background: The study aims to investigate Shear Wave Elastography's accuracy in assessing pediatric cholestasis and differentiating biliary atresia from other causes. The early diagnosis of cholestasis in newborns, when caused by true liver disease and not physiological or transient jaundice is usually eluded. To maximize the diagnostic reliability of ultrasound as an initial imaging modality to diagnose biliary atresia, ultrasound elastography has been introduced as a quantitative non-invasive sonographic technique to measure liver stiffness which is presumed to be different in biliary atresia from other cholestasis as well as follow-up liver stiffness post-Kasai operation. Results: Ten patients (34.48%) were diagnosed with cholestasis with normal/low GGT. Five patients (17.24%) were diagnosed with hepatitis, and 2 patients had syndromes (10.34%). Other causes were found in 11 patients (37.93%). Major bile duct obstruction with fibrosis/cirrhosis was the majority of findings found in biliary atresia patients (100%), most of them with mild fibrosis (40%), followed by moderate (36.67%), then marked fibrosis (13.33%) and secondary cirrhosis (10%). ROC curve analysis shows that the best cut-off value for SWE = 1.97 with a sensitivity of 75.0% and specificity of 83.5%, with positive and negative predictive values were 82.8% and 74.1%, respectively. Mean shear wave elastography was significantly higher among the biliary atresia group (median 2.86) than in non-biliary atresia (median 1.81) and control groups (median 1.64) (P < 0.001) The best cut-off value of shear wear elastography for predicting outcome in pediatric patients post-Kasai procedure was 3.55, with a sensitivity of 66.7%, specificity of 64.7%, positive and negative predictive values were 25% and 91.7% respectively, with accuracy 65%. Conclusion: The results of this study suggest that shear-wave elastography is a useful diagnostic tool for differentiating biliary atresia from other causes of cholestasis in pediatric patients, with a high sensitivity and specificity. Further studies are needed to validate the findings and determine the optimal cut-off value for shear wear elastography in clinical practice. [ABSTRACT FROM AUTHOR]

Controversies in management of biliary atresia (BA) after hepatoportoenterostomy (HPE) lead to variable treatment protocols. We implemented standardized medical management after HPE, customizing the use of antibiotics and corticosteroids based on patient-specific factors. In this retrospective analysis, 20 consecutive infants underwent HPE for BA and were compared to a historical cohort. Analysis of successful biliary drainage 3 months after HPE (defined as serum total bilirubin <2 mg/dL) was the primary endpoint; survival with native liver at 2 years was the secondary endpoint. Sixteen of 20 (80%) infants had successful bile drainage, compared to 8 of 20 (40%) infants in the historical cohort (P = 0.0225). Sixteen of 20 patients in the new protocol have reached 2 years of age or required liver transplantation. Among the sixteen, 11 (68.8%) are alive with native livers versus 10 of 20 (50%) in the historical cohort (P = 0.0970). This preliminary report suggests the potential benefit of tailored use of postoperative antibiotics and corticosteroids in improving biliary drainage after HPE. III. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Tikrit University for Humanities is the property of Republic of Iraq Ministry of Higher Education & Scientific Research (MOHESR) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Biliary atresia (BA) is a liver disease of infancy characterized by obstruction of the biliary tree. Infants with BA have the best outcomes when identified early and the Kasai portoenterostomy is performed before 45 days of life (DoL). In our hospital system, the average age at Kasai was 60 DoL. To address the problem of late presentation, we implemented a two-stage BA screening strategy utilizing direct bilirubin (DB).New institutional policies were established that all newborns were tested at 24–48 h of life, and those with levels ≥0.5 mg/dL were followed further. The infant's primary care provider was contacted to recommend a repeat DB at 2 weeks of life. If the repeat DB was ≥1.0 mg/dL, the patient was evaluated by gastroenterology.Over the 16 months, 3880 infants were born and 3861 (99.5%) were screened; 53 infants (1.3%) had DB levels ≥0.5 mg/dL initially. Upon repeat testing at 2 weeks, there were three groups of infants: not retested (n = 1), retested <1.0 mg/dL (n = 40), and retested ≥1.0 mg/dL (n = 12). The average time to be seen by gastroenterology was 4.3 days or 18.3 DoL.The screening included a series of steps that needed to be implemented effectively. Screening had a net false positive rate of 0.3% (12 out of 3861) and identified causes of cholestasis other than BA. BA was excluded by 28 DoL on average. Our results can provide a template for other institutions interested in implementing a BA screening protocol in their practice. [ABSTRACT FROM AUTHOR]

Meconium peritonitis (MP) combined with intestinal atresia (IA) is a rare neonatal condition, and it is even rarer in combination with biliary atresia (BA). We describe a case of an infant who developed short bowel syndrome after partial intestinal resection due to MP and IA, along with a Santullienterostomy. During continuous enteral and parenteral nutrition, the stool color became paler. BA was identified by elevated direct bilirubin (DBIL), gamma-glutamyltransferase (GGT), serum matrix metalloproteinase-7 (MMP-7), and hepatobiliary ultrasound; then, Kasai portoenterostomy (KPE) was performed promptly. The Roux-en-Y limb was adjusted intraoperatively to preserve the maximum length of the small intestine while closing the enterostomy. After the operation, the infant gradually adapted to enteral nutrition, his bilirubin level returned to normal, and his weight gradually caught up to the normal range. Although rare, BA should be suspected when MP is combined with IA and when the stool becomes paler in color in the enterostomy state.

