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Background: Robotic-assisted Kasai portoenterostomy (RAKPE) has been used to treat biliary atresia (BA). This study aimed to compare the efficacy of RAKPE and open Kasai portoenterostomy (OKPE) for BA., Methods: Thirty-one children with type III BA who underwent surgical treatment in two centers from January 2022 to December 2023 were retrospectively collected. According to the operative techniques, the participants were divided into the RAKPE group (13 cases) and the OKPE group (18 cases). The operative time, jaundice clearance (JC) rate, and incidence of cholangitis were analyzed., Results: The operative time in the RAKPE group (204.3 ± 19.9 min) was significantly longer than that in the OKPE group (186.2 ± 22.2 min), P < 0.05. However, the blood loss (8.1 ± 2.5 ml) in the RAKPE group was significantly decreased compared with the OKPE group (13.6 ± 4.8 ml), and 15.4% patient need blood transfusion in RAKEP group was litter than that 55.6% in the OKPE group, P < 0.05. The time to oral feeding (2.8 ± 0.4 days vs. 4.3 ± 0.7 days) and the time to pass ICG-positive stools (3.6 ± 0.6 days vs. 4.7 ± 0.9 days) in the RAKPE group were significantly shorter than those in the OKPE group, P < 0.05. No significant differences were observed in the bile excretion rate, hospital stay time, and JC rate. The incidence of cholangitis in the RAKPE group was significantly lower than that in the OKPE group during short follow-up., Conclusion: RAKPE may be associated with lower intraoperative blood loss, decrease need for postoperative transfusions and decreased rate of postoperative cholangitis compared to OKPE., Level of Evidence: III., Competing Interests: Conflict of interest The authors declare that they have no competing interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

One of the fundamental innovations of pediatric surgery is hepatic portoenterostomy for biliary atresia, widely known as the Kasai procedure for its originator, Morio Kasai (1922-2008), of Sendai, Japan. It was the first effective operation for the "uncorrectable" form of biliary atresia, where death within months from biliary cirrhosis was certain. His radical premise was that microscopic ductules present in the fibrous tissue of the porta hepatis, when transected, drained enough bile to relieve cholestatic jaundice. His first operations from 1955 to 1961 had a jaundice-free survival rate of only 8 percent, but the cures were durable. By 1982-1986 survival reached 55 percent. Adoption of his procedure in the West came slowly. The original operation in 1955 was reported in the Japanese literature in 1959, but the first English language communication came in 1968. Surgeons disbelieved his results. Scientific chauvinism held that advances in pediatric surgery came from America and Europe; Japan had little to add. Surgeons visited Sendai to learn from Kasai, notably John Lilly and R. Peter Altman, then of Washington, DC, Jacques Valayer in Paris, and Edward Howard in London. At first, mortality rates in their hands was high. As expertise developed in the West with the technically demanding operation, mortality fell and approached those reported by Kasai. Successes in America, France, and England proved Kasai's operation as effective and one of signal achievements in pediatric surgery., Competing Interests: Conflicts of interest The author declares no conflicts of interest in the preparation and submission of this article., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Aim: To evaluate the safety and outcomes of modified Kasai operation combined with autologous bone marrow mononuclear cell (BMMNC) infusion for biliary atresia (BA)., Methods: A matched control study was conducted between January 2015 and December 2021. Ten consecutive children with biliary atresia (BA) who underwent the modified Kasai operation combined with autologous BMMNC infusion (cell therapy group) and ten children who had only the modified Kasai operation (control group) were included in the study. The Kasai operation was performed with two modifications: partial exteriorization of the liver, and encirclement with lateral retraction of two hepatic pedicles to facilitate the removal of fibrotic tissue. Bone marrow was harvested through anterior iliac crest under general anesthesia then a modified Kasai operation was performed. After processing, bone marrow mononuclear cells were infused through the umbilical vein at the end of the operation. Serum bilirubin, albumin, alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, and prothrombin time were monitored at baseline, six months, twelve months, and the last follow-up (4.5 years) after the operation. In addition, esophagoscopy and liver biopsies were performed on patients whose parents agreed. Mixed-effects analysis was used to evaluate the changes in Pediatric End-Stage Liver Disease (PELD) scores., Results: There were no intraoperative or postoperative complications related to the operation or cell infusion. The average infused BMMNC and CD34 + cell counts per kg bodyweight were 85.5 ± 56.0 × 10 6 /kg and 10.0 ± 3.6 × 10 6 for the injection, respectively. Following the intervention, all ten patients in the cell therapy group survived, with a mean follow-up duration of 4.5 ± 0.9 years. Meanwhile, three patients in the control group died due to end-stage liver failure, with a mean follow-up time of 4.3 ± 0.9 years. Liver function of the cell therapy group was maintained or improved after the operation and cell infusion, as assessed by biochemical tests. The disease severity reduced markedly in the CT group compared to the control group, with a significant reduction in PELD scores (p < 0.05)., Conclusion: Autologous BMMNC administration combined with Kasai operation for BA is safe and may maintain or improve liver function in the studied patients., Trial Registration: ClinicalTrials.gov Identifier: NCT05517317 on August 26th, 2022., Competing Interests: Declarations. Ethics approval and consent to participate: Autologous BMMNC infusion for children with BA was approved by the Ministry of Health with approval number: 612/QD-BYT on February 21st, 2014. The Institutional Review Board of Vinmec International Hospital evaluated the ethical aspects of the study in accordance with the World Medical Association’s Declaration of Helsinki. The study was explained in detail to the parents of the participants. Consent to participate: This manuscript does not include personal information of participants. We confirm that informed consent to receive the cell therapy/interventions, containing explanation of benefits and risks, was obtained from the parents of all patients. Autologous BMMNC infusion for children with BA was approved by the Ministry of Health (Approval number: 612/QD-BYT). Participants in this study did pay for treatment. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

Purpose: Bile lake (BL) formation following Kasai portoenterostomy (KPE) can complicate the prognosis of biliary atresia (BA). Percutaneous transhepatic biliary drainage (PTBD) performed under fluoroscopic/ultrasonographic (US) guidance is discussed for the management of BL., Methods: A retrospective review of 64 BA patients treated by KPE (open = 31, laparoscopic = 33) at a single center (2004-2023) identified 9 BL cases (9/64; 14.1%). PTBD was indicated for jaundice or cholangitis refractory to antibiotic therapy., Results: All BL were asymptomatic, diagnosed after an episode of postoperative cholangitis. KPE type was not correlated with BL incidence; 2/31 (6.5%) for open and 7/33 (21.2%) for laparoscopic; p = 0.15. Median onset was postoperative day 273 (IQR: 170-920). One case resolved with antibiotics while another case required early liver transplantation (LTx) due to advanced hepatic dysfunction unrelated to BL. All remaining cases (7/9) had PTBD at a median of 14.3 months (IQR: 7.3-34.7) post-KPE, with successful resolution in 6/7; one case required two PTBD procedures. Post-PTBD biliary peritonitis (n = 3) was resolved by abdominal lavage (laparoscopic = 2; open = 1)., Conclusion: Screening for BL is advisable in all postoperative BA patients especially when cholangitis occurs. Although the risk for biliary peritonitis warrants caution, PTBD seems a viable option for managing BL formation after KPE., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: We investigate the histopathology of the portal vein branches and lymphatic vessels to elucidate the mechanism of atrophy of the left lateral segment (LLS) of the liver in biliary atresia (BA)., Methods: LLS and right anterior segment (RAS) liver biopsy samples obtained during Kasai portoenterostomy (KPE) from ten consecutive patients with BA underwent histopathological investigation of the portal vein and lymphatic vessels using double chromogenic immunostaining for CD31/D2-40 and the hepatitis-like findings (HLF) score. Each parameter and clinical data were compared between prognostic groups., Results: HLF scores in the LLS were always higher than those in the RAS. There was no difference in portal vein and lymphatic vascular morphology, whereas the number of lymphatic vessels was correlated with the fibrotic area of all specimen areas. Left-to-right ratio of the number of lymphatic vessels was correlated with the age at KPE (r = 0.784, p = 0.007) and the pre-KPE CRP value (r = 0.723, p = 0.018)., Conclusions: Lymphangiogenesis on the LLS compared to the RAS was significantly correlated with the degree of fibrosis and the age at KPE. Further investigation is warranted to clarify the causes of LLS atrophy and lymphangiogenesis relevant to immune dysregulation., (© 2024. The Author(s).)

