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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. [ABSTRACT FROM AUTHOR]

Aims: Biliary atresia (BA) is characterized by intrahepatic inflammation and rapid progression of liver fibrosis. Galectin‐3, a beta‐galactoside binding protein, is a key regulator of inflammation and fibrosis. The aim of this study was to characterize circulating and hepatic Galectin‐3 levels in children with BA. Methods: Plasma and liver samples were obtained from children with early BA at time of Kasai hepatoportoenterostomy, late BA at time of transplant, early and late other cholestatic liver diseases (CLD), and controls. Plasma Galectin‐3 was measured using standard enzyme‐linked immunoassay. Liver tissue was analyzed with multiplex immunohistochemistry and quantified using whole slide analysis. Statistical comparisons were made using nonparametric testing. Results: Plasma Galectin‐3 in late BA was significantly higher than in early BA (20.82 [12.45–30.46] vs. 11.30 [8.74–16.83] ng/mL, p = 0.0096). Galectin‐3 levels correlated with markers of disease severity and interleukin‐6. There were significantly more Galectin‐3+ M2 macrophages in late BA in comparison to late other CLD (162 [157–233] vs. 49 [33–59] cells/mm2, p = 0.03). The number of Galectin‐3+ M2 macrophages correlated with the number of activated hepatic stellate cells and bile duct proliferation. Conclusions: Plasma Galectin‐3 is higher in late BA at time of transplant in comparison to early BA at time of Kasai. The number of Galectin‐3 expressing M2 macrophages in late BA is elevated relative to late other CLD and was associated with other prognostic histological findings. Galectin‐3 targeted therapy may be beneficial in slowing disease progression to cirrhosis in children with BA. [ABSTRACT FROM AUTHOR]

Serum cortisol mainly binds to the cortisol-binding globulin (CBG). Children with biliary atresia (BA) may have low serum CBG levels; thus, low serum total cortisol (TC) levels and adrenal insufficiency (AI) may be overdiagnosed. This study aimed to assess adrenal function in children with BA. All the patients underwent adrenocorticotropic hormone (ACTH) stimulation tests. Plasma ACTH, serum TC, and CBG levels were measured at baseline, with additional TC measurements at 30 and 60 min during testing. Free cortisol (FC) index (FCI) and calculated FC (cFC) were also calculated. AI was defined as peak TC <500 nmol/L (<18 µg/dL), peak FCI <12 nmol/mg, or peak cFC <33 nmol/L (<1.2 µg/dL). This study enrolled 71 children with BA. The Median (IQR) age of the patients was 5.5 (1.7-11.4) years. Twenty-five (35%) patients were diagnosed with AI based on the peak TC. In the AI group, the median serum CBG level was significantly lower than that in the non-AI group (481 vs. 533 nmol/L, p = 0.03). Only eight patients (11%) met all three AI criteria (six secondary AI and two primary AI). In conclusion, low serum CBG levels contribute to reduced peak TC and, consequently, overdiagnosing AI. Peak FCI and cFC could help reduce the overdiagnosis of AI. [ABSTRACT FROM AUTHOR]

The impact of the coronavirus disease 2019 (COVID-19) pandemic has been immense, and it continues to have lasting repercussions. While the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus primarily infects the respiratory system, other organ systems are affected, including the liver. Scientific knowledge on the role of SARS-CoV-2 infection and liver injury has evolved rapidly, with recent data suggesting specific hepatotropism of SARS-CoV-2. Moreover, additional concerns have been raised in regard to long-term liver damage, related to emerging cases of post-COVID-19 cholangiopathy and chronic cholestasis. Great effort has also been focused on studying how specific subpopulations with chronic medical conditions might be disproportionately impacted by COVID-19. One such population includes individuals with chronic liver disease (CLD) and cirrhosis, with an expanding body of research indicating these patients being particularly susceptible to adverse outcomes. In this review, we provide an updated summary on the current pathogenesis and mechanism of liver injury in the setting of SARS-CoV-2 infection, the association between health outcomes and SARS-CoV-2 infection in patients with CLD, and the unique consequences of the COVID-19 pandemic on the routine care of patients with CLD. [ABSTRACT FROM AUTHOR]