Background: Biliary atresia (BA) is a serious pediatric condition that tends to progress to cirrhosis, liver failure, and death within a short time. It is the result of a continuous inflammatory, sclerosing, destructive process in the biliary tract and the most common indication for liver transplantation. Material and methods: The study included 46 patients up to 1 year of age hospitalized with cholestasis syndrome in IMSP IM and C, during the years 2014-2019. The basic methods in the diagnosis of BA were the biochemical examination, FGDS, USG doppler dupplex color of the biliary system before and after the meal intake, MRI with cholangiography, dynamic hepatobiliary scintigraphy. Results: Following the analysis of clinical and paraclinical results, surgical pathology was excluded in 25 patients, the diagnosis of BA was established in 11 cases. 6 patients with BA underwent Kasai surgical intervention , a primary liver transplant was performed in 3 cases, and 2 patients died before the surgery. Conclusions: Portoenteroanastamosis ( Kasai operation) performed as early as possible (up to 60 days postnatal) considerably increases life expectancy. The embryonic form of BA is a severe condition that is indicated for the initial liver transplant.The prognosis of untreated biliary atresia is unfavorable, leading to the death of most children in the first 2 years of life due to liver failure. In decopensated late-diagnosed cases, liver transplantation remains the only treatment option.

BackgroundThe failed clearance of jaundice (CJ) in patients with biliary atresia (BA) after the Kasai procedure (KP) often leads to a shorter native liver survival (NLS) time and earlier liver transplantation. We aimed to investigate risk factors of failed CJ and establish a novel nomogram model to predict the status of CJ.MethodsWe retrospectively reviewed institutional medical records from January 2015 to April 2020 and enrolled BA patients post-KP, randomly divided into training and testing cohorts at a ratio of 7:3, and further subdivided into cleared and uncleared jaundice groups. Univariate and multiple logistic regression analyses were used to select risk factors to establish the nomogram in the training cohort. The performance of the nomogram was evaluated by calculating the areas under the receiver operating curve (AUC) in both cohorts.ResultsThis study included 175 BA patients post-KP. After univariate and multiple logistic regression analyses, Cytomegalovirus IgM +ve associated BA (OR = 3.38; 95% CI 1.01–11.32; P = 0.04), ln γ-glutamyl transpeptidase (GGT) (OR = 0.41; 95% CI 0.22–0.80; P = 0.009), thickness of the fibrous portal plate (OR = 0.45; 95% CI 0.27–0.76; P = 0.003), liver stiffness measurement (LSM) (OR = 1.19; 95% CI 1.06–1.34; P = 0.002), and multiple episodes of cholangitis (OR = 1.65; 95% CI 1.13–2.41; P = 0.01) were identified as independent risk factors of unsuccessful CJ to construct the nomogram. The receiver operating characteristic curve (ROC) analysis suggested good nomogram performance in both the training (AUC = 0.96) and testing cohorts (AUC = 0.91).ConclusionOur nomogram model including several risk factors effectively predicts CJ in patients post-KP, which could aid in clinical decision-making.

Hepatoportoenterostomy remains the cornerstone of treatment for biliary atresia. Current employed techniques include laparoscopy and open surgery. This study aims to determine if either method provides an advantage. Following PRISMA guidelines, a systematic review was conducted. Nineteen studies were included. Mean operative time 34.98 (95% CI 20.10, 49.85; p ≤ 0.00001) was longer in laparoscopic while bleeding volumes - 16.63 (95% CI - 23.39, - 9.86; p ≤ 0.00001) as well as the time to normal diet - 2.42 (95% CI - 4.51, - 0.32; p = 0.02) were lower in the laparoscopic group. No differences were observed in mean length of stay - 0.83. Similar complication, transfusions, postoperative cholangitis 0.97, and transplant free survival rates 1.00 (0.63, 1.60; p = 0.99) were seen between groups. Laparoscopic portoenterostomy provides advantages on operative time and bleeding as well as to normal diet when compared to open procedures. Both procedures showed no differences in length of stay, complications, cholangitis, and importantly, native liver survival. Level of evidence: III. [ABSTRACT FROM AUTHOR]

We test the applicability of bivalve shell oxygen isotope composition to reconstruct hydrological dynamics in four riverine sites in the Congo River basin. Twenty-three specimens from the Unionoida order were collected from locations where long-term discharge data are available, and in situ measurements and water samples were collected over several years. Due to the highly variable (species-specific) shell morphology, various sampling techniques were used to analyze the shell sections; however, every specimen recorded the seasonality of the host water oxygen stable isotope composition (δ18O w) in its δ18O shell record. Discharge data showed an inverse relationship with δ18O w values, which was well described with a logarithmic fit. An exception was the Kasai River, where the δ18O w record shows an additional peak occurring during the high discharge period, which renders the discharge-δ18O w relationship more complex than in the other systems investigated. Low ratios of maximum to minimum discharge (Q max /Q min) were found to result in a low δ18O w amplitude, which was reflected as low δ18O shell variability. The Congo and Kasai rivers had Q max /Q min ratios ~2 to 2.5, while the Oubangui showed a much higher Q max /Q min (~19). Shells correspondingly showed a large δ18O shell range (amplitude between 2.4 and 5.0‰) for individual Oubangui shells, and lower amplitude for other sites (1.0 to 2.2‰). Thus, shells have a high resolving power to be used to record hydrological variability, since long-term changes in precipitation pattern, discharge, land-use change, or other hydrological changes have an influence on δ18O w values. Shells with wide range of δ18O values reflect high seasonal variability in rivers, while shells with lower δ18O amplitude correspond to sites with more steady river conditions over the year. Our study illustrates that fossil shell δ18O values could indicate Q max /Q min values in ancient African river systems. [ABSTRACT FROM AUTHOR]

