Article
Immunology
Jinchuan Liu, Yi Fei, Tao Zhou, Hao Ji, Ji Wu, Xiangqian Gu, Yi Luo, Jianjun Zhu, Mingxuan Feng, Ping Wan, Bijun Qiu, Yefeng Lu, Tian Yang, Pengfei Deng, Cuiping Zhou, Dongcheng Gong, Jun Deng, Feng Xue, Qiang Xia
Summary: This study reveals extremely low routine vaccination rates in Chinese children with BA and associates poor responses to the HBV vaccine with bile acid levels. Bile acids may impact vaccine responses by inhibiting post-class-switched memory B cell reactions.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Gastroenterology & Hepatology
Sarah Kemme, Jennifer D. Canniff, Amy G. Feldman, Krystle M. Garth, Shaobing Li, Zhaoxing Pan, Ronald J. Sokol, Adriana Weinberg, Cara L. Mack
Summary: This study aimed to investigate the relationship between cytomegalovirus (CMV) infection and the severity of disease and pretransplant outcomes in infants with biliary atresia (BA). The results showed that CMV infection was associated with worse liver injury, delayed surgery, and increased risk of pretransplant death, but had no effect on native liver survival.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Medicine, General & Internal
Won Shik Kim, Seung Han Kim, Moon Kyung Joo, Jong-Jae Park, Beom Jae Lee, Hoon Jai Chun
Summary: Objective Non-variceal upper gastrointestinal bleeding (NVUGIB) in patients receiving oral anticoagulants (OACs) may be fatal; however, little is known about re-bleeding and all-cause mortality after successful hemostasis. We investigated the clinical characteristics and risk factors for re-bleeding and death after successful hemostasis. The study found that 30.3% of patients experienced re-bleeding after successful hemostasis, with a mortality rate of 37.5% among these patients, and 2.3% of deaths were related to re-bleeding.
ANNALS OF MEDICINE
(2023)
Article
Pediatrics
Ioannis A. Ziogas, Fei Ye, Zhiguo Zhao, Shu Cao, Muhammad A. Rauf, Manhal Izzy, Lea K. Matsuoka, Lynette A. Gillis, Sophoclis P. Alexopoulos
Summary: The study identified factors such as increasing bilirubin level, portal vein thrombosis, and ventilator dependence at listing associated with higher risk of waitlist mortality, while weight >= 10 kg at listing associated with lower risk. Ascites at listing was also found to be associated with a higher risk for the composite outcome.
JOURNAL OF PEDIATRICS
(2021)
Article
Immunology
Wei Song, Li-Ying Sun, Zhi-Jun Zhu, Lin Wei, Wei Qu, Zhi-Gui Zeng, Ying Liu, Hai-Ming Zhang, Wei Guo
Summary: The study revealed that the gut microbiota structure in patients with biliary atresia was disturbed in both early and later stages, and specific bacteria such as Klebsiella, Streptococcus, Veillonella, and Enterococcus may be associated with disease progression, particularly in terms of metabolic dysregulation.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Pediatrics
Fang-Min Liao, Kai-Chi Chang, Jia-Feng Wu, Huey-Ling Chen, Yen-Hsuan Ni, Mei-Hwei Chang
Summary: This study aimed to determine the sensitivity and specificity of elevated direct bilirubin (D-bil) levels and the direct-to-total bilirubin (D/T) ratio for detecting biliary atresia (BA) in high-risk infants. The findings showed that D-bil >= 1.0 mg/dL was better for detecting BA in infants aged 3 to 60 days than the D/T ratio. However, a more definitive cutoff point is required for newborns aged <3 days.
Article
Gastroenterology & Hepatology
Anna M. Banc-Husu, Matt Hall, Cary Thurm, Bridget A. Whitehead, Justin A. Godown, Estella M. Alonso
Summary: Acute-on-chronic liver failure (ACLF) in children with biliary atresia (BA) awaiting liver transplantation (LT) is associated with increased resource utilization and longer waitlist times. However, ACLF status does not affect post-transplant mortality in these patients. Further studies are needed to better understand the spectrum of ACLF and prioritize critically ill children awaiting LT.