Biliary atresia is a rare congenital disease of children with unknown pathogenesis and biliary obstruction, which is mainly manifested with progressive inflammation and fibrous obstruction of the biliary system inside and outside the liver, and subsequently develop into cholestatic cirrhosis and liver failure. Although biliary atresia is rare, it is the most common cause of cholestasis during the infancy period. If surgical treatment were not performed (such as Kasai operation and liver transplantation), children may die in a short period of time. Kasai operation can improve biliary drainage, whereas it fails to change the outcomes of children with biliary atresia. Most of them will still suffer from persistent liver injury due to cholestasis, and eventually require liver transplantation. At present, there is no consensus on whether Kasai operation should be performed prior to liver transplantation. In this article, research progress on the diagnosis and surgical treatment of biliary atresia was reviewed, aiming to provide reference for clinical diagnosis and treatment of biliary atresia and improve the survival of children with biliary atresia. [ABSTRACT FROM AUTHOR]

Background: Liver cirrhosis is an end stage liver damage. Biliary atresia (BA) and inherited syndromes of intrahepatic cholestasis are the most frequent causes of liver cirrhosis in children especially in the first year of life. Objective: To compare the prognostic value of albumin-bilirubin grade (ALBI), an alternative predictor of mortality in cirrhotic adults, with Child-Pugh and Pediatric End-Stage Liver Disease (PELD) scores in cirrhotic children with variceal bleeding. Methods: We analyzed the patients' data using MedCalc software, and calculated area under the curves (AUC) of receiving-operator characteristic (ROC). Results: All scores were higher in expired compared to survived patients (p<0.001). The AUC values for predicting mortality in all patients were 0.879±0.04 for Child-Pugh and PELD scores and 0.733±0.06 for ALBI. The prognostic values in two subgroups of BA and those with other underlying diseases (non-BA) showed that in BA group, ALBI score with AUC of 0.607±0.102, p<0.29, had no significant difference between survived and non-survived patients. ALBI in non-BA group, with AUC of 0.863±0.063 showed closer value to other scores (0.954), and was differed between survived and non-survived groups (p<0.001). Conclusion: Our results suggested that ALBI might be useful in predicting mortality in non-BA patients. [ABSTRACT FROM AUTHOR]

Purpose: There is no consensus regarding adjuvant therapies following Kasai portoenterostomy (KP) for biliary atresia (BA). This study aimed to analyze the effect of extended perioperative intravenous antibiotics (PI-Abx) and adjuvant corticosteroid on cholangitis and jaundice clearance rates in the 3 years post-KP in children with BA. Methods: Data of patients who underwent KP between 1999-2018 at a single center were retrospectively analyzed. Group A (1999-2010) received PI-Abx for 5 days, Group B (2010-2012) received PI-Abx for 5 days plus low-dose prednisolone (2 mg/kg), and Group C (2012-2017) received PI-Abx for 14 days plus high-dose prednisolone (5 mg/kg). Results: Fifty-four patients were included with groups A, B, and C comprising 25, 9, and 20 patients, respectively. The number of episodes of cholangitis was 1.0, 1.6, and 1.3 per patient (p=NS) within the first year and 1.8, 2.3, and 1.7 (p=NS) over 3 years in Groups A, B, and C, respectively. The jaundice clearance rate at 6 months was 52%, 78%, and 50% (p=NS), and the 3-year native liver survival (NLS) rate was 76%, 100%, and 80% (p=NS) in Groups A, B, and C, respectively. A near-significant association was observed between the incidence of cholangitis within the first year and decompensated liver cirrhosis/death at 3 years post KP (p=0.09). Persistence of jaundice at 6 months was significantly associated with decompensated cirrhosis/death at 3 years (p<0.001). Conclusion: The extended duration of PI-Abx and adjuvant corticosteroids was not associated with improved rates of cholangitis, jaundice clearance, or NLS in patients with BA. [ABSTRACT FROM AUTHOR]