Although biliary atresia (BA) is a rare neonatal disorder, it remains the leading cause of pediatric end-stage liver disease. Early diagnosis of BA and treatment with the Kasai procedure can significantly reduce the need for pediatric liver transplant. Current data suggests that performing the Kasai procedure at 30-45 days of life is associated with longer native liver survival rates and reduction of the need for liver transplant. The incidence rate of BA in the state of Hawai'i is nearly double the incidence rate in the continental US. International studies have demonstrated that screening programs for BA reduce the age at diagnosis and treatment. However, there has been no statewide analysis on the ages at diagnosis or at Kasai, nor does a statewide screening program for BA exist. The purpose of this study is to review the age of diagnosis and treatment of BA to determine if the current practice in Hawai'i is in line with the published data. A retrospective chart review of all patients diagnosed with BA at the state's primary children's hospital was performed (2009-2023) and 19 patients who underwent the Kasai procedure were identified. The mean age at diagnosis is 71.4 days (n=19) and the mean age at Kasai procedure is 72.0 days (n=19). Both the average age at diagnosis and treatment for BA in Hawai'i is significantly higher than published data suggesting best outcomes at 30-45 days of life. This review suggests that the implementation of a statewide screening program for BA in Hawai'i is warranted., Competing Interests: None of the authors identify a conflict of interest., (©Copyright 2024 by University Health Partners of Hawai‘i (UHP Hawai‘i).)

Background: To predict native liver survival (NLS) after Kasai portoenterostomy (KP) for biliary atresia (BA) using pre-operative clinical data., Materials and Methods: Pre-operative data were collected from 29 patients with BA who underwent KP at our department between 1989 and 2017 and were analysed including serum albumin, bilirubin, prothrombin time-international normalised ratio, body height, body weight, age at KP, paediatric end-stage liver disease score calculated using the pre-operative data and the period of NLS., Results: The 10-year NLS rate of all patients was 51%. A multivariate analysis revealed that among all factors, the pre-KP serum albumin level was the only independent predictor of NLS ( P = 0.04, hazard ratio = 0.269, 95% confidence interval = 0.077-0.934). The area under the receiver operating characteristic curve for NLS, determined using pre-KP serum albumin was 0.760 and 3.75 mg/dl was selected as the cut-off value. There was a significant difference in NLS between patients with high (≥3.8 mg/dl) and low (≤3.7 mg/dl) pre-KP serum albumin (90.0% vs. 31.5%, P < 0.01)., Conclusions: Decreased pre-KP serum albumin may reflect not only functional impairment of the liver, but also the inflammatory process, which is hypothesized to occur during its advancement. The pre-KP serum albumin level may be a good prognostic factor for NLS in post-KP BA patients., (Copyright © 2024 Copyright: © 2024 African Journal of Paediatric Surgery.)

Background: In patients with biliary atresia (BA), severe portal hypertension (HTN) develops even with successful bile flow restoration, suggesting an intrinsic factor driving portal HTN independent from bile obstruction. We hypothesize that patients with BA have abnormal portal vein (PV) development, leading to PV hypoplasia., Methods: In this observational cohort study, we enrolled patients who were referred to a tertiary center from 2017 to 2021 to rule out BA. Newborns who underwent computed tomography angiogram as a clinical routine before intraoperative cholangiogram, and laparoscopic Kasai hepatoportoenterostomy. The diameter of the PV and hepatic artery (HA) were compared to the degree of liver fibrosis in the wedge biopsies. The jaundice clearance, native liver survival, and clinical portal hypertensive events, including ascites development and intestinal bleeding, were assessed., Results: 47 newborns with cholestasis were included in the cohort; 35 were diagnosed with BA. The patients with BA had a smaller median PV diameter (4.3 vs. 5.1 mm; p < 0.001) and larger median HA diameter (1.4 vs. 1.2 mm; p < 0.05) compared to the patients with other forms of cholestasis. The median PV and HA diameter did not correlate with the degree of liver fibrosis. Among 35 patients with BA, 29 patients (82.9%) achieved jaundice clearance, and 23 patients (65.7%) were alive with their native liver at two years of age. Seven patients (20%) developed intestinal bleeding, and seven patients (20%) developed ascites, with one overlapping patient., Conclusion: PV hypoplasia is present in patients with BA independent of liver fibrosis at the time of diagnosis., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Biliary atresia is a progressive cholangiopathy in neonates, which often results in liver failure. In high-income countries, initial treatment requires prompt diagnosis followed by Kasai portoenterostomy. For those with a late diagnosis, or those in whom Kasai portoenterostomy fails, liver transplantation is the only lifesaving treatment. Unfortunately, in low- and middle-income countries, timely diagnosis is a challenge and liver transplantation is rarely accessible. Here, we discuss the ethical dilemmas surrounding treatment of babies with biliary atresia in Uganda. Issues that require careful consideration include: risk of catastrophic health expenditure to families, ethical dilemmas of transplant tourism, medical risks of maintaining the transplant in a low-resourced health system, and difficult decisions encountered by the surgeon caring for these patients. Four distinct models of the patient-physician relationship are applied to biliary atresia in Uganda. These models describe differences in patient and physician roles, and patient values and autonomy. Solid organ transplantation is a rapidly evolving segment of healthcare in Uganda and ongoing policy advancements may shift ethical considerations in the future., (© 2024 International Society of Surgery/Société Internationale de Chirurgie (ISS/SIC).)

Demographic characteristics and clinical data of all newly diagnosed biliary atresia patients in Shanghai were collected from 1 January 2015 to 31 October 2016. The total number of live births was 377 420 during the study period, and the incidence of biliary atresia in Shanghai was 10.86 per 100 000 (95% CI 7.8 to 17.74), with 62.9% and 45.7% cases retaining native liver survival at 2 and 5 years after Kasai procedure, respectively. Implementation of systematic screening measures for biliary atresia in China is needed., Competing Interests: Competing interests: None., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Prenatally diagnosed hepatic hilar cysts are a challenging finding for the clinician. They can either be a sign of cystic biliary atresia (BA) or a choledochal cyst (CC), two diagnoses with different postnatal management and prognosis. Based on a case report of four patients, we aim to propose a management algorithm for prenatally diagnosed "hepatic hilar cysts"., Case Presentation: A hepatic hilar cyst, ranging from 5 to 25 mm, was detected prenatally in all four girls confirmed postnatally along with the presence of a gallbladder. Stool color was normal until two weeks of life at which time the stool color became lighter, and the patients developed cholestasis. All were operated before seven weeks of life: Case 1 had a CC with patent but irregular intrahepatic bile ducts at intraoperative cholangiogram, and no communication with the duodenum. A Roux-en-Y bilioenteric anastomosis was performed. The cyst showed complete epithelial lining loss, and liver pathology showed BA features. Case 2 had the final diagnosis of cystic BA with patent but abnormal intrahepatic bile ducts. She underwent two operations: the first operation at four weeks as described for case 1, since intraoperative findings were similar, as was histology. As cholestasis increased postoperatively, she underwent a Kasai hepato-porto-enterostomy six weeks later, where distinct BA findings were found with complete scarring of the hilar plate. Case 3 had a cystic BA with the cyst located within the common bile duct and atretic bile ducts proximal to the porta hepatis. It exhibited no communication with the liver or duodenum. A Kasai operation was performed, with histology showing complete epithelial loss within the cyst wall and scarring of the hilar plate. Case 4 had a cystic BA presenting a completely obliterated hepatic duct with the cyst lying within the common bile duct. A Kasai procedure was performed. Histology showed a common bile duct with a residual lumen of 0.1 mm., Conclusions: The spectrum of disease from CC to BA in the setting of a prenatally discovered hepatic hilar cyst is emphasized. Even if cholangiogram differentiates most patients with BA from those with CC, caution is advised for transitional types., (© 2024. The Author(s).)

