Article
Medicine, General & Internal
Fuat H. Saner, Alexandra Frey, Bjoern-Ole Stueben, Dieter P. Hoyer, Katharina Willuweit, Martina Daniel, Jassin Rashidi-Alavieh, Jurgen W. Treckmann, Hartmut H. Schmidt
Summary: In this retrospective study of 82 patients who underwent liver transplantation for primary sclerosing cholangitis (PSC), the authors assessed morbidity, survival rates, PSC recurrence, and the impact of donor characteristics in long-term follow-up. The study showed that PSC patients had excellent 10-year survival rates and a lower lab-MELD score was associated with longer survival. Donor characteristics did not affect patient survival.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Gastroenterology & Hepatology
Areej Mazhar, Mark W. Russo
Summary: This systematic review analyzed 40 studies on prognostic tests for PSC, comprising a total of 16,094 subjects. Non-invasive tests were associated with transplant-free survival and reduced risk of hepatobiliary cancers, but the consistency of cut-off values for alkaline phosphatase varied among studies. The Mayo Risk Score (MRS) is the most studied prognostic model, although the UK-PSC score and PREsTo showed better test performance. Further studies are needed to compare these models.
ALIMENTARY PHARMACOLOGY & THERAPEUTICS
(2021)
Article
Gastroenterology & Hepatology
Atsushi Oyama, Akinobu Takaki, Takuya Adachi, Nozomu Wada, Yasuto Takeuchi, Hideki Onishi, Hidenori Shiraha, Hiroyuki Okada, Motoyuki Otsuka
Summary: This study aimed to investigate the clinical course and oxidative stress-related condition of primary sclerosing cholangitis (PSC) to determine prognostic factors. The Mayo risk, Child-Pugh, MELD-Na scores, and FIB-4 index were significantly correlated with overall survival. Among patients with high and intermediate Mayo risk scores, physiologically high oxidative stress status was associated with low IgA levels and a good prognosis.
HEPATOLOGY INTERNATIONAL
(2023)
Article
Nutrition & Dietetics
Maryam Ebadi, Elora Rider, Catherine Tsai, Sarah Wang, Ellina Lytvyak, Andrew Mason, Aldo J. Montano-Loza
Summary: This study aimed to investigate the association between severe vitamin D deficiency and disease progression, hepatobiliary malignancies, liver-related mortality, and the need for liver transplantation in primary sclerosing cholangitis (PSC). The results showed that severe vitamin D deficiency was related to an increased risk of hepatobiliary malignancies, and a severe deficiency at diagnosis and persistent deficiencies over time were independently associated with poor clinical liver outcomes.
Review
Medicine, General & Internal
Paul A. Schmeltzer, Mark W. Russo
Summary: This study systematically reviewed 37 studies comparing various non-invasive biochemical prognostic models to the revised Mayo Risk Score (rMRS) in predicting outcomes in patients with Primary sclerosing cholangitis (PSC). The results showed that newer prognostic models, such as the UK-PSC score and PREsTo, were more accurate at predicting clinical endpoints in PSC compared to the rMRS. Time frames and clinical endpoints varied among the studies.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Gastroenterology & Hepatology
Damjana Bogatic, Robert Bryant, Kate D. Lynch, Samuel P. Costello
Summary: Primary sclerosing cholangitis (PSC) is a progressive liver disease with poor prognosis and no effective therapies to prevent progression. This study evaluated potential medical therapies that may exert their effects in PSC by modulation of the gut-liver axis.
ALIMENTARY PHARMACOLOGY & THERAPEUTICS
(2023)
Article
Gastroenterology & Hepatology
Mercedes Martinez, Emily R. Perito, Pamela Valentino, Cara L. Mack, Madeleine Aumar, Annemarie Broderick, Laura G. Draijer, Eleonora D. T. Fagundes, Katryn N. Furuya, Nitika Gupta, Simon Horslen, Maureen M. Jonas, Binita M. Kamath, Nanda Kerkar, Kyung Mo Kim, Kaija-Leena Kolho, Bart G. P. Koot, Trevor J. Laborda, Christine K. Lee, Kathleen M. Loomes, Tamir Miloh, Douglas Mogul, Saeed Mohammed, Nadia Ovchinsky, Girish Rao, Amanda Ricciuto, Alexandre Rodrigues Ferreira, Kathleen B. Schwarz, Vratislav Smolka, Atsushi Tanaka, Mary E. M. Tessier, Venna L. Venkat, Bernadette E. Vitola, Marek Woynarowski, Melissa Zerofsky, Mark R. Deneau
Summary: This retrospective study evaluated risk factors for rPSC in 140 children and found that patients with rPSC were younger at LT, had faster progression from PSC diagnosis to LT, and had a higher prevalence of inflammatory bowel disease. rPSC subjects experienced more episodes of acute rejection, complications of portal hypertension, and higher mortality rates post-LT.