In RCT of adults with decompensated cirrhosis, GCSF mobilizes hematopoietic stem cells HSC and improves short-term outcome. An FDA-IND for sequential Kasai-GCSF treatment in biliary atresia BA was approved. This phase 1 study examines GCSF safety in Kasai subjects. Preliminary short-term outcome was evaluated. GCSF (Neupogen) at 5 or 10 μg/kg (n = 3/group) was given in 3 daily doses starting on day 3 of Kasai surgery (NCT03395028). Serum CD34+ HSC cell counts, and 1-month of GCSF-related adverse events were monitored. The 6-months Phase 1 clinical outcome was compared against 10 subsequent post Phase 1 Kasai patients who did not receive GCSF. With GCSF, WBC and platelet count transiently increased, LFT and serum creatinine remained stable. Reversible splenic enlargement (by 8.5–20%) occurred in 5/6 subjects. HSC count increased 12-fold and 17.5-fold for the 5 μg/kg and10 ug/kg dose respectively; with respective median total bilirubin levels for GCSF vs no-GCSF groups of 55 vs 91 μM at 1 month, p = 0.05; 15 vs 37 μM at 3 months, p = 0.24); and the 6-months cholangitis frequency of 40% vs 90%, p = 0.077. GCSF safely mobilizes HSC in Kasai infants and may improve short-term biliary drainage and cholangitis. Phase 2 efficacy outcome of GCSF adjunct therapy for sequential Kasai and GCSF is pending. [ABSTRACT FROM AUTHOR]

Background/Aims: Data from Western countries indicate that biliary atresia (BA) is the leading cause of end-stage liver disease in children and the most common indication for liver transplantation (LT) in the pediatric population. There is no data on the epidemiology and outcome of BA in Saudi Arabia. The main objective of our study was to understand the clinical and epidemiological characteristics and outcome of BA in the Saudi population. Patients and Methods: We retrospectively reviewed the database of infantile cholestasis cases that presented to our center from 2008 to 2015 and identified BA cases. Data on clinical, biochemical, imaging, and histopathological characteristics were collected by chart review. The two primary study outcomes were (1) successful Kasai portoenterostomy (KPE) defined as resolution of jaundice (total serum bilirubin

Objectives: In 2022, the Biliary Atresia and Related Diseases (BARD) community reached a consensus for the definition of suspected and confirmed cholangitis for biliary atresia (BA) patients after hepatoportoenterostomy (HPE). This study assessed the new standardized BARD definition in a retrospective, multicenter cohort study., Methods: We included BA cases managed between 2010 and 2020 at the Hannover Medical School and Geneva University Hospitals' Swiss Pediatric Liver Center. The standardized BARD cholangitis definition assesses four clinical items and four imaging/laboratory items to define cholangitis. The definition was retrospectively applied to all BA cases having presented, according to their physician, cholangitis within the first year after the HPE. The diagnosis defined by the standardized BARD definition was compared with the final clinical diagnosis made by physicians. The Spearman's correlation coefficient was used to test for correlation between diagnoses made by standardized and clinical appreciation., Results: Of 185 consecutive BA patients, 59 (32%) had at least one episode of cholangitis within the first year after HPE. The correlation between the clinician's impression and the standardized BARD definition was very strong ( r  = 0.8). Confirmed cholangitis definition coincided with the clinician's impression (2.5 [±0.7]/4 clinical items, 2.6 [±0.5]/4 imaging/laboratory items). For suspected cholangitis, the threshold for diagnosis was lower within the standardized BARD definition (1.1 [±0.3]/4 clinical items, 2.2 [±0.8]/4 laboratory/imaging items)., Conclusions: This first retrospective application of the standardized BARD cholangitis definition reveals a very strong correlation with the physician's assessment before standardization. A prospective study is needed to further refine the standardized definition for cholangitis in BA patients., Competing Interests: The authors declare no conflict of interest., (© 2024 The Authors. JPGN Reports published by Wiley Periodicals LLC on behalf of The European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Kasai portoenterostomy (KPE) remains the first-line operation for patients with biliary atresia (BA), but ultimately fails in up to 60% of cases. This study sought to identify factors contributing to hospital readmission and early liver transplant. The Nationwide Readmissions Database from 2010 to 2014 was used to identify patients with BA who underwent KPE on index admission. Patient factors, hospital characteristics, and complications of BA were compared by readmission rates and rate of liver transplant within 1 y. The results were weighted for national estimates. Nine hundred and sixty three patients were identified. The readmission rate within 30-d was 36% (n = 346) and within 1-y was 67% (n = 647). Only 9% (n = 90) received a liver transplant within a year. The most common complications after KPE were cholangitis in 58%, decompensated cirrhosis in 54%, and recurrent jaundice in 34%. Male patients (OR 1.5, P = 0.02) with comorbid gastrointestinal anomalies (OR 2.1, P < 0.01) from lower income households (OR 4.6, P < 0.01) and early development of cirrhosis (OR 3.0, P < 0.01) were more likely to be readmitted. Liver transplant was more common in men (OR 4.0, P < 0.01) and those from lower income households (OR 5.2, P < 0.01) with decompensated cirrhosis (OR 8.6, P < 0.01), cholangitis (OR 5.0, P < 0.01), or sepsis (OR 5.7, P < 0.01) on index admission. This is the first nationwide study to evaluate readmissions in patients with BA undergoing KPE. Although KPE is a lifesaving procedure, hospital readmission rates are high and complications are common. Cholangitis, early progression of cirrhosis, and infections are highly associated with readmission and failure of KPE. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is a neonatal liver disease characterized by progressive obstruction and fibrosis of the extrahepatic biliary tree as well as fibrosis and inflammation of the liver parenchyma. Recent studies found that infants who will go on to develop BA have elevated direct bilirubin levels in the first few days of life, suggesting that the disease starts in utero. The etiology and pathogenesis of BA, however, remain unknown. Here, we discuss recent studies examining potential pathogenetic mechanisms of BA, including genetic susceptibility, involvement of the immune system, and environmental insults such as viruses and toxins, although it is possible that there is not a single etiological agent but rather a large group of injurious insults that result in a final common pathway of extrahepatic bile duct obstruction and liver fibrosis. The management and diagnosis of BA have not advanced significantly in the past decade, but given recent advances in understanding the timing and potential pathogenesis of BA, we are hopeful that the next decade will bring early diagnostics and novel therapeutics.