Introduction: Biliary atresia is a rare liver disease of unknown etiology affecting approximately 1 in 10,000 children. This disease initially presents as inflammatory obstruction of bile ducts leading to cholestasis and eventually fibrosis of hepatic tissue. Affected patients are ideally treated early with portoenterostomy (Kasai procedure) as age at surgery is an important prognostic factor for native liver survival and need for liver transplant. This study aimed to evaluate the age at which patients in the United States are receiving this procedure., Methods: The American College of Surgeons National Surgical Quality Improvement Program Pediatric database was used to identify patients between 2012 and 2021 who underwent a primary procedure of portoenterostomy. The age at time of surgery and perioperative analysis was performed. The data underwent simple descriptive statistics., Results: Eight hundred twenty four patients were identified who underwent Kasai procedure. Four hundred seventy four (58.2%) were female with the predominant race being White (49.5%). The median age at surgery was 57 d old (interquartile range 41-71). Readmission and reoperation rates within 30 d were 30% and 15.2%, respectively. There were no deaths within 30 d., Conclusions: Within the National Surgical Quality Improvement Program database, the median age of pediatric patients undergoing Kasai procedure for biliary atresia in the United States exceeds the goal of 45 d. Further studies are needed to investigate factors that may affect time to diagnosis and time to Kasai procedure., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Biliary atresia (BA) is a congenital disease that occurs when extrahepatic bile ducts are either absent or deficient, resulting in liver fibrosis, portal hypertension, and eventually cirrhosis. It is the most common cause of persistent obstructive jaundice in newborns lasting more than two weeks is this condition. Abdominal ultrasound (US) is the primary imaging technique used to diagnose BA, while computed tomography (CT) is reserved for more complex cases. The gold standard for diagnosing BA is still intraoperative cholangiogram with liver biopsy. Treatment for BA usually involves Kasai hepatoportoenterostomy, but some patients still require liver transplantation due to diagnostic delays and advanced disease. In this study, the authors present the case of a 3-month-old infant with biliary atresia and its ultrasound characteristics, who underwent liver transplantation due to advanced disease. The primary objective of imaging is to provide a prompt diagnosis, given the crucial significance of timely surgical intervention., (© 2024. Società Italiana di Ultrasonologia in Medicina e Biologia (SIUMB).)

Background: Newborn screening for biliary atresia (BA) may facilitate earlier diagnosis and intervention for improved clinical outcomes., Methods: We systematically reviewed the accuracy of population-based screening strategies for BA in the newborn using PRISMA-DTA guidelines. We included cohort or cross-sectional studies. The screening (index) tests included stool color card (SCC) and direct/conjugated bilirubin (DB/CB) and the reference standard was intraoperative cholangiogram. Meta-analysis was performed using random-effects logistic regression models., Results: We included 15 studies (1,816,722 participants) that assessed 5 different population-based screening strategies. QUADAS-2 assessment revealed high risk of bias for patient selection in one study and uncertain risks for reference standard in multiple studies. High certainty evidence suggests that DB/CB assessed after birth had a summary sensitivity of 100% (95% CI 100,100) and specificity of 98.8% (98.8,98.9) (5 studies, 662141 participants). Moderate certainty evidence suggests that SCC screening at a month of age had summary sensitivity of 79.6% (95% CI 70.6, 86.4) and specificity of 99.9% (95% CI 99.9, 99.9) (7 studies, 996262 participants)., Conclusions: DB/CB in the first few days of life has the best diagnostic accuracy for population screening for biliary atresia in the newborn. Future research should focus on cost-effectiveness and combinations of screening strategies., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Gopal et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Purpose: Early diagnosis of biliary atresia (BA) is critical for best outcomes, but is challenged by overlapping clinical manifestations with other causes of obstructive jaundice in neonates. We evaluate the performance of the modified Simple BA Scoring System (SBASS) in diagnosing BA., Methods: We performed a prospective, cross-sectional study on infants with cholestatic jaundice (June 2021-December 2022). Modified SBASS scoring was applied and compared to the eventual diagnosis (as per intraoperative cholangiogram (IOC) and liver histopathology). The score (0-6), consists of gall bladder length < 1.6 cm (+ 1), presence of triangular cord sign (+ 1), conjugated bilirubin:total bilirubin ratio > 0.7(+ 2), gamma-glutamyl transferase (GGT) ≥ 200 U/L (+ 2)., Results: 73 were included: Fifty-two (71%) had BA. In the non-BA group, 6 (28%) had percutaneous cholangiography (PTC) while 15 (72%) had intraoperative cholangiogram (IOC). At a cut-off of 3, the modified SBASS showed sensitivity of 96.2%, specificity of 61.9% and overall accuracy of 86.3% in diagnosing BA. Area under receiver operating characteristic curve was 0.901. GGT had the highest sensitivity (94.2%), while triangular cord sign showed the highest specificity at 95.2%., Conclusion: The SBASS provides a bedside, non-invasive scoring system for exclusion of BA in infantile cholestatic jaundice and reduces the likelihood of negative surgical explorations., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Biliary atresia (BA) poses a persistent challenge characterized by ongoing liver inflammation and subsequent fibrosis even after the clearance of jaundice (COJ). This study aimed to evaluate the therapeutic potential of eicosapentaenoic acid (EPA) in alleviating liver inflammation and limiting fibrosis during the post-COJ phase of BA., Methods: Among the BA patients undergoing laparoscopic Kasai portoenterostomy (lapKP) between December 2016 and October 2021, EPA (20-40 mg/kg/day) was administered orally to those whose parents consented. The study included patients from January 2014 to October 2021, classifying them into two groups: EPA-treated (Group E) and untreated (Group N). Their liver fibrosis and clinical course at 1 and 2 years post-lapKP were compared., Results: Group E consisted of 25 patients, while Group N comprised 32 patients. Twenty-one patients in Group E and 25 patients in Group N achieved COJ (p = 0.74). Among jaundice-free patients at 1 and 2 years post-lapKP, Group E exhibited significantly lower M2BPGi levels and platelet counts, and Group E showed a significant reduction in Aminotransferase-to-Platelet Ratio Index (APRI) at 2 years post-lapKP., Conclusion: Although EPA administration did not improve COJ, it attenuated the progression of liver fibrosis during the 2 years following lapKP in jaundice-free patients. (200/200Words)., (© 2024. The Author(s).)