Background: Although identifying cases in large administrative databases may aid future research studies, previous reports demonstrated that the use of the International Classification of Diseases, Tenth Revision (ICD-10) code alone for diagnosis leads to disease misclassification. Purpose: We aimed to assess the value of the ICD-10 diagnostic code for identifying potential children with biliary atresia. Methods: Patients aged <18 years assigned the ICD-10 code of biliary atresia (Q44.2) between January 1996 and December 2016 at a quaternary care teaching hospital were identified. We also reviewed patients with other diagnoses of code-defined cirrhosis to identify more potential cases of biliary atresia. A proposed diagnostic algorithm was used to define ICD-10 code accuracy, sensitivity, and specificity. Results: We reviewed the medical records of 155 patients with ICD-10 code Q44.2 and 69 patients with other codes for biliary cirrhosis (K74.4, K74.5, K74.6). The accuracy for identifying definite/probable/possible biliary atresia cases was 80%, while the sensitivity was 88% (95% confidence interval [CI], 82%-93%). Three independent predictors were associated with algorithm-defined definite/probable/possible cases of biliary atresia: ICD-10 code Q44.2 (odds ratio [OR], 2.90; 95% CI, 1.09-7.71), history of pale stool (OR, 2.78; 95% CI, 1.18-6.60), and a presumed diagnosis of biliary atresia prior to referral to our hospital (OR, 17.49; 95% CI, 7.01-43.64). A significant interaction was noted between ICD-10 code Q44.2 and a history of pale stool (P<0.05). The area under the curve was 0.87 (95% CI, 0.84-0.89). Conclusion: ICD-10 code Q44.2 has an acceptable value for diagnosing biliary atresia. Incorporating clinical data improves the case identification. The use of this proposed diagnostic algorithm to examine data from administrative databases may facilitate appropriate health care allocation and aid future research investigations. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia is a progressive obliterative cholangiopathy affecting both extrahepatic and intrahepatic biliary trees, resulting in fibrous obliteration of the biliary tract and subsequent development of cirrhosis. Objective: The aim of this study was to find noninvasive indices to predict the status of hepatic fibrosis in children with biliary atresia. Materials and methods: We retrospectively measured the volume of the hepatic lobes and spleen from MR images, obtained biochemical data and analyzed the relationship between the imaging and biochemical indices, and the pathological status of hepatic fibrosis in 35 children with biliary atresia. Results: A combined index was obtained by logistic regression: logit (likelihood of cirrhosis) = 0.00043 x age at MR examination + 1.67 x aspartate aminotransferase and platelet ratio index (APRI) + 0.0029 x body-surface-area-adjusted left liver lobe volume (BSA adLLV) – 6.57 (log-likelihood chi-square P<0.05, pseudo-R2=0.59). The area under the receiver operator characteristic curve of age at MR examination, APRI, BSA adLLV and the combined index for prediction of cirrhosis were 0.91, 0.86, 0.83 and 0.94, respectively. The optimal cut-off value (sensitivity and specificity) of age at MR examination, APRI, BSA adLLV and combined index were 132 (86% and 92%), 1.3 (91% and 85%), 855.5 (96% and 62%) and 0.689 (91% and 92%). The accuracy of age at MR examination, APRI, BSA adLLV and combined index were 89%, 89%, 83% and 91%, respectively. Conclusion: A combined noninvasive index of age, aspartate aminotransferase and platelet ratio index, and the body-surface-area-adjusted left liver lobe volume measured from MR images is a potential marker of liver cirrhosis in children with biliary atresia. [ABSTRACT FROM AUTHOR]