LIVER TRANSPLANTATION
(2023)
Article
Physiology
Wei Song, Li-Ying Sun, Zhi-Jun Zhu, Lin Wei, Wei Qu, Zhi-Gui Zeng, Yun-Sheng Yang
Summary: The study analyzed the changes in gut microbiota (GM) before and after liver transplantation (LT) in patients with biliary atresia (BA), finding that LT partially improved the dysbiosis of GM by reducing certain bacteria and increasing the levels of short chain fatty acid (SCFA)-producing bacteria, though not fully restoring to normal levels.
FRONTIERS IN PHYSIOLOGY
(2021)
Article
Medicine, General & Internal
Thai Doan Ky, Nguyen Thi Huyen Trang, Mai Thanh Binh, Gian Paolo Caviglia
Summary: The ABC score is closely associated with the rates of early rebleeding and in-hospital mortality in cirrhotic patients with variceal bleeding, and has potential for clinical application.
Article
Immunology
Ting Yang, Shen Yang, Jiawei Zhao, Peize Wang, Siqi Li, Yuyan Jin, Zhaozhou Liu, Xinyue Zhang, Yanan Zhang, Yong Zhao, Junmin Liao, Shuangshuang Li, Kaiyun Hua, Yichao Gu, Dingding Wang, Jinshi Huang
Summary: There are differences in gut microbiota and bile acid composition in patients with biliary atresia, and their interaction is related to the occurrence of cholangitis and jaundice clearance.
FRONTIERS IN CELLULAR AND INFECTION MICROBIOLOGY
(2022)
Article
Pediatrics
Tingting Li, Xinzhe Wei, Xiaoye Hao, Xuying Ye, Chao Li, Qi Li, Zhuqing Li, Wei Gao, Chengzhi Lu
Summary: Subclinical cardiac abnormalities in pediatric patients with biliary atresia are associated with post-liver transplantation mortality and morbidity. Left ventricular mass index can predict the occurrence of death and serious adverse events after liver transplantation.
FRONTIERS IN PEDIATRICS
(2023)
Article
Gastroenterology & Hepatology
Julie Osborn, Reena Mourya, Unmesha Thanekar, Weizhe Su, Lin Fei, Pranavkumar Shivakumar, Jorge A. Bezerra
Summary: Through large-scale proteomics, we identified SEMA6B, SFRP3, COMMD7, BMX, and VCAM1 as serum biomarkers highly associated with clinical portal hypertension in children with biliary atresia. Immunostaining of the new protein biomarkers showed increased expression in hepatic endothelial cells, cholangiocytes, and immune cells within portal triads in BA livers with clinical PHT compared to healthy livers.
HEPATOLOGY COMMUNICATIONS
(2022)
Article
Cell & Tissue Engineering
Thanh Liem Nguyen, Hoang Phuong Nguyen, Duy Minh Ngo, Thu Hien Thi Ha, Kieu-Anh Mai, Thu Hang Bui, Phan Van Nguyen, Lan Huong Pham, Duc Minh Hoang, Anh Dao Thi Cao
Summary: This study aimed to evaluate the safety and early outcomes of autologous bone marrow mononuclear cell (BMMNC) infusion for liver cirrhosis caused by biliary atresia (BA) after Kasai operation. The results showed that this treatment approach was safe and may help maintain or improve liver function, reducing the severity of the disease.
STEM CELL RESEARCH & THERAPY
(2022)
Article
Pediatrics
Andrea Ho, Marla A. Sacks, Amita Sapra, Faraz A. Khan
Summary: The study found that the absence of gallbladder on ultrasound has high specificity and positive predictive value for diagnosing BA, but it is not useful for ruling out BA due to its low sensitivity.
FRONTIERS IN PEDIATRICS
(2021)
Article
Pediatrics
Jin-qiao Liu, Wen-juan Chen, Meng-jie Zhou, Wen-feng Li, Ju Tang, Qi-chang Zhou
Summary: A nomogram was established to predict the prognosis of children with biliary atresia within 2 years after Kasai portoenterostomy. Independent predictors such as age at surgery, METAVIR score, liver stiffness measurement at 3 months, first onset of cholangitis within 3 months, and jaundice clearance time were identified. The nomogram showed good discrimination and calibration in internal and external validation, outperforming individual predictors in predicting the prognosis.