Purpose: We aimed to investigate whether prediction of liver fibrosis using two-dimensional shear wave elastography (2D-SWE) and vascular tree grading using superb microvascular imaging (SMI) are useful for postoperative follow-up in patients with biliary atresia (BA)., Methods: We retrospectively collected data from medical records of 134 patients who underwent ultrasound examination with 2D-SWE or SMI, including 13 postoperative patients with BA and 121 non-BA patients. We investigated the distribution of liver stiffness values with SWE and vascular tree grading with SMI and evaluated correlations between these findings and biochemical indices of liver fibrosis in postoperative BA patients., Results: The SWE values of the BA group were not significantly different from that of any other disease groups in non-BA patients. In postoperative BA patients, SWE values correlated significantly with aspartate aminotransferase to platelet ratio index (Spearman rank correlation coefficient [r s ] = 0.6380, p = 0.0256) and with the Fib-4 index (r s  = 0.6526, p = 0.0214). SMI vascular tree grading of the BA group was significantly higher than that of the choledochal cyst group (p = 0.0008) and other hepatobiliary disorder group (p = 0.0030). In postoperative BA patients, SMI vascular tree grading was not positively correlated with any biochemical marker of fibrosis., Conclusion: 2D-SWE appears to be useful for follow-up in postoperative BA patients., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: We aimed to identify factors predicting the need for future liver transplantation (LT) at 18 years of age in patients with biliary atresia (BA)., Methods: BA patients with native liver survival at > 18 years of age were retrospectively reviewed. The clinical characteristics, outcomes, hepatobiliary function, and liver fibrosis markers of native liver survivors (NLS group) were compared with patients who subsequently underwent LT (LT group)., Results: The study population included 48 patients (NLS, n = 34; LT, n = 14). The male-to-female ratio, age at Kasai procedure, and type of BA in the two groups did not differ to a statistically significant extent. There was no significant difference in the MELD scores between the groups at 18 years of age. The aspartate aminotransferase-to-platelet ratio index (APRI), albumin-bilirubin (ALBI), and BA liver fibrosis (BALF) scores at 18 years of age were significantly higher in the LT group. The AUCs for APRI, ALBI, and BALF were 0.91, 0.79, and 0.85, respectively., Conclusion: Adult BA patients have limited options for LT owing to the lack of donor candidates and the low prevalence of deceased donors. The elucidation of prognostic factors for LT in adulthood is important. APRI was the most useful marker in this study., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Biliary atresia (BA) is the leading cause of liver cirrhosis and chronic liver insufficiency in children in the world. Gastroesophageal varices bleeding is an ominous complication of cirrhosis in BA patients and is associated with high morbidity and mortality. In this study, we aimed to investigate the utility of noninvasive Baveno VI and Baveno VII criteria for the screening of varices need treatment (VNT) and the need for liver transplantation in BA patients., Methods: This study enrolled 48 BA patients (23 females and 25 males) who underwent an esophagogastroduodenoscopy (EGD) and transient elastography at a mean age of 11.18 ± 1.48 years; the clinical data were surveyed in a retrospective design., Results: The sensitivity and negative predictive value of Baveno VI and Baveno VII criteria for the prediction of VNT in BA patients are both 100% and 100%, respectively. The VNT missing rate of Baveno VI and Baveno VII criteria are both 0% in our cohort. The Baveno VI, expanded Baveno VI, and Baveno VII criteria are also predictive of the need for liver transplantation in our cohort (OR = 10.33, 4.24, and 21.33; p = 0.009, 0.03, and 0.007, respectively)., Conclusion: The Baveno VI and Baveno VII criteria are useful for the screening of VNT and minimize non-necessary invasive EGD in BA patients with low VNT missing rates. The Baveno VI, expanded Baveno VI, and Baveno VII criteria are associated with the need for liver transplantation., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

The role of liver transplantation as a primary procedure in biliary atresia has been argued over for at least 40 years, indeed since the coming of age of safe liver transplantation during the 1980s. Yet, it is not a common option in most series (usually ≤5%) and typically reserved for those with late presentations (arguably >100 days) with established cirrhosis. This review presents the pros and cons of primary liver transplant. The pros are based upon the observation that at best a Kasai portoenterostomy (KPE) is simply palliative in most, and at worse has no effect whatsoever on restoration of bile flow and is therefore pointless. Set against this are the cons: there is a dearth of prognostic tests (clinical, biochemical, or histological) at the time of presentation which may predict inevitable failure; the possibility of long-term native liver survival to adulthood in a proportion (albeit a minority); and the implied increased need for donor organs suitable for infants - a stressor for an already overstressed system. Improving results from KPE in terms of increasing the proportions clearing their jaundice and minimizing the effects of chronic liver fibrosis and cirrhosis would surely limit the siren calls for primary transplants but the key must be better discrimination at presentation with the use of biomarkers (circulatory or histological, individually or together) to enable better decision making., Competing Interests: Conflict of interest None to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: The prognostic factors of subsequent liver transplantation (LT) in patients with biliary atresia (BA) who presented with jaundice-free native liver survival were investigated., Methods: This study retrospectively reviewed patients who underwent portoenterostomy (PE) for BA. Patients with jaundice-free native liver survival at 1 year postoperatively were divided into the autologous liver survivor and liver transplant recipient groups. Peri- and postoperative data were compared between the two groups., Results: Among 97 patients with BA, 29 who received LT within 1 year after PE were excluded from the analysis. Further, 48 patients currently living with native liver and 20 who received LT after 1 year postoperatively were compared. Bile lake (BL) was the strongest risk factor of LT. The risk score was 2.38 ∗ B L s c o r e + 0.00466 ∗ T B A , and the area under the receiver operating characteristic curve was 0.83. Patients with BL and those without significantly differed in terms of the native liver survival rate. Patients with BL who presented with not only cholangitis but also gastrointestinal hemorrhage and hepatopulmonary syndrome received LT., Conclusion: BL can cause different pathologies. Moreover, it is an evident risk factor of subsequent LT in patients with BA who are living with native liver at 1 year after PE., (© 2024. The Author(s).)

Purpose: This study evaluated portal hypertension (PHT) and its predictors among native liver survivors (NLS) of biliary atresia (BA) after Kasai portoenterostomy (KPE)., Methods: This was a multicenter study using prospectively collected data. The subjects were patients who remained transplant-free for 5 years after KPE. Their status of PHT was evaluated and variables that predicted PHT were determined by regression analysis and receiver operating characteristic (ROC) curve., Results: Six centers from East Asia participated in this study and 320 subjects with KPE between 1980 to 2018 were analyzed. The mean follow-up period was 10.6 ± 6.2 years. At the 5th year after KPE, PHT was found in 37.8% of the subjects (n = 121). Patients with KPE done before day 41 of life had the lowest percentage of PHT compared to operation at older age. At 12 months after KPE, PHT + ve subjects had a higher bilirubin level (27.1 ± 11.7 vs 12.3 ± 7.9 µmol/L, p = 0.000) and persistent jaundice conferred a higher risk for PHT (OR = 12.9 [9.2-15.4], p = 0.000). ROC analysis demonstrated that a bilirubin level above 38 µmol/L at 12 months after KPE predicted PHT development (sensitivity: 78%, specificity: 60%, AUROC: 0.75)., Conclusions: In BA, early KPE protects against the development of PHT among NLSs. Patients with persistent cholestasis at one year after KPE are at a higher risk of this complication. They should receive a more vigilant follow-up., Level of Evidence: Level III., (© 2024. The Author(s).)