• Patients with biliary atresia are at risk of needing a liver transplant when >16 years old. • Higher bilirubin and lower creatinine at 16 years of age are predictors of the need for liver transplant. • Cholangitis and varices in adolescence increase the risk of needing a liver transplant when >16 years old. 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. [ABSTRACT FROM AUTHOR]

Objective: To study the ideal Kasai portoenterostomy (KPE) time for preterm infants with biliary atresia (BA) through evaluation of the postoperative effects.Methods: Retrospectively, 34 preterm infants with BA from 2012 to 2016 were recruited in the present study. The following three groups were established according to their chronological and corrected age at the time of KPE operation: chronological age ≤ 90 days, chronological age > 90 days and corrected age ≤ 90 days, and corrected age > 90 days. For chronological age ≤ 90 days at operation, patients were further divided into another three groups: chronological age ≤ 60 days, chronological age > 60 days and corrected age ≤ 60 days, and corrected age > 60 days. Postoperative effects were then followed up and recorded.Results: First, of those patients divided according to 90-day chronological and corrected age, postoperative total bilirubin levels (TBL), direct bilirubin levels (DBL), alanine aminotransferase (ALT), and aspartate aminotransferase (AST) of the group whose chronological age was ≤ 90 days were lower than the levels of the group whose chronological age was > 90 days and corrected age ≤ 90 days (P = 0.0472, P = 0.0358, P = 0.0083, and P = 0.0491), and the group whose corrected age was > 90 days (P = 0.0383, P = 0.0392, P = 0.0043, and P = 0.0107). Second, for those patients whose chronological age was ≤ 90 days, the group whose corrected age was > 60 days showed a higher ALT level than the other two groups with chronological age ≤ 60 days (P = 0.0472) and chronological age > 60 days and corrected age ≤ 60 days (P = 0.0258).Conclusion: According to the present study, the ideal KPE time for preterm BA infants should meet two conditions: chronological age ≤ 90 days and corrected age ≤ 60 days. The groups with a chronological age ≤ 60 days, and chronological age > 60 days and corrected age ≤ 60 days show similar postoperative effects. [ABSTRACT FROM AUTHOR]

Background/Aims: Data from Western countries indicate that biliary atresia (BA) is the leading cause of end-stage liver disease in children and the most common indication for liver transplantation (LT) in the pediatric population. There is no data on the epidemiology and outcome of BA in Saudi Arabia. The main objective of our study was to understand the clinical and epidemiological characteristics and outcome of BA in the Saudi population. Patients and Methods: We retrospectively reviewed the database of infantile cholestasis cases that presented to our center from 2008 to 2015 and identified BA cases. Data on clinical, biochemical, imaging, and histopathological characteristics were collected by chart review. The two primary study outcomes were (1) successful Kasai portoenterostomy (KPE) defined as resolution ofjaundice (total serum bilirubin <20 µmol/L) and (2) survival with native liver. Results: Over the study period, we evaluated 450 cases of infantile cholestasis. In all, 21 cases (11 males) were diagnosed with BA (4.7%). BA cases were first seen by pediatric gastroenterologists at a median age of 65 days. KPE was performed in 12 cases at a median age of 73 days. Successful KPE was achieved in four cases (33%). Five of the remaining eight cases had LT and three died before LT. Nine of the 21 BA cases were denied KPE and had primary LT at median 8 months of age. The native liver 4-year survival rate was 14.3% and the overall survival rate was 81%. Conclusion: BA is an uncommon cause of infantile cholestasis in Saudi Arabia. Our study provides a snapshot of the epidemiology of BA in Saudi Arabia that is characterized by late referral to pediatric gastroenterologists and poor outcome without LT. [ABSTRACT FROM AUTHOR]

Abstract Background To assess the outcome of patients with biliary atresia (BA) who underwent a surgical shunt (SS) for severe portal hypertension (PH) following a Kasai procedure. Methods We collected and analyzed the data and outcomes of patients with BA who underwent SS for severe PH following a Kasai procedure between 1974 and 2014, focusing on complications related to the procedure, overall survival (OS), and transplant-free survival (TFS). Results SS was performed at a median age of 5.5 years [2–13.5] in 38 patients. Conjugated bilirubin level (cBL) was ≤ 20 μmol/l in 24 patients at time of SS. Median follow-up was 15 years [1–32]. OS at 5 and 10 years was 91% and 87% respectively. TFS at 5 and 10 years was 84% and 70% respectively. Long-term complications included hepatic encephalopathy in 9 patients, and hepatopulmonary syndrome in 3. At last follow-up, 10/14 patients without LT and 18/ 24 who had a delayed LT at a median delay of 11 years [1.5–22] were alive. Conclusion Surgical shunt for severe portal hypertension in biliary atresia may delay the need for liver transplantation. However complications are indications for transplantation. Level of evidence Type of study: Therapeutic. Level of evidence III. [ABSTRACT FROM AUTHOR]