FRONTIERS IN PEDIATRICS
(2021)
Review
Gastroenterology & Hepatology
Binita M. Kamath, Estella M. Alonso, James E. Heubi, Saul J. Karpen, Shikha S. Sundaram, Benjamin L. Shneider, Ronald J. Sokol
CLINICS IN LIVER DISEASE
(2022)
Article
Gastroenterology & Hepatology
Ariel Porto, Zhaoxing Pan, Wenru Zhou, Ronald J. Sokol, Kelly Klaczkiewicz, Shikha S. Sundaram
Summary: This study aimed to evaluate the macronutrient and micronutrient intake in adolescents with NAFLD and determine nutritional predictors of disease severity. The results showed that NAFLD patients had similar intake of total calories, protein, fat, and carbohydrates as those without NASH, but NASH patients had higher sugar intake. The severity of the liver disease was correlated with total carbohydrate and added sugar intake.
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2022)
Article
Critical Care Medicine
Matthew K. Wynia, Laurel E. Beaty, Tellen D. Bennett, Nichole E. Carlson, Christopher B. Davis, Bethany M. Kwan, David A. Mayer, Toan C. Ong, Seth Russell, Jeffrey D. Steele, Heather R. Stocker, Adane F. Wogu, Richard D. Zane, Ronald J. Sokol, Adit A. Ginde
Summary: This observational cohort study found that treatment with neutralizing monoclonal antibodies (mAbs) was associated with reduced hospitalization and deaths among COVID-19 outpatients. The benefits of mAb treatment persisted during the Delta variant phase.
Article
Gastroenterology & Hepatology
Daniel H. Leung, Sridevi Devaraj, Nathan P. Goodrich, Xinpu Chen, Deepthi Rajapakshe, Wen Ye, Victor Andreev, Charles G. Minard, Danielle Guffey, Jean P. Molleston, Lee M. Bass, Saul J. Karpen, Binita M. Kamath, Kasper S. Wang, Shikha S. Sundaram, Philip Rosenthal, Patrick McKiernan, Kathleen M. Loomes, M. Kyle Jensen, Simon P. Horslen, Jorge A. Bezerra, John C. Magee, Robert M. Merion, Ronald J. Sokol, Benjamin L. Shneider
Summary: This study investigated biomarkers for hepatic fibrosis in pediatric cholestasis patients. The researchers found that endoglin, IL-8, and MMP-7 were positively correlated with liver stiffness in children with biliary atresia, while CTGF was negatively correlated in alpha-1 antitrypsin deficiency patients. These biomarkers may enhance the prediction of liver stiffness and facilitate early interventions.
Article
Gastroenterology & Hepatology
Sanjiv Harpavat, Kieran Hawthorne, Kenneth D. R. Setchell, Monica Narvaez Rivas, Lisa Henn, Charlotte A. Beil, Saul J. Karpen, Vicky L. Ng, Estella M. Alonso, Jorge A. Bezerra, Stephen L. Guthery, Simon Horslen, Kathy M. Loomes, Patrick McKiernan, John C. Magee, Robert M. Merion, Jean P. Molleston, Philip Rosenthal, Richard J. Thompson, Kasper S. Wang, Ronald J. Sokol, Benjamin L. Shneider
Summary: Serum bile acid levels may help predict outcomes in infants with normalized bilirubin levels after Kasai portoenterostomy.
Article
Multidisciplinary Sciences
Colin T. Shearn, Aimee L. Anderson, Michael W. Devereux, David J. Orlicky, Cole Michel, Dennis R. Petersen, Colin G. Miller, Sanjiv Harpavat, Edward E. Schmidt, Ronald J. Sokol
Summary: This study investigates the interactions between oxidative stress and autophagy in cholestasis using mouse and human liver tissue. The results show a dysregulation of oxidative stress and autophagy in the same periportal hepatocyte compartment, suggesting that increased expression of oxidatively-modified proteins and ineffective autophagic degradation may contribute to liver cell injury.