Purpose: Frequent post-operative cholangitis in biliary atresia (BA) affects the long-term native liver survival. This study assessed the characteristics of early cholangitis and their influence on the prognosis., Methods: Forty-three patients with BA who underwent surgery between 2000 and 2020 were analyzed for routine inflammatory markers. Early cholangitis characteristics were compared between native liver survivor (NLS) and living donor liver transplant (LDLT) patients., Results: Among the 43 patients, 30 (69.8%) experienced 130 episodes of cholangitis. In the area under the receiver operating characteristics curve (AUROC) analysis, the cutoff value of the total cholangitis episodes was 3, with an area under the AUROC curve of 0.695 (95% confidence interval 0.522-0.868). Before 3 years old, 113 episodes (86.9%) of cholangitis were observed. The white blood cell, C-reactive protein, and alanine aminotransferase values at cholangitis onset did not markedly differ between the LDLT and NLS groups. Conversely, the neutrophil-to-lymphocyte ratio in the NLS group was significantly lower than in the LDLT group (0.85 vs. 1.63, p < 0.001)., Conclusions: Cholangitis in the NLS group was lymphocyte-dominant and atypical in its pathogenesis. Lymphocyte-dominant cholangitis is non-suppurative, and future research should clarify its pathogenesis to improve the treatment and prognosis of BA., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Hereditary angioedema is a potentially life-threatening autosomal dominant condition, causing attacks of angioedema due to failure to regulate bradykinin. Nearly all cases of hereditary angioedema are caused by mutations in the gene encoding C1 inhibitor, SERPING1 . C1 inhibitor is a multifunctional protein produced in the liver that regulates the kallikrein-kinin system at multiple points. An infant with genetically confirmed hereditary angioedema and low C1 inhibitor levels (but without previous episodes of angioedema) underwent liver transplantation for biliary atresia, an unrelated condition. Liver transplantation led to normalization of the C1 inhibitor level and function. To our knowledge, this represents the first patient to be potentially cured of hereditary angioedema., (Copyright © 2024 Massachusetts Medical Society.)

Background: Biliary atresia is the most common cause of obstructive jaundice in infants and conventional cholangiography is the current diagnostic gold standard. Fluorescent cholangiography with indocyanine green can enhance biliary tree visualization during surgery because it is exclusively excreted into the bile ducts and eventually into the intestine. Therefore, we hypothesized that indocyanine green presence in stool could confirm bile duct patency in infants., Methods: A prospective single center cohort study was performed on infants (age ≤ 12 months) with and without jaundice after obtaining IRB approval. Indocyanine green was administered intravenously (0.1 mg/kg). Soiled diapers collected post-injection were imaged for fluorescence., Results: After indocyanine green administration, fluorescence was detected in soiled diapers for control patients (n = 4, x = 14 h22 m post-injection) and jaundiced patients without biliary atresia (n = 11, x = 13 h28 m post-injection). For biliary atresia patients (n = 7), post-injection soiled diapers before and after Kasai portoenterostomy were collected. Fluorescence was not detected in stool from 6 of 7 biliary atresia patients. As a test, indocyanine green detection in stool was 97% accurate for assessing biliary patency., Conclusion: Fluorescent Imaging for Indocyanine Green (FIInd Green) in stool is a fast and accurate approach to assess biliary patency non-invasively in infants., Level of Evidence: Level III., Competing Interests: Conflicts of interest The authors have no conflicts of interests to declare and no financial disclosures., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Systemic Immune-Inflammation Index (SII), known as an easy, economical and useful marker, correlates with the balance of inflammation and immune response. However, the usefulness of SII in biliary atresia (BA) remains unclear. Therefore, we evaluated the relationship of SII level and postoperative clinical outcomes of BA., Methods: Retrospective review of 168 patients with BA was conducted with assessments of demographic information, histological findings, laboratory parameters, and clinical outcomes. The LASSO logistic regression analysis was established using the "glmnet" software package to explore the influencing factors related to native liver survival time. Numerical variables were dichotomized based on the receiver operating characteristic (ROC) curve and Youden index yielding the best performance of prediction. R software was used for statistical analysis., Results: Overall, the 24 month native liver survival rate was 43.5% (73 cases) after Kasai portoenterostomy. LASSO logistic regression analysis show that preoperative malnutrition (OR = 0.032, 95%CI 0.001-0.424), gamma-glutamyltransferase (GGT, OR = 0.994, 95%CI 0.987-0.998), lymphocyte count (LY, OR = 2.426, 95%CI 1.467-4.604), SII (OR = 0.977, 95%CI 0.960-0.989), and liver fibrosis grading (LFG, reference: Grade 1, Grade 3, OR = 0.076, 95%CI 0.007-0.614) were the independent influencing factors for 24 month native liver survival. ROC curve analysis showed that the area under the curve of SII level (0.919) was larger than that of preoperative malnutrition (0.690), LFG (0.759), GGT (0.747), and Ly (0.773). A SII < 140.09 was found to be a significant marker in the prediction of 24-month native liver survival, with 90.41% sensitivity and 93.68% specificity. Furthermore, the rates of 24-month native liver survival (33.1% vs. 72.7%), jaundice clearance (46.8% vs. 75.0%), and good liver function recovery (46.8% vs. 65.9%) were lower in the SII ≥ 140.09 group than that in the SII < 140.09 group (all P < 0.05), but there was no difference in the occurrence of cholangitis (P > 0.05)., Conclusion: Preoperative malnutrition, GGT, Ly, SII, and LFG were independent influencing factors for postoperative 24-month native liver survival of BA. The SII level, as a routine haematological marker, has better universality and simplicity and is related to clinical outcomes after Kasai portoenterostomy., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: To identify infants with biliary atresia (BA), European Society of Paediatric Gastroenteroloy and Nutrition (ESPGHAN)/North American Society of Pediatric Gastroenteroloy and Nutrition (NASPGHAN) guidelines recommend measurement of conjugated/direct bilirubin in infants with prolonged jaundice and using a stool colour card (SCC). The 'Quality of Care' Task Force of ESPGHAN performed two surveys to assess current case finding for BA and age at Kasai portoenterostomy (KPE)., Methods: The first survey approached 26 European hepatology centres to report age at referral and age at KPE of all infants diagnosed with BA from 2015 to 2019. The second survey targeted paediatricians in France to assess awareness and compliance with the recently introduced SCC., Results: Data from 785 patients with BA from 18 centres in 15 countries revealed a mean age at referral to tertiary centre of 55 days (median 53, IQR 48-60) (n = 636). The mean age at KPE was 61 days (median 60; IQR 54-67) (n = 772). For 6% of patients, cirrhosis was too advanced for surgery. Of 392 paediatricians answering the second survey, 53% felt familiar with the target diseases, 80% correctly identified cholestasis and 59% always inquired about the infant's stool colour. If abnormal, 93% would order blood tests and 85% call for advice. The SCC screening was considered helpful for case finding and improving knowledge of cholestatic diseases by 62% and 45% paediatricians, respectively., Conclusions: Referral of infants for KPE remains late, indicating low adherence to search for cholestasis in icteric infants by age 2-3 weeks. Knowledge and structures need improvement to allow earlier guideline conform case finding, diagnosis and therapy., (© 2024 The Authors. Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: The survival rate of children with biliary atresia (BA) after liver transplantation (LT) is significantly improved, and their quality of life has attracted much attention.This study aimed to investigate the cognition and its influencing factors in children with BA after primary living donor LT (BA-pLDLT) during infancy., Methods: Children with BA were recruited 6 months after pLDLT at Children's Hospital of Chongqing Medical University (2018-2022). Demographic and clinical data were collected from the health information system. Cognition was assessed using the Chinese version of the Griffiths Mental Development scale (GMDS-C). Multivariate linear regression were used to analyze the influencing factors of their cognitive function., Results: In total, 57 children with BA-pLDLT, aged 5.00(3.90-9.30) months at transplantation and 25.00(14.00-60.80) months at evaluation were included. The general developmental quotient (89.02 ± 12.07) and motor, language, eye-hand coordination, performance, and practical reasoning quotients of these children were significantly lower than the normative mean values of GMDS-C(P < 0.05). Of the 57 children, 16 (28.07%) had borderline developmental delay (DQ between 70 and 84), 3 (5.26%) had developmental delay (DQ < 70), and 11(19.29%) had language delay. Reoperation for biliary or vascular complications after pLDLT was a risk factor for decreased general development quotient and motor quotient and lower Z W at assessment was associated with decline motor quotient., Conclusion: Children with BA-pLDLT have varying degrees of developmental delays in early life. Reoperation and nutritional deficiencies had adverse effects on cognitive development., (© 2024. The Author(s).)

