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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]

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]

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]