Article
Surgery
Marko J. Vannas, Fredrik Aberg, Arno Nordin, Eija Tukiainen, Johanna Savikko, Helena Isoniemi
Summary: The objective of this study was to investigate the short-term and long-term morbidity after liver transplantation in patients with primary sclerosing cholangitis. The results showed that a 1-year Comprehensive Complication Index (CCI) cutoff of 42 could significantly predict post-transplant survival. Factors such as cholangitis, infections, hypertension, Model for End-stage Liver Disease scores, and body mass indices were found to be associated with high CCI.
Article
Immunology
Ramon Cortez, Luana N. Moreira, Marina Padilha, Mariana D. Bibas, Ricardo K. Toma, Gilda Porta, Carla R. Taddei
Summary: Studies have shown that the composition of intestinal microbiome in pediatric patients with primary sclerosing cholangitis (PSC) is influenced by disease, age, and disease activity status, highlighting significant differences in microbiome between PSC patients. Specific genera Veillonella and Megasphaera have increased relative abundance depending on age at diagnosis.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Gastroenterology & Hepatology
Natassia Tan, N. Ngu, T. Worland, T. Lee, T. Abrahams, K. Pandya, E. Freeman, N. Hannah, K. Gazelakis, R. G. Madden, K. D. Lynch, Z. Valaydon, S. Sood, A. Dev, S. Bell, A. Thompson, J. Ding, A. J. Nicoll, K. Liu, P. Gow, J. Lubel, W. Kemp, S. K. Roberts, A. Majeed
Summary: This study evaluated the epidemiology and clinical outcomes of primary sclerosing cholangitis (PSC) in Australia and compared them to the general population. The results showed that PSC patients had a higher risk of developing cirrhosis and death, especially from cholangiocarcinoma. Age and time from diagnosis were closely associated with disease severity and prognosis.
HEPATOLOGY INTERNATIONAL
(2022)
Article
Gastroenterology & Hepatology
Mikal Jacob Hole, Kristin Kaasen Jorgensen, Kristian Holm, Peder R. Braadland, Malin Holm Meyer-Myklestad, Asle Wilhelm Medhus, Dag Henrik Reikvam, Alexandra Gotz, Krzysztof Grzyb, Kirsten Muri Boberg, Tom Hemming Karlsen, Martin Kummen, Johannes R. Hov
Summary: This study investigated the characteristics of the gut microbiota in patients with primary sclerosing cholangitis (PSC) and found several features associated with PSC, including an expansion of Proteobacteria and increased variability. Furthermore, certain genera and the presence of the pathobiont Klebsiella were associated with reduced survival in PSC patients, and concomitant inflammatory bowel disease (IBD) was associated with reduced levels of Akkermansia.
Article
Gastroenterology & Hepatology
Aristeidis Grigoriadis, Kristina Imeen Ringe, Johan Bengtsson, Erik Baubeta, Cecilia Forsman, Nafsika Korsavidou-Hult, Fredrik Rorsman, Emma Nilsson, Nikolaos Kartalis, Annika Bergquist
Summary: Magnetic resonance cholangiopancreatography (MRCP) plays an important role in the prognosis assessment of primary sclerosing cholangitis (PSC). A novel, simple, and reproducible risk-score (DiStrict score) based on MRCP findings was developed and showed a strong association with prognosis in individuals with PSC. This score can be easily used in clinical practice and has the potential to be useful in clinical trials and patient counseling and management.
Review
Gastroenterology & Hepatology
David Tornai, Peter Laszlo Ven, Peter Laszlo Lakatos, Maria Papp
Summary: The clinical manifestations and progression of primary sclerosing cholangitis (PSC) vary, and its pathogenesis is not well understood. Gut-liver interactions play an important role in the development of PSC. Recent advances in biomarkers research may help identify clinically relevant PSC subgroups and assist in diagnosis, disease stratification, and potential therapeutic targets.
WORLD JOURNAL OF GASTROENTEROLOGY
(2022)
Article
Gastroenterology & Hepatology
Christina Villard, Ingalill Friis-Liby, Fredrik Rorsman, Karouk Said, Anna Warnqvist, Martin Cornillet, Stergios Kechagias, Nils Nyhlin, Marten Werner, Izabella Janczewska, Therese Hagstrom, Emma Nilsson, Annika Bergquist
Summary: This study prospectively evaluated the effectiveness of yearly MRI/MRCP surveillance in detecting cholangiocarcinoma (CCA) in patients with primary sclerosing cholangitis (PSC) and found it to be ineffective in detecting cancer early enough to support long-term survival. Personalized follow-up strategies and improved diagnostic methods for PSC-related CCA are needed.