Background: Multicenter study was undertaken to analyze the results of laparoscopic and open Kasai portoenterostomy. Methods: Subjects were infants with type III biliary atresia who underwent open operation (n = 106) or laparoscopic operation (n = 21) between January 2012 and December 2015. Clinical data were compared between open and laparoscopic operations (2016‐0534). Propensity score matching was performed to reduce the effect of treatment selection bias. Multivariate analyses were used to estimate the effect of the surgical approach on the jaundice clearance rate and the native liver survival rate. Results: The postoperative jaundice clearance rate and the 1‐year native liver survival rate were not significantly different between open and laparoscopic operations. Rates of cholangitis and major complications of laparoscopic operation were comparable to those of open operation. Blood loss, time to resume oral intake, time to drain removal, and duration of analgesic usage of laparoscopic operation were significantly superior to those of open operation. Similar results were observed when analysis was adjusted based on propensity score. Multivariate analyses demonstrated that only age at operation was a poor prognostic factor. Conclusion: Laparoscopic Kasai portoenterostomy was associated with several favorable perioperative outcomes compared with open Kasai portoenterostomy. The difference of surgical approach was not a significant independent predictor. Highlight In this multicenter study, Murase and colleagues analyzed patients with biliary atresia who underwent either open or laparoscopic Kasai portoenterostomy. The laparoscopic procedure was associated with several favorable perioperative outcomes. The difference in surgical approach was not a significant independent predictor, and only age at surgery was a poor prognostic factor. [ABSTRACT FROM AUTHOR]

Introduction:  Biliary atresia (BA) is a rare destructive inflammatory obliterative cholangiopathy of neonates. Early diagnosis is important in disease management. The aim was to evaluate the role of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosing BA in a large cohort. In addition, we evaluated whether parameters such as bile trace, GGT, bilirubin, and laboratory values in combination can be used to develop a risk score that could indicate the referral to specialized centers.Materials and Methods:  All infants with neonatal cholestasis (2000-2014) who presented to our endoscopy unit for suspected BA were included. Demographics, laboratory parameters, ultrasound findings, liver biopsy results, ERCP diagnosis, and surgical outcome were collected. Value and safety of ERCP and risk factors for BA were retrospectively analyzed.Results:  We included 251 infants in our cohort (55% males, median age: 53 days). BA was intraoperatively diagnosed in 155 (83.4%) patients and was excluded in 30 (16.2%). Fifty-six cases were not operated due to the ERCP findings. ERCP was successful in 224/251 patients (89.2%) with no procedure-related complications. The operative and endoscopic diagnosis matched in 96.6% of the patients (positive predictive value: 92.2%, negative predictive value: 97.1%). In comparison to cases with excluded BA, the ones with this disease were significantly associated with absence of duodenal bile traces (98.4 vs. 1.6%, p < 0.001), higher bilirubin (p < 0.001, cutoff 7.3 mg/dL), and higher GGT (p < 0.001, cutoff 250 U/L).Conclusion:  ERCP is safe and accurate in the hands of experts in diagnosing BA if the cause of cholestasis is unclear. While evaluating the role of ERCP for diagnosing this disease, we found that the secondary parameters GGT > 250 U/L, bilirubin > 7.3 mg/dL (125 μmol/L), and the absence of bile traces are risk factors. [ABSTRACT FROM AUTHOR]

Objectives: The importance of case load for treatment of extrahepatic biliary atresia (BA) is debated. The aim of this study was to register results of BA treatment in a small volume centre. Methods: Retrospective chart review study of patients with BA treated from 2000 to 2017. The institutional review board approved the study. Results: Forty-five babies were identified of which 42 (93%) are alive. Forty-one patients had a Kasai portoenterostomy (PE), two had a hepaticojejunostomy and two had a primary liver transplantation. The age at PE/hepaticojejunostomy was median 63 (4-145 days). Seven surgeons performed the operations, and the median duration of the diagnostic work-up was 8 (3-24) days. Clearance of jaundice was achieved in 23/43 (53%) babies, and 3- and 5-year native liver survival was 47% and 40%, respectively. Clearance of jaundice post-PE/hepaticojejunostomy was a strong predictor of native liver survival (adjusted OR: 0.027; 95%; p = .009). Plasma level of conjugated bilirubin at time of referral was also a significant predictor of native liver survival (adjusted OR: 1.053; p = .017). Conclusion: A small volume centre may achieve satisfactory results for BA patients. The study has, however, identified factors that may further improve results; earlier referral, optimizing diagnostic work-up and establishing one dedicated surgical team. [ABSTRACT FROM AUTHOR]