Aim: To determine factors associated with severe hospitalized Respiratory syncytial virus (RSV)‐associated LRTI and to describe management in tertiary care center. Methods: Retrospective medical record review was conducted among children under 5 years old hospitalized with RSV‐associated LRTI at King Chulalongkorn Memorial Hospital. Severe RSV‐associated LRTI was defined as death, mechanical ventilator, or positive pressure ventilation use, prolonged hospitalization >7 days. Factors associated with severe RSV were analyzed using univariate and multivariate logistic regression. Results: From January 2011 to December 2016, 427 children were hospitalized. Median age was 10 months (IQR 4.2‐23.0). One hundred seventy‐four (41%) patients had severe RSV (11 deaths, 56 mechanical ventilators, 19 positive pressure ventilation, and 88 prolonged hospitalization). Factors associated with severe RSV were chronic lung disease (aOR 15.16 [4.26‐53.91]), cirrhosis/biliary atresia (aOR 15.01 [3.21‐70.32]), congenital heart disease (aOR 5.11 [1.97‐13.23]), chemotherapy (aOR 4.7 [1.34‐16.56]), and pre‐term (aOR 2.03 [1.13‐3.67]). Oxygen therapy was mainly low flow oxygen delivery. 88% of cases received bronchodilator. Parenteral antibiotics were prescribed in 37.9% of cases. Conclusions: Children with co‐morbidities have higher risk of severe RSV‐associated LRTI. More than two‐third of patients received bronchodilator, of which was not recommended by American Academy of Pediatrics. The specific treatment and prevention for RSV are urgently needed. [ABSTRACT FROM AUTHOR]

Cirrhosis is the frightening complication of chronic liver disease. Common causes include biliary atresia, a pathology with a poor prognosis in the absence of a prompt diagnosis and therapeutic intervention. We present the case of a patient diagnosed with biliary atresia who underwent Kasai intervention. After an initial period with disappearance of the jaundice and normal color of the stool, the patient reached the stage of decompensated cirrhosis. Among the complications suffered by the patient we mention ascites, esophageal varices, upper gastrointestinal hemorrhage, repeated infections, anemia, rickets, and hepatic osteodystrophy. For this patient, the only long-term therapeutic option remains liver transplantation. Although cases of pediatric patients with an early Kasai intervention and survival with native liver are described, the long-term evolution depends on the occurrence of possible complications and most patients eventually require liver transplantation. [ABSTRACT FROM AUTHOR]

Background & Aims Primary prophylaxis of bleeding is debated for children with portal hypertension because of the limited number of studies on its safety and efficacy, the lack of a known endoscopic pattern carrying a high-risk of bleeding for all causes, and the assumption that the mortality of a first bleed is low. We report our experience with these issues. Methods From 1989 to 2014, we managed 1300 children with portal hypertension. Endoscopic features were recorded; high-risk varices were defined as: grade 3 esophageal varices, grade 2 varices with red wale markings, or gastric varices. Two hundred forty-six children bled spontaneously and 182 underwent primary prophylaxis. The results of primary prophylaxis were reviewed as well as bleed-free survival, overall survival and life-threatening complications of bleeding. Results High-risk varices were found in 96% of children who bled spontaneously and in 11% of children who did not bleed without primary prophylaxis ( p <0.001), regardless of the cause of portal hypertension. Life-threatening complications of bleeding were recorded in 19% of children with cirrhosis and high-risk varices who bled spontaneously. Ten-year probabilities of bleed-free survival after primary prophylaxis in children with high-risk varices were 96% and 72% for non-cirrhotic causes and cirrhosis respectively. Ten-year probabilities of overall survival after primary prophylaxis were 100% and 93% in children with non-cirrhotic causes and cirrhosis respectively. Conclusion In children with portal hypertension, bleeding is linked to the high-risk endoscopic pattern reported here. Primary prophylaxis of bleeding based on this pattern is fairly effective and safe. Lay summary In children with liver disease, the risk of bleeding from varices in the esophagus is linked to their large size, the presence of congestion on their surface and their expansion into the stomach but not to the child’s age nor to the cause of portal hypertension. Prevention of the first bleed in children with high-risk varices can be achieved by surgery or endoscopic treatment, and decreases mortality and morbidity. [ABSTRACT FROM AUTHOR]