Article
Gastroenterology & Hepatology
Ronald J. Sokol, Emmanuel M. Gonzales, Binita M. Kamath, Alastair Baker, Pamela Vig, Douglas B. Mogul, Will Garner, Bettina E. Hansen, Emmanuel Jacquemin, Richard J. Thompson
Summary: Improvement in pruritus by 48 weeks, and lower W48 bilirubin and serum bile acid levels were associated with fewer events in patients with Alagille syndrome (ALGS) treated with maralixibat (MRX).
Article
Gastroenterology & Hepatology
James E. Squires, Alexander G. Miethke, C. Alexander Valencia, Kieran Hawthorne, Lisa Henn, Johan L. K. H. Van Hove, Robert Squires, Kevin Bove, Simon Horslen, Rohit Kohli, Jean P. Molleston, Rene Romero, Estella M. Alonso, Jorge A. Bezerra, Stephen L. Guthery, Evelyn Hsu, Saul J. Karpen, Kathleen M. Loomes, Vicky L. Ng, Philip Rosenthal, Krupa Mysore, Kasper S. Wang, Marisa W. Friederich, John C. Magee, Ronald J. Sokol
Summary: This study aims to enhance the understanding of pathogenesis and natural history of mitochondrial hepatopathies (MH), a range of liver-based conditions caused by alterations in mitochondrial DNA and nuclear DNA genes. The study found differences in clinical presentation, genetic etiology, and survival between acute liver failure (ALF) and chronic liver disease (Chronic) phenotypes of MH. In addition, a subset of patients could not be identified with a specific genetic or enzymatic diagnosis, highlighting the need for further research.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Gastroenterology & Hepatology
Swati Ghosh, Michael W. Devereaux, Aimee L. Anderson, Karim C. El Kasmi, Ronald J. Sokol
Summary: The study showed that FXR activation through IL-6-STAT3 signaling in a PNAC mouse model alleviated cholestatic liver injury and prevented cholestasis.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Gastroenterology & Hepatology
Antonia Felzen, Daan B. E. van Wessel, Emmanuel Gonzales, Richard J. Thompson, Irena Jankowska, Benjamin L. Shneider, Etienne Sokal, Tassos Grammatikopoulos, Agustina Kadaristiana, Emmanuel Jacquemin, Anne Spraul, Patryk Lipinski, Piotr Czubkowski, Nathalie Rock, Mohammad Shagrani, Dieter Broering, Emanuele Nicastro, Deirdre Kelly, Gabriella Nebbia, Henrik Arnell, Bjoern Fischler, Jan B. F. Hulscher, Daniele Serranti, Cigdem Arikan, Esra Polat, Dominique Debray, Florence Lacaille, Cristina Goncalves, Loreto Hierro, Gema Munoz Bartolo, Yael Mozer-Glassberg, Amer Azaz, Jernej Brecelj, Antal Dezsofi, Pier Luigi Calvo, Enke Grabhorn, Steffen Hartleif, Wendy J. van der Woerd, Binita M. Kamath, Jian-She Wang, Liting Li, Ozlem Durmaz, Nanda Kerkar, Marianne Horby Jorgensen, Ryan Fischer, Carolina Jimenez-Rivera, Seema Alam, Mara Cananzi, Noemie Laverdure, Cristina Targa Ferreira, Felipe Ordonez Guerrero, Heng Wang, Valerie Sency, Kyung Mo Kim, Huey-Ling Chen, Elisa de Carvalho, Alexandre Fabre, Jesus Quintero Bernabeu, Aglaia Zellos, Estella M. Alonso, Ronald J. Sokol, Frederick J. Suchy, Kathleen M. Loomes, Patrick J. McKiernan, Philip Rosenthal, Yumirle Turmelle, Simon Horslen, Kathleen Schwarz, Jorge A. Bezerra, Kasper Wang, Bettina E. Hansen, Henkjan J. Verkade
Summary: This study aimed to evaluate the relationship between genetic mutations related to BSEP deficiency and clinical features. The results showed that the combination of one relatively mild mutation and one severe mutation resulted in a clinical presentation similar to patients with two severe mutations, and they had a poor response to surgical interruption of the enterohepatic circulation.