JOURNAL OF HEPATOLOGY
(2023)
Article
Pathology
Laura Valestrand, Fei Zheng, Simen H. Hansen, Jonas Ogaard, Johannes R. Hov, Niklas K. Bjorkstrom, Tom H. Karlsen, Xiaojun Jiang, Espen Melum
Summary: This study found that bile from patients with PSC may contain antigens that activate MAIT cells. Data from sequencing analysis suggested a higher abundance of ribD gene expression in bile samples that could activate MAIT cells. Furthermore, it was observed that cholangiocytes could take up and present biliary antigens to MAIT cells. These findings reveal a pathophysiological pathway connecting the immune system and the microbiome in PSC.
AMERICAN JOURNAL OF PATHOLOGY
(2022)
Article
Gastroenterology & Hepatology
Catharine M. Walsh, Jenifer R. Lightdale, Douglas S. Fishman, Raoul Furlano, Petar Mamula, Peter M. Gillett, Priya Narula, Iva Hojsak, Salvatore Oliva, Matjaz Homan, Matthew R. Riley, Hien Q. Huynh, Joel R. Rosh, Kevan Jacobson, Marta Tavares, Ian H. Leibowitz, Elizabeth C. Utterson, Nicholas M. Croft, David R. Mack, Herbert Brill, Quin Y. Liu, Patrick Bontems, Diana G. Lerner, Jorge Amil-Dias, Robert E. Kramer, Anthony R. Otley, Lusine Ambartsumyan, Veronik Connan, Graham A. McCreath, Mike A. Thomson
Summary: This study aims to identify the standardized elements for high-quality pediatric endoscopy reports and obtain international consensus. Consensus was achieved through the Delphi method and online voting, resulting in the identification of 30 essential reporting elements.
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2022)
Editorial Material
Gastroenterology & Hepatology
Amanda Ricciuto, Ingrid Rauter, Dermot P. B. Mcgovern, Robert M. Mader, Walter Reinisch
Article
Gastroenterology & Hepatology
Christopher L. Bowlus, Lionel Arrive, Annika Bergquist, Mark Deneau, Lisa Forman, Sumera I. Ilyas, Keri E. Lunsford, Mercedes Martinez, Gonzalo Sapisochin, Rachna Shroff, James H. Tabibian, David N. Assis
Article
Infectious Diseases
Thu Giang Le Thi, Katharina Werkstetter, Kallirroi Kotilea, Patrick Bontems, Jose Cabral, Maria Luz Cilleruelo Pascual, Michal Kori, Josefa Barrio, Matjaz Homan, Nicolas Kalach, Rosa Lima, Marta Tavares, Pedro Urruzuno, Zrinjka Misak, Vaidotas Urbonas, Sibylle Koletzko
Summary: The EuroPedHp-registry aims to monitor the management, antibiotic resistance, and eradication success of tailored triple therapy for Helicobacter pylori-infected children. The study found that guideline-conform 2-week therapy achieved primary eradication of >= 90%, but higher failure rates were observed in single-resistant strains.
Article
Pediatrics
Lisa M. McElroy, Abigail E. Martin, Amy G. Feldman, Vicky L. Ng, Tomoaki Kato, Trevor Reichman, Pamela L. Valentino, Ravinder Anand, Sarah G. Anderson, Debra L. Sudan
Summary: This study compares patient and graft survival between whole liver grafts (WLG) and split liver grafts from living donors (TVG-LD) or deceased donors (TVG-DD) in a pediatric liver transplant cohort. The results show no significant differences in survival rates between the different graft types. Biliary complications were more common in TVG recipients, while hepatic artery thrombosis was more common in WLG recipients. The primary diagnosis was found to be the most significant predictor of patient survival, and the development of biliary and vascular complications over time predicted graft survival.
PEDIATRIC TRANSPLANTATION
(2023)
Editorial Material
Clinical Neurology
Fabiola Di Dato, Raffaele Iorio
PARKINSONISM & RELATED DISORDERS
(2023)
Article
Gastroenterology & Hepatology
Brian G. Feagan, Bruce E. Sands, William J. Sandborn, Matthew Germinaro, Marion Vetter, Jie Shao, Shihong Sheng, Jewel Johanns, Julian Panes
Summary: This study compared the efficacy of combination therapy with guselkumab and golimumab to monotherapy for ulcerative colitis, and found that combination therapy achieved a higher clinical response rate at 12 weeks. However, these findings need to be confirmed in larger trials.