Background: Preliminary studies have shown that diffusion tensor imaging (DTI) is helpful in evaluating liver disorders. However, there is no published literature on the use of DTI in the diagnosis of biliary atresia (BA). This study aimed to investigate the diagnostic value of the liver average apparent diffusion coefficient (ADC) and fractional anisotropy (FA) measured using DTI for BA in neonates and infants. Methods: Fifty-nine patients with infant jaundice were included in this study. DTI was performed with b factors of 0 and 1000 s/mm2. Liver fibrosis in the BA group was determined and graded (F0, F1, F2, F3, F4) based on the pathological findings. Statistical analyses were performed to determine the diagnostic accuracy of DTI for BA. Results: The ADC value was significantly lower in the BA group [(1.262±0.127)×10−3 mm2/s] than in the non-BA group [(1.430±0.149)×10−3 mm2/s, (P<0.001)]. The area under the receiver operating characteristic curve was 0.805±0.058 (P<0.001) for ADC. With a cut-off value of 1.317×10−3 mm2/s, ADC achieved a sensitivity of 75% and a specificity of 81.5% for the differential diagnosis of BA and non-BA. In the BA group, the ADC value was significantly correlated with fibrotic stage. Further analysis showed that the ADC value of stage F0 was significantly higher than that of stages F1, F2, F3 and F4, whereas there were no significant differences among stages F1, F2, F3 and F4. Conclusion: Hepatic ADC measured with DTI can be used as an adjunct to other noninvasive imaging methods in the differential diagnosis of BA and non-BA. ADC was helpful in detecting liver fibrosis but not in differentiating the fibrotic grades. [ABSTRACT FROM AUTHOR]

Purpose: To compare preoperative CT findings before liver transplantation between patients with Alagille syndrome (AGS) and those with end-stage biliary atresia (BA). Materials and Methods: The institutional review board approved this retrospective study. Eleven children with AGS (median age, 19.0 ± 13.0 months; male to female ratio, 3:8) and 109 children with end-stage BA (median age, 17.9 ± 25.8 months; male to female ratio, 37:72) who underwent abdomen CT as candidates for liver transplant were included. CT images were reviewed focusing on hepatic parenchymal changes, vascular changes, presence of focal lesions, and signs of portal hypertension. Results: Hepatic parenchymal changes were present in 27% (3/11) of AGS patients and 100% (109/109) of end-stage BA patients (P < .001). The hepatic artery diameter was significantly smaller (1.9 mm versus 3.6 mm, P = 008), whereas portal vein diameter was larger (6.8 mm versus 5.0 mm, P < .001) in patients with AGS compared with patients with end-stage BA. No focal lesion was seen in patients with AGS, whereas 44% (48/109) of patients with end-stage BA had intrahepatic biliary cysts (39%, 43/109) and hepatic tumors (8%, 9/109) (P = .008). Splenomegaly was commonly seen in both groups (P = .082), and ascites (9% [1/11] versus 50% [54/109], P = .010) and gastroesophageal varix (0% [0/11] versus 80% [87/109], P < .001) were less common in patients with AGS than in patients with end-stage BA. Conclusion: Fibrotic or cirrhotic changes of the liver, presence of focal lesions, and relevant portal hypertension were less common in patients with AGS than in patients with end-stage BA. [ABSTRACT FROM AUTHOR]