Article
Gastroenterology & Hepatology
Richard J. Thompson, Reha Artan, Ulrich Baumann, Pier Luigi Calvo, Piotr Czubkowski, Buket Dalgic, Lorenzo D'Antiga, Angelo Di Giorgio, Ozlem Durmaz, Emmanuel Gonzales, Tassos Grammatikopoulos, Girish Gupte, Winita Hardikar, Roderick H. J. Houwen, Binita M. Kamath, Saul J. Karpen, Florence Lacaille, Alain Lachaux, Elke Lainka, Kathleen M. Loomes, Cara L. Mack, Jan P. Mattsson, Patrick Mckiernan, Quanhong Ni, Hasan Ozen, Sanjay R. Rajwal, Bertrand Roquelaure, Eyal Shteyer, Etienne Sokal, Ronald J. Sokol, Nisreen Soufi, Ekkehard Sturm, Mary Elizabeth Tessier, Wendy L. van der Woerd, Henkjan J. Verkade, Jennifer M. Vittorio, Terese Wallefors, Natalie Warholic, Qifeng Yu, Patrick Horn, Lise Kjems
Summary: The ongoing PEDFIC 2 study evaluated the effectiveness of odevixibat in patients with progressive familial intrahepatic cholestasis. The study found that odevixibat can reduce serum bile acids and alleviate pruritus symptoms. This suggests that odevixibat may be a safe and effective treatment option for patients with progressive familial intrahepatic cholestasis.
Article
Gastroenterology & Hepatology
Sarah Kemme, Jennifer D. Canniff, Amy G. Feldman, Krystle M. Garth, Shaobing Li, Zhaoxing Pan, Ronald J. Sokol, Adriana Weinberg, Cara L. Mack
Summary: This study aimed to investigate the relationship between cytomegalovirus (CMV) infection and the severity of disease and pretransplant outcomes in infants with biliary atresia (BA). The results showed that CMV infection was associated with worse liver injury, delayed surgery, and increased risk of pretransplant death, but had no effect on native liver survival.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Gastroenterology & Hepatology
Colin T. Shearn, Aimee L. Anderson, Colin G. Miller, Reed C. Noyd, Michael W. Devereaux, Nata Balasubramaniyan, David J. Orlicky, Edward E. Schmidt, Ronald J. Sokol
Summary: This study investigates the impact of thioredoxin reductase 1 (TrxR1) on liver inflammation and injury during cholestatic liver injury. The results show that TrxR1 expression is increased in inflammatory cells and hepatocytes surrounding fibrosis in cholestatic liver injury patients and mouse models. Inhibition of TrxR1 can alleviate inflammation, necrosis, and the expression of pro-inflammatory cytokines, and increase the expression of antioxidant proteins and bile acid transporters. Therefore, the TrxR1 signaling pathway plays an important role in regulating inflammation and bile acid homeostasis in cholestatic liver injury.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Gastroenterology & Hepatology
Alexander Chaidez, Zhaoxing Pan, Shikha S. Sundaram, Julia Boster, Mark Lovell, Ronald J. Sokol, Cara L. Mack
Summary: Vibration controlled transient elastography (Fibroscan) can be used to predict the severity of liver fibrosis and steatosis. This study investigated the correlation between FibroScan measurements and liver histologic features in pediatric nonalcoholic fatty liver disease (NAFLD). The study also assessed the predictive capacity of the FibroScan-aspartate aminotransferase (FAST) score in pediatric NAFLD.
HEPATOLOGY COMMUNICATIONS
(2022)
Article
Gastroenterology & Hepatology
Benjamin L. Shneider, Catherine A. Spino, Binita M. Kamath, John C. Magee, Rosalinda Ignacio, Suiyuan Huang, Simon P. Horslen, Jean P. Molleston, Alexander G. Miethke, Rohit Kohli, Daniel H. Leung, M. Kyle Jensen, Kathleen M. Loomes, Saul J. Karpen, Cara Mack, Philip Rosenthal, Robert H. Squires, Alastair Baker, Sanjay Rajwal, Deirdre Kelly, Ronald J. Sokol, Richard J. Thompson
Summary: Maralixibat administration is associated with significant improvement in pruritus and quality of life in children with severe cholestasis secondary to Alagille Syndrome.
HEPATOLOGY COMMUNICATIONS
(2022)