Biliary atresia is characterized by the obliteration or discontinuity of the extrahepatic or intrahepatic biliary system, resulting in the obstruction of bile flow, the disorder representing the most common surgically treatable cause of cholestasis encountered during the newborn period. Recent studies show that biliary atresia is the most common hepatobiliary malformation with manifestations in the neonatal period, characterized by a progressive inflammatory lesion, which suggests a role of infectious and/or toxic agents causing bile duct obliteration. No single etiologic factor has been identified, but infectious agents seem to be the most plausible candidates, particularly in the isolated (neonatal) form of atresia. Prior to the development of liver transplantation as a therapeutic option for children with end-stage liver disease, the long-term survival rate for infants with biliary atresia following portoenterostomy was 47-60% at 5 years and 25-35% at 10 years. Extrahepatic biliary atresia is more common in females than in males. Biliary atresia is a disorder unique to the neonatal period. Once biliary atresia is suspected, surgical intervention is the only option available for a definitive diagnosis (intraoperative cholangiogram) and therapy (Kasai portoenterostomy). [ABSTRACT FROM AUTHOR]

Aim: The aim of this report was to present the laparoscopic portoenterostomy (LapPE) procedure developed by the Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine (JLapPE). We also attempted to obtain an understanding of the current status of laparoscopic portoenterostomy in the world as reported in the English literature to compare with our experience.Methods: There were 22 BA patients who had JLapPE between 2009 and 2016. BA classification was type III (n = 19) and type II (n = 3). There was 1 case of syndromic BA and 1 case was positive for cytomegalovirus. A systematic search in PubMed of all BA patients treated by LapPE in the English literature was conducted. Jaundice clearance (JC) and survival with the native liver (SNL) were compared.Results: Mean age at JLapPE was 67.1 days (range 29-119). Mean postoperative follow-up was 4.6 years (1.3-8.3). Mean operative time was 514 min (240-662) and mean blood loss was 13.4 g (3-21). Postoperative JC (Total bilirubin ≤ 1.5 mg/dL) was 77.3% (17/22) at 3 months and 90.9% (20/22) at 6 months. SNL at 6 months of age was 90.9% (20/22); at 1 year of age was 77.3% (17/22), at 2 years of age was 73.7% (14/19); and at 3 years of age was 81.3% (13/16).Conclusions: Despite recent reports that outcome of LapPE for BA may be unfavorable compared with the conventional open portoenterostomy, our results would suggest that JLapPE can be performed successfully, because it is performed exactingly according to a standard protocol. JLapPE will continue to be our procedure of choice for treating BA. [ABSTRACT FROM AUTHOR]

Purpose of Review: Biliary atresia is the most common indication for liver transplantation among children. In recent years, prospective, multi-centre collaboration has been underway with the aim of providing high-quality data on the natural history of the condition, prior to and following hepatic portoenterostomy. Recent Findings: There is increasing evidence that specific histological findings, and age, at the time of portoenterostomy have relevance as prognostic indicators. Recent data suggest that the sub-type of biliary atresia, its co-existence with other anomalies and concurrent infection may also be important variables. Summary: This review provides a comprehensive summary of the histological predictors of outcome following portoenterostomy. Later age at portoenterostomy, advanced degree of hepatic fibrosis and co-existence with other congenital anomalies are strongly correlated with poor outcomes following portoenterostomy. There is increasing evidence to suggest that common serological indices and the presence or absence of cytomegalovirus (CMV) co-infection may have utility as early prognostic indicators. [ABSTRACT FROM AUTHOR]

We investigated the overall situation of LT for BA in mainland China and analyzed their survival outcomes based on data from CLTR. Between January 1996 and December 2013, 509 liver transplants for BA were performed in mainland China and were included in this study. Patients' median age was 9.6 months (range: 4.8-175.2 months). KP was previously performed in 194 cases (38.1%). Grafts from living donors were used in 380 cases (74.7%). Era I (1996.1-2010.12) and era II (2011.1-2013.12) comprised 151 cases (29.7%) and 358 cases (70.3%), respectively. Twenty-five centers had performed at least one liver transplant for children with BA. Centers from Shanghai (197 cases), Tianjin (143 cases) and Beijing (81 cases) involved 82.7% of the 509 cases. One- and five-yr graft survival rates were 84.7% and 72.6%, respectively. Split grafts, center volume <20, GRWR ≥4.0%, and steroid-free immunosuppression regimen were independent risk factors for graft survival. In conclusion, the dramatic expansion of pediatric liver transplant programs in mainland China has enabled improved survival for those children affected by this devastating disease. However, screening of BA in neonates should be emphasized throughout the country to enhance early referrals for KP. [ABSTRACT FROM AUTHOR]

Abstract Infants with cholestasis and conjugated hyperbilirubinemia persisting beyond two weeks of age must be evaluated promptly to exclude biliary atresia (BA). BA progresses to cirrhosis and liver failure if portoenterostomy (PE) is not performed in a timely fashion. The gold standard to diagnose BA is intraoperative cholangiography (IOC) via laparotomy. Neonates that are critically ill or have significant co-morbidities may be high-risk surgical candidates, and thus minimizing operative time or avoiding an operation may be beneficial. An additional modality for excluding BA is percutaneous transhepatic cholecysto-cholangiography (PTCC). We present three infants undergoing PTCC by interventional radiology (IR) revealing patency of the biliary system without the need for IOC. [ABSTRACT FROM AUTHOR]