Liver diseases in children have variable clinical manifestations, from asymptomatic increase of transaminases to fulminant liver failure. The disease evolution could be very severe, even fatal without right treatment on right moment. The level of transaminases is not a very valuable for predicting the severity of the disease. In neonatal cholestasis, biliary atresia is very important as it could have a severe evolution to cirrhosis and death. The most important prognostic factors in biliary atresia are the precocity of the diagnostic, the age at the surgical intervention, the experience of the surgical team. In acute liver failure there are many prognostic scores that include level of transaminases, coagulation factors or albumin level, with some differences depending on the etiologies. One of the clinical onset forms in Wilson disease is the fulminant liver failure. The severity and the need of liver transplantation could be assessed using a dedicated prognostic score. Even considered irreversible, liver fibrosis is a dynamic process potential reversible. The quick diagnostic, correct evaluation and timely therapy could limit the unfavorable consequences of liver cirrhosis. Liver biopsy is the gold standard in fibrosis evaluation, but sometimes the risks could limit its value. Non-invasive evaluation of liver fibrosis is based on measuring the serum level of makers involved in fibrosis or on imagistic methods and tend to replace the liver biopsy. In liver cirrhosis, there are different scores used for evaluation of the severity. Among those scores, PELD (pediatric end-stage liver disease) is used to evaluate the need of liver transplantation and also the urge of it. In conclusion, knowing the prognostic factors in different liver diseases is very important in order to assess the need of high specialized care in a liver center including liver transplantation in order to increase the survival of those children. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is a disease of the extrahepatic biliary tree that presents with biliary obstruction in the neonatal period, which is caused by fibro-obliterative process. Kasai procedure, a hepatoportoenterostomy (HPE) as an attempt to restore bile flow from the liver to the proximal small bowel, has been shown to improve survival in BA patients. Many BA survivals who had undergone Kasai HPE will have slowly progressive liver disease and the majority of patients will ultimately require liver transplantation. In spite of many experimental treatments, cirrhosis still occurs in BA patients survival. This case report presents a male patient with biliary atresia that has survived for 27 years after Kasai procedure. He had been repeatedly admitted to hospital with complications caused by cirrhosis, such as repeated variceal and hemorrhoid bleeding and also refractory ascites. These complications are indications for liver transplantation. Although Kasai HPE procedure improves survival in BA patients in Indonesia, long-term complications of cirrhosis makes the patient awaits for liver transplantation. [ABSTRACT FROM AUTHOR]

Biliary atresia is the result of progressive fibrosis and obliteration of intra and extrahepatic bile ducts, this condition is particularly pediatric and occurs mainly between the second and fourth weeks of life and is the leading cause of liver cirrhosis and fatal within the first year of life, and the most common indication for liver transplantation in children. [ABSTRACT FROM AUTHOR]

de Vries W, de Langen ZJ, Aronson DC, Hulscher JBF, Peeters PMJG, Jansen-Kalma P, Verkade HJ also on behalf of NeSBAR. Mortality of biliary atresia in children not undergoing liver transplantation in the Netherlands. Pediatr Transplantation 2011: 15:176-183. © 2010 John Wiley & Sons A/S. In order to further improve the outcome of BA, we characterized the mortality of BA patients who did not undergo OLT in the Netherlands, and compared our results with international data. For this purpose, we analyzed the causes of mortality of non-transplanted BA patients before the age of five yr, using the NeSBAR database. To evaluate trends in mortality, we compared the cohort 1987-1996 (n = 99) with 1997-2008 (n = 111). We compared clinical condition at OLT assessment with available international data, using the PELD-score. Mortality of non-transplanted BA children was 26% (26/99) in 1987-1996 and 16% (18/111) in 1997-2008 (p = 0.09). Sepsis was the prevailing direct cause of death (30%; 13/44). PELD-scores at the time of assessment were higher in non-transplanted BA patients (median 20.5; range 13-40) compared with international data (mean/median between 11.7 and 13.3). Based on our national data, we conclude that pretransplant mortality of BA patients is still considerable, and that sepsis is a predominant contributor. Our results strongly indicate that the prognosis of patients with BA in the Netherlands can be improved by earlier listing of patients for OLT and by improving pretransplant care. [ABSTRACT FROM AUTHOR]