For patients with Biliary atresia, antibiotic prophylaxis after Kasai portoenterostomy is a common practice. Societal guidelines often cite one reference as supportive evidence for this practice. In this paper, we go back to review the quality of this evidence and suggest more research is required to demonstrate the efficacy of antibiotic prophylaxis in this population., (© The Author(s) 2024. Published by Oxford University Press on behalf of The Journal of the Pediatric Infectious Diseases Society. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Introduction: Acute-on-chronic liver failure (ACLF) is associated with increased mortality and morbidity in patients with biliary atresia (BA). Data on impact of ACLF on postoperative outcomes, however, are sparse., Method: We performed a retrospective analysis of patients with BA aged <18 years who underwent LT between 2011 and 2021 at our institution. ACLF was defined using the pediatric ACLF criteria: ≥1 extra-hepatic organ failure in children with decompensated cirrhosis., Results: Of 107 patients (65% female; median age 14 [9-31] months) who received a LT, 13 (12%) had ACLF during the index admission prior to LT. Two (15%) had Grade 1; 4 (30%) had Grade 2; and 7 (55%) had Grade ≥3 ACLF. ACLF cohort was younger at time of listing (5 [4-8] vs. 9 [6-24] months; p < .001) and at LT (8 [8-11] vs. 16 [10-40] months, p < .001) compared to no-ACLF group. Intraoperatively, ACLF patients had higher blood loss (40 [20-53] vs. 10 [6-19] mL/kg; p < .001) and blood transfusion requirements (33 [21-69] vs. 18 [7-25] mL/kg; p = .004). Postoperatively, they needed higher vasopressor support (31% vs. 10.6%; p = .04) and had higher total hospital length of stay (106 [45-151] vs. 13 [7-30] days; p = .023). Rate of return to the operating room, hospital readmission rates, and 1-year post-LT survival rates were comparable between the groups., Conclusion: Despite higher perioperative complications, survival outcomes for ACLF in BA after LT are favorable and comparable to those without ACLF. These encouraging data reiterate prioritization during organ allocation of these critically ill children for LT., (© 2024 Wiley Periodicals LLC.)

Objectives: To identify and distinguish between racial and socioeconomic disparities in age at hepatology care, diagnosis, access to surgical therapy, and liver transplant-free survival in patients with biliary atresia (BA)., Methods: Single-center retrospective cohort study of 69 BA patients from 2010 to 2021. Patients were grouped into White and non-White cohorts. The socioeconomic milieu was analyzed utilizing neighborhood deprivation index, a census tract-based calculation of six socioeconomic variables. The primary outcomes of this study were timing of the first hepatology encounter, surgical treatment with hepatic portoenterostomy (HPE), and survival with native liver (SNL) at 2 years., Results: Patients were 55% male and 72% White. White patients were referred at a median of 34 days (interquartile range [IQR]: 17-65) vs. 67 days (IQR: 42-133; p = 0.001) in non-White patients. White infants were more likely to undergo HPE (42/50 patients; 84%) compared to non-White (10/19; 53%), odds ratio (OR) 4.73 (95% confidence interval: 1.46-15.31; p = 0.01). Independent of race, patients exposed to increased neighborhood-level deprivation were less likely to receive HPE (OR: 0.49, p = 0.04) and achieve SNL (OR: 0.54, p = 0.02)., Conclusions: Racial and socioeconomic disparities are independently associated with timely BA diagnosis, access to surgical treatment, and transplant-free survival. Public health approaches to improve screening for pathologic jaundice in infants of diverse racial backgrounds and to test and implement interventions for socioeconomically at-risk families are needed., (© 2024 The Authors. Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: Sir Roy Calne in 1976 described "Biliary reconstruction is the Achilles heel of liver transplantation," and it remains true. In some patients, such as those with short-gut syndrome and concomitant biliary atresia, neither duct to duct nor Roux biliary reconstruction is feasible., Methods: We present a case of child's third liver transplant (LT), where an innovative extra-anatomical biliary bypass was created using a sleeve from greater curvature of the stomach., Results: The patient is well nearly 10 years following the LT., Conclusions: This technique could prove to be an important addition to the armamentarium of a surgeon in difficult retransplants and in patients with short-gut syndrome as it provides a viable option with good long-term outcome., (© 2024 Wiley Periodicals LLC.)

Purpose: We compared the clinical features of patients with biliary atresia (BA) associated with a bleeding tendency (BT) at the time of the diagnosis with those of patients without a bleeding tendency (NBT)., Methods: The patients' background characteristics, age in days at the first visit, Kasai portoenterostomy (KPE), and postoperative course were retrospectively analyzed., Results: Nine of the 93 BA patients (9.7%) showed a BT, including 7 with intracranial hemorrhaging (ICH), 1 with gastrointestinal bleeding, and 1 with a prothrombin time (PT) of 0%. The age at the first visit was 62 ± 12 days old for BT patients and 53 ± 27 days old for NBT patients (p = 0.4); the age at KPE was 77 ± 9 days old for BT patients and 65 ± 24 days old for NBT patients (p = 0.2); the time from the first visit to surgery was 13 ± 7 days for BT patients and 11 ± 10 days for NBT patients (p = 0.5); and the native liver survival rate was 56% for BT patients and 58% for NBT patients (p = 1), with no significant difference in any of the parameters. The neurological outcomes of survivors of ICH were favorable., Conclusions: Appropriate BT correction allowed early KPE even after ICH, resulting in native liver survival rates comparable to those of NBT patients without significant neurological complications., (© 2023. The Author(s) under exclusive licence to Springer Nature Singapore Pte Ltd.)

Background and Study Aims: Biliary atresia (BA) is the most common cause of neonatal cholestasis, negatively affecting nutritional status, growth, and development. It is the most frequent paediatric indication for liver transplantation. The Kasai portoenterostomy (KPE) operation is an effective procedure with favourable outcomes when performed before two months of age. The present study aimed to assess the nutritional status of patients with biliary atresia who underwent the Kasai operation and to evaluate the effectiveness of nutritional counselling using medium-chain triglyceride (MCT) formulas and proper supplementation on their nutritional status, growth, and vitamin D levels., Patients and Methods: This prospective observational study included 36 infants with biliary atresia who underwent Kasai portoenterostomy. All patients underwent clinical assessment, anthropometric evaluation, nutritional counselling, and an evaluation of vitamin D levels. Only compliant patients (22/36) were followed up after 3 and 6 months of nutritional counselling., Results: Z-scores for weight, triceps skinfold thickness, and mid-upper arm circumference improved significantly after three months, and the height velocity Z-score improved after six months of nutritional counselling using an MCT-containing formula and supplementations. Patients who showed an improvement in cholestasis had better responses. The initial assessment revealed low serum levels of 25-hydroxyvitamin D in 77.8 %, which increased significantly (p = 0.012)., Conclusion: Dietary intervention and supplementation with MCT and micronutrients can improve the nutritional status of children with BA following KPE., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Pan-Arab Association of Gastroenterology. Published by Elsevier B.V. All rights reserved.)