Background Recent follow-up studies have demonstrated significant improvement in overall survival as well as survival with native liver following geographic centralization of services to three centres in the UK. However, this model has not been replicated in countries with relatively low population density such as Australia and Canada. Methods Retrospective evaluation of all patients born with biliary atresia ( BA) in South Australia from 1989 to 2010 was performed. Thirty-one patients with BA were discovered. Two patients were excluded because the initial Kasai procedure ( KP) was performed interstate. Outcome parameters measured were (i) clearance of jaundice (bilirubin of less than 20 μmol/L, by 6 months); (ii) survival with native liver; and (iii) overall survival. Kaplan- Meier survival curves were plotted for both survival with native liver and overall survival. Results The incidence of BA in South Australia between 1989 and 2010 was 7.48 per 100 000 live births. Following KP, clearance of jaundice was achieved in 42.9% of patients. Five-year actuarial survival with native liver was 55.2%, and overall 5-year actuarial survival was 89.3%. Conclusions The results of KP performed at Women's and Children's Hospital from 1989 to 2010 can be considered comparable with international benchmarks. Based on these results, we propose the creation of a 'centralized' pool of surgeons in Australia to help continue providing 'decentralized' care of BA. [ABSTRACT FROM AUTHOR]

Purpose : To report the results of surgical treatment of biliary atresia with patent distal extra hepatic bile ducts (BA with PDEBD) with special reference to hepatic portocholecystostomy (HPC) operation. Materials and Methods : The study reviews records of children operated for BA with PDEBD. The type of operation, results of surgery, postoperative course and complications during follow-up are noted. Results : Five children (mean age 83 days) underwent surgery for biliary atresia with patent extra hepatic bile ducts. The diagnosis was confirmed by intraoperative cholangiography in each case. Three children underwent HPC and two had standard hepatic portoenterostomy (HPE) as HPC was not technically feasible. The operation was considered successful in three of five children (60%, two HPC and one HPE), partially successful in one. The mean follow-up was 22 months. None of the children with HPC had cholangitis at follow-up; one child with HPE had recurrent cholangitis. Conclusions : Biliary atresia (BA) with PDEBD may be a variant with a fair chance for surgical success. When feasible, HPC may be a good treatment option in this group with acceptable results and practically no risk of postoperative cholangitis.

Purpose: Apparently superior result was observed after open Kasai portoenterostomy in infant with biliary atresia. Our institute stopped performing laparoscopic portoenterostomy since 2007. We aimed to investigate the outcome after reintroduction of open portoenterostomy. Methods: 27 non-syndromic infants underwent open Kasai portoenterostomy from 2007 to 2012. The age and the sex of the patient, the bilirubin level before the operation, the early clearance of jaundice (total bilirubin <20 μmol/L within 6 month of portoenterostomy), the native liver survival at 2 years after the operation were reviewed. The results were retrospectively compared with all 16 infants who underwent laparoscopic Kasai portoenterostomy before 2007. Results: All infants had type III biliary atresia. No statistical difference was observed regarding the age at operation and the pre-operative bilirubin level. The early clearance of jaundice rate was 81 % (22/27) after open operation and was 50 % (8/16) after laparoscopic operation ( p = 0.03). At 2 years after the operation, the native liver survival was 81 % (22/27) after open operation and was 50 % (8/16) after laparoscopic operation ( p = 0.03). Conclusion: Reintroduction of open Kasai portoenterostomy was associated with superior early clearance of jaundice rate and 2-year native liver survival rate. [ABSTRACT FROM AUTHOR]

Purpose: Laparoscopic Kasai portoenterostomy was reported to be a safe and feasible procedure in infant with biliary atresia. We aimed to investigate the long-term results after laparoscopic portoenterostomy as such data in the literature are lacking. Methods: Sixteen infants underwent laparoscopic Kasai portoenterostomy from 2002 to 2006. The age and the sex of the patient, the bilirubin level before the operation, the early clearance of jaundice (total bilirubin <20 μmol/L within 6 months of portoenterostomy), the native liver survival at 2 and 5 years after the operation were reviewed. The results were retrospectively compared with 16 consecutive infants who underwent open Kasai portoenterostomy before 2002. Results: All infants had type III biliary atresia. The early clearance of jaundice rate at 6 months was 50 % (8/16) after laparoscopic operation and was 75 % (12/16) after open operation ( p = 0.144). Two years after the operation, the native liver survival was 50 % (8/16) in the laparoscopic group and was 81 % (13/16) in the open group ( p = 0.076). Five years after the operation, the native liver survival rate was 50 % (8/16) in the laparoscopic group and was 81 % (13/16) in the open group ( p = 0.076). The jaundice-free native liver survival rate at 5 years was 50 % (8/16) in laparoscopic group and was 75 % (12/16) in the open group. In the laparoscopic group, all patients with early clearance of jaundice survived and remained jaundice freed 5 years after the operation. Conclusion: The 5-year native liver survival rate after laparoscopic portoenterostomy was 50 %. Apparently superior result was observed in the open group (81 %) although the figures did not reach statistical difference because of the small sample size. A larger scale study is required to draw a more meaningful conclusion. [ABSTRACT FROM AUTHOR]

Purpose: This study was designed to evaluate the validity of the pediatric end-stage liver disease (PELD) score as a prognostic index of native liver survival in biliary atresia before Kasai portoenterostomy. Methods: Medical records of 63 patients who underwent Kasai portoenterostomy at our hospital were reviewed retrospectively. Rate of jaundice clearance and cumulative survival with native liver were calculated. Patients who were alive and did not require transplantation until the last follow up were included in the native-liver survival group. And patients who underwent any event, defined as death without transplantation, transplantation or listing for transplantation, were included in the native-liver failure group. Cox analysis was done. Results: 10-year survival rate with native liver was 41.9%. PELD scores of ≥15 and an age at operation of ≥60 days were found to be significant risk factors of failure( P = 0.007, OR = 2.851 and P = 0.010, OR = 3.222, respectively). Furthermore, patients with both risk factors showed significantly higher risk of failure ( P = 0.001, OR = 8.809). Conclusion: Since failure rate is higher for patients with high PELD score and older age, these factors should be helpful in estimating the prognosis. And with high-risk patients, cautious monitoring and consultation should be made whether the liver fails and requires transplantation. [ABSTRACT FROM AUTHOR]