Biliary atresia is the most common cholangiopathy of childhood. With complete obstruction of segments or the entire length of extrahepatic bile ducts, the timely pursuit of hepatoportoenterostomy is the best strategy to restore bile drainage. However, even with prompt surgical intervention, ongoing injury of intrahepatic bile ducts and progressive cholangiopathy lead to end-stage cirrhosis. The pace of disease progression is not uniform; it may relate to clinical forms of disease and/or staging of liver pathology at diagnosis. Although the etiology of disease is not yet defined, several biological processes have been linked to pathogenic mechanisms of bile duct injury. Among them, there is increasing evidence that the immune system targets the duct epithelium and disrupts bile flow. We discuss how careful clinical phenotyping, staging of disease, and basic mechanistic research are providing insights into clinical trial designs and directions for development of new therapies to block progression of disease. [ABSTRACT FROM AUTHOR]

This report correlates the clinical and biological findings, liver hemodynamics and histological features of focal INL in an infant with BA cirrhosis. An eight month old boy with BA, with previous successful porto-enterostomy, was admitted with signs of cholangitis and ascites. He was treated with antibiotics and diuretics with subsequent clinical improvement. Eight days later, while being fed with hyper-osmolar milk, he became febrile again: ASAT/ALAT climbed (9000/2300 IU/L), liver function deteriorated. Infectious work-up was negative. Liver-ultrasound showed reversed portal flow and a negative arterial diastolic flow. The patient recovered within five days under supportive treatment. A similar event recurred five days later. INL was suspected and semi-urgent living-related liver transplantation was performed, with uneventful post-operative course. Histology of the explanted liver showed extensive foci of INL of different ages. This report illustrates how the association of reversed portal and arterial diastolic flows, with subsequent liver hypoperfusion, may repeatedly cause foci of INL in BA cirrhosis, and lead to rapid progression to liver failure. Because of precarious hepatic blood supply in such patients, close monitoring of portal and diastolic arterial flows is recommended. [ABSTRACT FROM AUTHOR]

Objective: Biliary atresia is the result of a fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Without surgical intervention, mortality reaches 100%. The 5-year survival rate after portoenterostomy ranges from 13% to 60%, with approximately 60% of patients requiring liver transplantation at a later stage because of insufficient bile flow. Methods: This retrospective analysis includes 30 consecutive patients undergoing portoenterostomy for biliary atresia at our hospital. Results: The 5-year actuarial survival of the 30 patients was 68%. Thirteen patients (43.3%) died 3 days to 7 years after portoenterostomy. Four patients (13.3%) underwent liver transplantation 3 to 24 months after the Kasai procedure with a 100% survival. In 65% of patients without presence of cirrhosis, the portoenterostomy was successful, compared with 35% of cases with liver cirrhosis (p = 0.0148). Liver cirrhosis with extrahepatic biliary atresia alone was present in 5 of 17 patients (29%) as compared with 8 of 12 patients (66%) with intrahepatic biliary hypoplasia in addition to extrahepatic biliary atresia and cirrhosis. Conclusions: Portoenterostomy remains the treatment of choice for patients with extrahepatic biliary atresia. However, the presence of cirrhosis portends a poorer prognosis and may be an indication for early transplantation. Cirrhosis is more commonly present in the setting of intrahepatic biliary hypoplasia and may account for the lower success rates of portoenterostomy in this group of patients. Five-year survival of the female patients was 88% as compared with 55% of the male patients. [Copyright &y& Elsevier]

The majority of patients with biliary atresia (BA) develop progressive liver disease and serious complications of portal hypertension, including esophageal varices and ascites, despite successful Kasai operation. The aim of this study was to evaluate whether serum hyaluronan (HA) reflects the severity of cirrhosis and its complications in patients with postoperative BA. Fifty-four postoperative BA patients aged 1 to 19 years were recruited into this study. Serum HA was determined by ELISA-based assay, and serum HA levels were compared among groups of patients according to Child’s classification as well as the presence of esophageal varices and ascites. Mean serum HA concentration was significantly increased in patients with Child C (534.35±573.02 ng/ml) in comparison to patients with Child B (97.67±104.22 ng/ml,p=0.002) and Child A (36.27±71.41 ng/ml,p<0.001). Mean serum HA concentration was also significantly higher in patients who had ascites or esophageal varices than in those who did not suffer from these complications. Furthermore, patients who had variceal hemorrhage had significantly higher mean serum HA levels than those without bleeding. Thus, serum HA levels may reflect the severity of cirrhosis and its complications, and may be useful in monitoring the clinical outcomes of patients with postoperative BA. [ABSTRACT FROM AUTHOR]