LANCET GASTROENTEROLOGY & HEPATOLOGY
(2023)
Article
Gastroenterology & Hepatology
Amanda Ricciuto, Binita M. Kamath, Gideon M. Hirschfield, Palak J. Trivedi
Summary: Autoimmune liver diseases are classified into three syndromes, but they are challenging due to variant presentations and lack of definable disease aetiologies. This article argues that "autoimmune sclerosing cholangitis" and "PSC/AIH-overlap" in young patients represent inflammatory phases of primary sclerosing cholangitis and should be considered as the same entity to facilitate unified care and collaborative studies.
JOURNAL OF HEPATOLOGY
(2023)
Article
Nutrition & Dietetics
Vikram K. K. Raghu, Scott D. D. Rothenberger, Jeffrey A. A. Rudolph, George V. V. Mazariegos, Simon P. P. Horslen, Kenneth J. J. Smith
Summary: This study evaluated the costs and length of stay in children who underwent intestine transplant. It found that intestine transplant has high immediate costs and long length of stay, which vary based on center, graft type, and immunosuppression regimen.
JOURNAL OF PARENTERAL AND ENTERAL NUTRITION
(2023)
Article
Gastroenterology & Hepatology
A. A. Nugud, Christos Tzivinikos, Amit Assa, Osvaldo Borrelli, Ilse Broekaert, Javier Martin-de-Carpi, Marco Deganello Saccomani, Jernej Dolinsek, M. Homan, Emmanuel Mas, Erasmo Miele, Mike A. Thomson, Marc Benninga, Gastrointestinal Comm ESPGHAN
Summary: Magnet ingestion is a serious medical emergency worldwide, especially when multiple magnets or other metallic foreign bodies are ingested. Ingestion of earth magnets, which have a stronger magnetic force, can have worse outcomes. Legislative bodies, scientific societies, and community-led initiatives have been implemented globally to address this issue. This study provides a literature review on the epidemiology, diagnosis, management, and prevention of magnet ingestion and presents an algorithm for its diagnosis and management, focusing on identifying staggered/multiple magnet ingestion and early consultation with gastroenterologists and surgeons.
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2023)
Article
Gastroenterology & Hepatology
Oren Ledder, Marek Woynarowski, Diana Kaminska, Izabella Lazowska-Przeorek, Stanislaw Pieczarkowski, Claudio Romano, Raffi Lev-Tzion, Magdalena Holon, Andreia Nita, Anna Rybak, Elzbieta Jarocka-Cyrta, Bartosz Korczowski, Elzbieta Czkwianianc, Iva Hojsak, Anna Szaflarska-Poplawska, Bruno Hauser, Isabelle Scheers, Shishu Sharma, Salvatore Oliva, Raoul Furlano, Christos Tzivinikos, Quin Y. Liu, Matthew Giefer, Petar Mamula, Andrew Grossman, Judith Kelsen, Brian Edelstein, Matthieu Antoine, Mike Thomson, Matjaz Homan
Summary: This study analyzes data from centers in Europe, North America, and the Middle East to investigate pediatric gastrointestinal endoscopy-related viscus perforation. The study aims to characterize the clinical presentation of these perforations to assist in developing post-procedure recommendations for timely management.
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2023)
Article
Cardiac & Cardiovascular Systems
Alexis Gumm, Salil Ginde, George Hoffman, Melodee Liegl, Cara Mack, Pippa Simpson, Nghia Vo, Grzegorz Telega, Bernadette Vitola, Ankur Chugh
Summary: High-intensity exercise does not cause acute liver injury in patients with Fontan physiology, although it may lead to a reduction in hepatic tissue oxygenation.
PEDIATRIC CARDIOLOGY
(2023)
Editorial Material
Gastroenterology & Hepatology
Raffaele Iorio, Fabiola Di Dato, Maria Immacolata Spagnuolo
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2023)
Article
Virology
Yasmina Sirgi, Maja Stanojevic, Jaeil Ahn, Nada Yazigi, Stuart Kaufman, Khalid Khan, Bernadette Vitola, Cal Matsumoto, Alexander Kroemer, Thomas Fishbein, Udeme D. Ekong
Summary: Planning for future resurgences in SARS-CoV-2 infection is necessary for providers who care for immunocompromised patients. This study aimed to determine factors associated with COVID-19 disease severity in immunosuppressed children. The findings showed that a high absolute lymphocyte count at diagnosis decreased the odds of having severe COVID-19 disease, and a high absolute monocyte count in the months preceding infection increased the odds of having severe disease. These observations may have important ramifications in resource management.
Editorial Material
Pediatrics
Raffaele Iorio, Fabiola Di Dato, Maria Immacolata Spagnuolo
LANCET CHILD & ADOLESCENT HEALTH
(2023)