Introduction Infants with biliary atresia (BA) develop a degree of hepatic fibrosis as a consequence of their cholangiopathy. Some of them present clinically evident ascites thatmight predispose to inguinal hernias.We aimed to investigate whether infants with BA have a higher incidence of inguinal hernias. Methods Single-center retrospective review of all BA infants diagnosed between January 2006 and December 2010. Infants with a clinical diagnosis of inguinal hernia were identified and compared with those without. Data were expressed as median (range) and compared with nonparametric statistical tests. p _ 0.05 was regarded as significant. Results A total of 123 infants underwent Kasai portoenterostomy (KP) during the period. Of these, 10 (8.1%) infants (7 boys) developed inguinal hernias (bilateral n = 4, right n = 5, left n = 1); 9 were repaired (at KP [n = 3] and post-KP [n = 6] at 15 [7 to 30] days) using nonabsorbable sutures, and 1 died before repair. There was no difference in median age at KP (66 vs. 58 days, p = 0.31); cytomegalovirus (IgM positive) status (p = 1.0); use of postoperative corticosteroids (p = 0.49); or ultimate need for liver transplant (p = 1.0). However, aspartate aminotransferase-to-platelet ratio (surrogate marker of liver fibrosis) was higher in hernia infants (2.0 vs. 1.0; p = 0.02). Recurrence has not been identified at a follow-up of 27 months (4 to 55). Conclusions This is the first report to suggest that BA infants have a high incidence of inguinal hernias, which seems related to degree of liver fibrosis at presentation and presumably degree of ascites and increased intra-abdominal pressure. [ABSTRACT FROM AUTHOR]

Background: The portoenterostomy (PE) procedure for treating biliary atresia (BA) has been so repeatedly modified that it currently hardly resembles Kasai's original PE (KOPE). Now PE involves an extended lateral dissection and a wide anastomosis (extended PE: EPE). We reappraised KOPE and created our-KOPE (OKOPE) by adhering strictly to its principles and techniques. We compared outcome of EPE and OKOPE. Methods: We reviewed 24 consecutive cases of PE for BA performed at our institution from 2005 to 2011. Thirteen had EPE, and 11 had OKOPE. Body weight, serum total bilirubin, age at PE, total steroid dosage required for jaundice clearance (JC: total bilirubin ≤1.2 mg/dL), JC ratio, time taken for JC, survival rate with the native liver (SNL), and SNL after JC (SNL + JC) were compared at 16 months (shortest mean follow-up). Postoperative management protocols were identical for both groups. Results: The JC ratio was significantly higher for OKOPE (90.9%) than EPE (46.2%) ( p = 0.02). Both SNL and SNL + JC were significantly higher for OKOPE (90.9 and 72.7%) than EPE (30.8 and 30.8%) ( p = 0.003 and p = 0.04, respectively). All other variables were similar for both groups. Conclusions: Jaundice clearance following OKOPE would appear to be better than after EPE. [ABSTRACT FROM AUTHOR]

Background and aims. Effects of caseload and organization of care on outcomes of biliary atresia (BA) remain unclear. We compared outcomes before and after national centralization of BA treatment in Finland with a population of 5.4 million people and 60,000 live births/year. Methods. All children born in Finland from 1987 to 2010 with BA were included. Complete patient identification was ascertained from the national Register of Congenital Malformations. Hospital records were reviewed for confirmation of the diagnosis, treatment, and follow-up data. Clearance of jaundice (serum bilirubin ≤20 μmol/l) and survival modalities were compared before and after centralization from five centers to Helsinki. Results. The incidence of BA was 1 in 20,100 live births. A total of 72 BA patients of whom 64 had undergone surgery for BA were identified. After centralization, the median caseload per center increased from 0 (range, 0-3) to 4 (2-5) patients/year ( p < 0.001), clearance of jaundice rate increased from 27% to 75% ( p == 0.001), 2-year jaundice-free native liver survival from 25% to 75% ( p == 0.002), transplant-free survival from 27% to 75% ( p == 0.005), and overall survival from 64% to 92% ( p == 0.082). Baseline patient characteristics including type of BA and age at portoenterostomy remained unaltered. In a logistic regression analysis including treatment era, operating center, BA splenic malformation syndrome, and age at portoenterostomy as variables, only treatment in Helsinki after centralization predicted clearance of jaundice (odds ratio 4.2; 95% confidence interval 1.05-16.5; p == 0.043). Conclusions. In small countries, BA treatment should be centralized to appointed multidisciplinary teams allowing high quality results with a median of four cases/year. [ABSTRACT FROM AUTHOR]

Biliary diseases in children are infrequent; however, they can be associated with high morbidity and mortality if an accurate diagnosis is not made and adequate treatment provided in a timely fashion. Biliary atresia, choledochal cysts, gallbladder disease, and Alagille syndrome can be associated with similar clinical symptoms, laboratory findings, and radiographic findings, which makes accurate diagnosis difficult. The correct treatment for each of these clinical entities is different and can significantly reduce morbidity and mortality from these diseases. In this article, we discuss the epidemiology, approach to diagnosis, prognosis, and treatment modalities for these four disease processes. [ABSTRACT FROM AUTHOR]