To determine the role of redo hepatic portoenterostomy (HPE) in biliary atresia (BA) patients with insufficient bile excretion after the initial HPE, 25 patients (type I, correctable: 2; type III, uncorrectable: 23) undergoing the initial HPE at 25 to 119 days of age were studied. Four patients achieved disappearance of jaundice (total bilirubin [T.Bil] < 2 mg/dl) postoperatively. A redo HPE was performed at 2 to 8 months of age with sufficient and extensive removal of granulation and scar tissue at the hepatic hilum. Five patients became free of jaundice in 3 to 6 months (group 1), while the remaining 20 did not (group 2). Disappearance of jaundice after the initial HPE had been achieved in 2 of 5 patients (40%) in group 1 and 2 of 20 (10%) in group 2 ( P < 0.05). Age, serum T.Bil, aspartate aminotransferase albumin, prothrombin time, cholinesterase, total cholesterol, and Fischer's ratio at redo HPE showed no significant differences between the two groups. On liver histology obtained at redo HPE, cirrhosis and hepatocyte degeneration were seen in 1 of 5 cases (20%) in group 1 and 12 of 20 (60%) in group 2 ( P < 0.05). Redo HPE may thus be effective in BA patients with insufficient bile drainage who achieved disappearance of jaundice after the initial HPE and have not developed cirrhosis. [ABSTRACT FROM AUTHOR]

Background/Aims Variceal bleeding (VB) is seen both with childhood cholestatic as well as hepatocellular disorders. There is limited data on morbidity related to VB in infants and children. We aimed to study the organ failures and outcome of cirrhotic children with VB necessitating liver transplantation (LT). Methods We retrospectively collected and analyzed data of all infants and children with presence of cirrhosis and variceal bleed who presented between December 2010 and March 2019. Severity scores, organ failures and outcome were analysed. Results We identified 101 variceal bleeding episodes in 79 children (30.4% females)-median one episode (range, 1 to 8) and >/=3 VB happened in 14 children. The commonest etiologies were biliary atresia (35.4%), autoimmune hepatitis (21.5%), hepatic venous outflow tract obstruction (11.4%) and other genetic and cholestatic conditions (25.3%). Sixteen of these episodes were tolerated well without development of second decompensation. One or more organ failure was present in 98 episodes-median Pediatric CLIF-SOFA score was 6 (interquartile range, 4 to 10). Seven children developed advance (grade 3-4) hepatic encephalopathy while stage 3-4 acute kidney injury was seen in 11 children. By 90 days, 12 children received living donor LT, while 11 died. On univariate analysis, mortality at day-90 was associated with presence of Hepatopulmonary syndrome, hepatic encephalopathy, pneumonia and spontaneous bacterial peritonitis and with high bilirubin, creatinine, international normalized ratio, PELD/Model for End Stage Liver Disease, Child-Pugh and pediatric CLIF-SOFA scores and low sodium. On multivariate analysis, pediatric CLIF-SOFA (Wald, 10.593; p=0.001) and Hepatopulmonary syndrome (Wald, 4.079; p=0.043) were associated with poor outcome. There was recurrence of VB in 31 children over a median duration of 36 months. Conclusions Variceal bleeding in children is associated with organ failures which affect short term prognosis. Hepatopulmonary syndrome also relates to poor outcome in these children. [ABSTRACT FROM AUTHOR]

An abstract of the article "Effect of Ursodeoxycholic Acid on Liver Function in Children After Successful Surgery for Biliary Atresia," by Stephanie Willot, Stephanie Uhlen, Laurent Michaud, Gilbert Briand, Michel Bonnevalle, Rony Sfeir, and Frédéric Gottrand is presented.