Background: Liver failure and gastrointestinal bleeding occur in the end-stage of biliary atresia (BA). Living-donor liver transplantation (LDLT) is a standard treatment in Japan. Our program actively provides pre-transplant total parenteral nutrition (TPN) for such patients, and here we report its efficiency and safety., Methods: Patients with BA for whom LDLT was indicated were identified. Those with a long-term external central venous catheter and TPN, longer than 4 weeks before LDLT, were analyzed. Ascites was controlled with diuretics. TPN indications, efficacy, and complications were assessed along with patient growth, biochemical markers, and gastrointestinal bleeding., Results: Fourteen patients were included in the study, of whom 8 were girls and 6 were boys. The median age at LDLT was 0.9 years. Body weight (BW) at TPN initiation averaged 6799 g, and the median serum total bilirubin was 9.5 mg per dL. The median catheterization duration was 54 days, and 1 patient received home TPN. Indications for TPN were gastrointestinal bleeding and/or massive esophageal varices in 4 patients and poor nutritional status in 10 patients. No complications were observed except for 1 catheter infection and 1 catheter occlusion. The median final body weight before LDLT was 7906 g. The mean rate of BW gain was significantly higher after TPN than before (149 vs 32 g/wk, respectively, P = .0002). Mean prothrombin time and levels of albumin, cholinesterase, and total bilirubin were not significantly different at the start and end of TPN., Conclusions: Pre-transplant TPN was safe and effective for patients with end-stage BA., Competing Interests: Declaration of competing interest All the authors declare no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: Serum matrix metalloproteinase-7 (MMP-7) levels can precisely differentiate biliary atresia (BA) from non-BA cholestasis. However, serum MMP-7 levels of some BA patients were within normal range or slightly elevated. This study aimed to investigate the clinical characteristics and prognosis of biliary atresia with low serum MMP-7 levels., Method: This is a retrospective cohort study. Cases of BA from July 2020 to December 2022 were consecutively enrolled. They were divided into low-MMP-7 group (MMP-7 ≤ 25 ng/ml) and high-MMP-7 group (MMP-7 > 25 ng/ml) according to serum MMP-7 levels preoperatively. The perioperative clinical characteristics, the 3-month and 6-month jaundice clearance rate post-Kasai procedure, and the native liver survival were compared between the two groups., Results: A total of 329 cases were included in this study, 40 of which were divided into the low-MMP-7 group. Preoperative GGT and direct bilirubin levels in the low-MMP-7 group were significantly lower than those in the high-MMP-7 group (258.6 U/L, interquartile range [IQR]: 160.4411.6 vs. 406.8 IU/L, IQR: 215,655.0, P = 0.0076; 103.8 μmol/L, IQR: 79.0,121.4 vs. 115.3 μmol/L, IQR: 94,138.8, P = 0.0071), while the gender, the day at surgery and preoperative ALT, AST, TBA, total bilirubin levels showed no significant differences (P > 0.05). The 3-month and 6-month jaundice clearance rate post-Kasai procedure in the low-MMP-7 group were lower than those in the high-MMP-7 group (29.73% vs. 53.09%, P = 0.049; 32.14% vs. 54.73%, P = 0.023). The 1-year native liver survival rate was 29.63% for the low-MMP-7 group and 53.02% for the high-MMP-7 group (P = 0.022)., Conclusion: Preoperative clinical characteristics were similar between low-MMP-7 group and high-MMP-7 group, while patients with low serum MMP-7 levels showed worse prognosis, indicating that this might be listed as a new clinical subtype of BA which could contribute to designing new treatment strategies for BA in the future., Study Type: Cohort Study., Level of Evidence: Level II., Competing Interests: Conflicts of interest All authors declare that they have no conflict of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: To identify patients with biliary atresia (BA) with extremely poor outcomes of bile drainage surgery using the infant BA liver fibrosis (iBALF) score, a liver fibrosis marker based on standard blood analysis., Background: Although primary liver transplantation is beginning to be considered as an alternative to bile drainage surgery in patients with BA, those most likely to benefit from this procedure have not yet been identified., Methods: The medical records of 380 patients with BA with bile drainage surgery between 2015 and 2019 were collected for retrospective analysis from 60 participating hospitals. To predict native liver survival at age 1 year, a receiver operating characteristic curve was drawn for the iBALF score. The cutoff value was determined as the point indicating >99% sensitivity., Results: The median age at surgery was 56 days (range: 4-183 days), and native liver survival at age 1 year was achieved in 258 (67.9%) patients. An iBALF score of 5.27 was chosen as the cutoff, and 18 patients (4.7%) were found to have an iBALF score >5.27; of these, only 2 (95% CI: 1.4%-34.7%) had native liver survival at age 1 year, indicating a significantly poorer outcome than in the other patients (95% CI: 65.7%-75.4%). Moreover, patients with an iBALF score >5.27 had significantly higher mortality and younger age at salvage liver transplantation., Conclusions: Patients with BA having a preoperative iBALF score >5.27 had extremely poor outcomes of bile drainage surgery and may be considered candidates for primary LTx., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Several diseases originate from bile duct pathology. Despite studies on these diseases, certain etiologies of some of them still cannot be concluded. The most common disease of the bile duct in newborns is biliary atresia, whose prognosis varies according to the age of surgical correction. Other diseases such as Alagille syndrome, inspissated bile duct syndrome, and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction. The majority of these diseases present with cholestatic jaundice in the newborn or infant period, which is quite difficult to differentiate regarding clinical acumen and initial investigations. Intraoperative cholangiography is potentially necessary to make an accurate diagnosis, and further treatment will be performed synchronously or planned as findings suggest. This article provides a concise review of bile duct diseases, with interesting cases., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Objectives: To investigate the feasibility of using preoperative imaging indices to predict 2-year native liver survival after the Kasai procedure in patients with biliary atresia (BA)., Materials and Methods: The retrospective review included 190 BA patients who underwent the Kasai procedure between 2000 and 2020, with preoperative US and/or MRI, excluding cases with less than 2-year follow-up period. Multivariable logistic regression analysis was performed to identify imaging indices to predict 2-year native liver survival. Kasai failure was defined as the need for liver transplantation or death within 2 years of the Kasai procedure., Results: Of the 90 patients included, all had preoperative US, and 61 also had MRI. Kasai failure occurred in 52% (47/90). Preoperative US identified gallbladder length (OR 0.40, 95% CI 0.17-0.95, p = 0.039; cutoff 1.6 cm, AUC 67.66) and biliary cysts (OR 24.64, 95% CI 1.97-308.08, p = 0.013) as significant Kasai failure predictors, with a combined accuracy of 73% (60/82). For patients having both preoperative US and MRI, significant predictors were hepatic artery diameter (OR 6.75, 95% CI 1.31-34.88, p = 0.023; cutoff 2 mm, AUC 73.83) and biliary cysts (OR 23.89, 95% CI 1.43-398.82, p = 0.027) on US, and gallbladder length (OR 0.25, 95% CI 0.08-0.76, p = 0.014; cutoff 1.2 cm, AUC 74.72) and spleen size (OR 2.53, 95% CI 1.02-6.29, p = 0.045; cutoff 6.9 cm, AUC 73.72) on MRI, with a combined accuracy of 85% (52/61)., Conclusion: Preoperative US and/or MRI enhance the 2-year native liver survival prediction in BA patients after the Kasai procedure., Clinical Relevance Statement: BA patients with hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and absence of biliary cysts (US/MRI) have a decreased likelihood of 2-year native liver survival., Key Points: • Preoperative US and/or MRI can predict the probability of achieving 2-year native liver survival following the Kasai procedure. • Combining US and MRI improved the accuracy to 85% for predicting 2-year native liver survival in BA patients. • The hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and no biliary cysts (US/MRI) are significant predictors of Kasai failure in patients with biliary atresia., (© 2023. The Author(s), under exclusive licence to European Society of Radiology.)

Background: We utilized the data from the Saudi national biliary atresia (BA) study (2000-2018) to describe the clinical, biochemical, imaging, and histopathological features of BA and the perioperative clinical practices among local pediatric gastroenterologists., Methods: This is a retrospective, multicenter, nationwide study that included 10 tertiary care governmental hospitals including the four liver transplant (LT) centers in different regions across Saudi Arabia., Results: BA was diagnosed in 204 infants (106 females; 10% preterm). The median age at referral was 65 days. Congenital anomalies were present in 68 patients (33%); 22 were splenic malformation (10.8%). The medians of laboratory investigations were total bilirubin (189 μmol/l), direct bilirubin (139 μmol/l), ALT (164 u/l), and GGT (472 u/l). The level of serum GGT was normal in 26 cases (12.7%). The ultrasound findings included hypoplastic or atrophic gall bladder (GB) (65%), normal GB (30%), and cord sign (5%). A HIDA scan was performed in 99 cases (48.52%). Magnetic resonance cholangiopancreatography (MRCP) was performed in 27 cases (13%). A total of 179 liver biopsies (88%) were obtained. The most common histopathologic findings were bile duct proliferation (92%), canalicular cholestasis (96%), bile plugs (84%), and portal fibrosis (95%). Cholangiography was performed in 139 cases (68%): operative in 122 (60%) and percutaneous in 17 (8%). A total of 143 children (70%) underwent Kasai portoenterostomy (KPE) at a median age of 70 days. After KPE, steroid was used in 37% of the cases and 100 cases (70%) were prescribed prophylactic antibiotics for variable duration (ranging between 3 and 12 months)., Conclusion: Our data show marked variation in the diagnostic evaluation and perioperative management of BA cases among the different tertiary centers. There is a need to establish a national BA registry in Saudi Arabia aiming to standardize pre- and postoperative clinical practices. Additionally, normal serum GGT level, normal GB size on ultrasound, and being a premature baby should not preclude the diagnostic workup for BA., (Copyright © 2023 Copyright: © 2023 Saudi Journal of Gastroenterology.)

Bile acids have received increasing attention as a marker of the long-term prognosis and a potential therapeutic target in patients with biliary atresia, which is a progressive disease of the hepatobiliary system. A detailed analysis of serum and urinary bile acid compositions was conducted to assess the characteristics of bile acid profiles and the correlation between bile acid profiles and liver fibrosis markers in adult patients with biliary atresia who achieved bilirubin normalization. Serum total bile acids and glucuronide-conjugated (glyco- and tauro-) cholic acids (GCA and TCA) and chenodeoxycholic acids (GCDCA and TCDCA) were significantly higher in patients with biliary atresia than in healthy controls, whereas unconjugated CA and CDCA showed no significant difference. There were no significant differences in CA to CDCA ratios and glycine-to-taurine-conjugated ratios. Urinary glycocholic acid 3-sulfate (GCA-3S) was significantly higher in patients with biliary atresia. Serum GCDCA showed a strong positive correlation with Mac-2 binding protein glycosylation isomer (M2BPGi). These results demonstrate that bile acid congestion persists into adulthood in patients with biliary atresia, even after cholestasis has completely improved after Kasai portoenterostomy. These fundamental data on bile acid profiles also suggest the potential value of investigating bile acid profiles in patients with biliary atresia., (© 2024. The Author(s).)

Biliary atresia is an occlusive biliary disease involving intrahepatic and extrahepatic bile ducts. Its etiology and pathogenesis are unclear. There are many manifestations of bile duct involvement in biliary atresia, but little is known about its occurrence and development. In addition, different classification methods have been proposed in different periods of biliary atresia, each with its advantages and disadvantages. The combined application of biliary atresia classification will help to improve the survival rate of patients with native liver. Therefore, this article reviews the development, pathological features, and classification of intrahepatic and extrahepatic bile ducts in biliary atresia, to provide a reference for the study of the pathogenesis and the choice of treatment methods., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Biliary atresia (BA) is a severe congenital disorder with progressive obstructive cholangiopathy in young children. The inflammatory process has been recognized as one of the pathological mechanisms driving bile duct injury. Since interleukin-34 (IL-34) has been reportedly linked to several pathological liver disorders, including inflammation, the current study aimed to analyze circulating IL-34 and the association of circulating IL-34 with hepatic deterioration and clinical outcomes in post-Kasai BA children., Methods: Circulating IL-34 levels were analyzed in 89 post-Kasai BA subjects and 45 healthy individuals using an ELISA. Liver stiffness (hardness) was measured by ultrasound elastography., Results: Circulating IL-34 was substantially higher in BA children than in control individuals, particularly those with unfavorable outcomes including hepatic dysfunction, jaundice, and portal hypertension. In BA group, circulating IL-34 was positively correlated with liver stiffness (r = 0.515, p < 0.001), AST (r = 0.403, p < 0.001), ALT (r = 0.279, p = 0.008), total bilirubin (r = 0.224, p = 0.03), ALP (r = 0.255, p = 0.016), and serum IL-6 (r = 0.590, p < 0.001) but inversely correlated with albumin (r = -0.417, p < 0.001). Kaplan-Meier survival analysis showed that higher circulating IL-34 levels were significantly associated with reduced survival rates in BA subjects (p = 0.002)., Conclusion: Higher circulating IL-34 values were directly associated with hepatic impairment and the BA severity, implicating thatserum IL-34 could be applied as a noninvasive marker for the monitoring of the severity in BA subjects following Kasai portoenterostomy and therapeutic efficacy., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Purpose: Many patients with biliary atresia (BA) after the Kasai procedure (KP) progress to death or require liver transplantation to achieve long-term survival; however, most cases of death/liver transplantation (D/LT) occur in the early period after KP (usually within 1 year). This study was designed to construct a convenient nomogram for predicting early D/LT in patients with BA after KP., Methods: A BA cohort was established in May 2017, and up to May 2023, 112 patients with 1-5 years of follow-up were enrolled in the study and randomly (ratio, 3:1) divided into a training cohort for constructing a nomogram (n = 84) and a validation cohort (n = 28) for externally validating the discrimination and calibration. The training cohort was divided into two groups: the early D/LT group (patients who died or had undergone LT within 1 year after KP [n = 35]) and the control group (patients who survived through the native liver more than 1 year after KP [n = 49]). Multivariate logistic regression and stepwise regression were applied to detect variables with the best predictive ability for the construction of the nomogram. The discrimination and calibration of the nomogram were internally and externally validated., Results: The Kaplan-Meier (K-M) curve showed an actual 1-year native liver transplantation (NLS) rate of 57.1% and an estimated 2-year NLS rate of 55.2%. By multivariate regression and stepwise regression, age at KP, jaundice clearance (JC) speed 1 month after KP, early-onset PC (initial time < 36.5 days) after KP, sex, aspartate aminotransferase-to-platelet ratio index (APRI), and weight at KP were identified as the independent variables with the best ability to predict early D/LT and were used to construct a nomogram. The developed nomogram based on these independent variables showed relatively good discrimination and calibration according to internal and external validation., Conclusion: Most D/LTs were early D/LTs that occurred within 1 year after KP. The established nomogram based on predictors, including sex, weight at the KP, the APRI, age at the KP, JC speed 1 month after the KP, and early PC, may be useful for predicting early D/LT and may be helpful for counseling BA patients about patient prognosis after KP. This study was retrospectively registered at ClinicalTrials.gov (NCT05909033) in June 2023., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Aim: to review information resources and analysis of the own experience on this problem for the provision of modern knowledge in the pathogenesis of the pathology, the latest diagnostic and treatment technologies, with consideration of the need to adhere to a single strategy in the management of patients with BA., Patients and Methods: Materials and Methods: The analysis of the data regarding the results of existing studies evaluating the clinical benefit and safety of diagnostic and treatment methods in Biliary atresia., Conclusion: Conclusions: BA is the leading cause of neonatal cholestasis development. Early diagnostics of BA, based on the complex evaluation of clinical-laboratory, instrumental and morphological signs of the pathology, has a significant meaning. Surgical correction during the first 2 months of life - the Kasai procedure, as well as dynamic post-surgery follow-up significantly prolong the life of children and allow postponing liver transplantation. The highest patient survival both at the first stage of treatment - conduction of the Kasai procedure and the stage of liver transplantation may be achieved by joined work of surgeons and pediatricians, which allows considering the whole row of possible problems.