Results 101 to 110 of about 58,413 (245)
Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study
Hepatology, EarlyView., 2022 Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study. Abstract Background and Aims
Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, and real‐world data are lacking.Shannon M. Vandriel, Li‐Ting Li, Huiyu She, Jian‐She Wang, Melissa A. Gilbert, Irena Jankowska, Piotr Czubkowski, Dorota Gliwicz‐Miedzińska, Emmanuel M. Gonzales, Emmanuel Jacquemin, Jérôme Bouligand, Nancy B. Spinner, Kathleen M. Loomes, David A. Piccoli, Lorenzo D'Antiga, Emanuele Nicastro, Étienne Sokal, Tanguy Demaret, Noelle H. Ebel, Jeffrey A. Feinstein, Rima Fawaz, Silvia Nastasio, Florence Lacaille, Dominique Debray, Henrik Arnell, Björn Fischler, Susan Siew, Michael Stormon, Saul J. Karpen, Rene Romero, Kyung Mo Kim, Woo Yim Baek, Winita Hardikar, Sahana Shankar, Amin J. Roberts, Helen M. Evans, M. Kyle Jensen, Marianne Kavan, Shikha S. Sundaram, Alexander Chaidez, Palaniswamy Karthikeyan, Maria Camila Sanchez, Maria Lorena Cavalieri, Henkjan J. Verkade, Way Seah Lee, James E. Squires, Christina Hajinicolaou, Chatmanee Lertudomphonwanit, Ryan T. Fischer, Catherine Larson‐Nath, Yael Mozer‐Glassberg, Cigdem Arikan, Henry C. Lin, Jesus Quintero Bernabeu, Seema Alam, Deirdre A. Kelly, Elisa Carvalho, Cristina Targa Ferreira, Giuseppe Indolfi, Ruben E. Quiros‐Tejeira, Pinar Bulut, Pier Luigi Calvo, Zerrin Önal, Pamela L. Valentino, Dev M. Desai, John Eshun, Maria Rogalidou, Antal Dezsőfi, Sabina Wiecek, Gabriella Nebbia, Raquel Borges Pinto, Victorien M. Wolters, María Legarda Tamara, Andréanne N. Zizzo, Jennifer Garcia, Kathleen Schwarz, Marisa Beretta, Thomas Damgaard Sandahl, Carolina Jimenez‐Rivera, Nanda Kerkar, Jernej Brecelj, Quais Mujawar, Nathalie Rock, Cristina Molera Busoms, Wikrom Karnsakul, Eberhard Lurz, Ermelinda Santos‐Silva, Niviann Blondet, Luis Bujanda, Uzma Shah, Richard J. Thompson, Bettina E. Hansen, Binita M. Kamath, The Global ALagille Alliance (GALA) Study Group +93 morewiley +1 more sourceThe Age at Onset of LRRK2 p.Gly2019Ser Parkinson's Disease Across Ancestries and Countries of Origin
Annals of Neurology, EarlyView.Objectives
The objective of this study was to elucidate differences in the cumulative incidence of Leucine‐rich repeat kinase 2 (LRRK2) p.Gly2019Ser‐related Parkinson's disease (PD; LRRK2‐PD) between ancestries and countries. Methods
We included 922 unrelated p.Gly2019Ser variant carriers (affected = 762 and unaffected = 160) from the Global Parkinson ...Theresa Lüth, Björn‐Hergen Laabs, Sebastian Sendel, Inke R. König, Amke Caliebe, Alastair J. Noyce, Laurel A. Screven, Soraya Bardien, Matthew Farrer, Faycel Hentati, Rim Amouri, Christine Klein, Samia Ben Sassi, Joanne Trinh, Global Parkinson's Genetics Program (GP2) +14 morewiley +1 more sourceA shared mucosal gut microbiota signature in primary sclerosing cholangitis before and after liver transplantation
Hepatology, EarlyView., 2022 In this study of the gut mucosal microbiota in primary sclerosing cholangitis (PSC), we found consistent microbiota features associated with PSC and recurrent PSC, PSC with inflammatory bowel disease and a persistent gut dysbiosis after liver transplantation Abstract Background and Aims
Several characteristic features of the fecal microbiota have been ...Mikal Jacob Hole, Kristin Kaasen Jørgensen, Kristian Holm, Peder R. Braadland, Malin Holm Meyer‐Myklestad, Asle Wilhelm Medhus, Dag Henrik Reikvam, Alexandra Götz, Krzysztof Grzyb, Kirsten Muri Boberg, Tom Hemming Karlsen, Martin Kummen, Johannes R. Hov +12 morewiley +1 more sourceBlood Lactate as a Prognostic Biomarker for Survival and Weight Loss in Amyotrophic Lateral Sclerosis: An Exploratory–Validation Study
Annals of Neurology, EarlyView.Objective
Lactate is increasingly recognized as an energy substrate, but its relevance to amyotrophic lateral sclerosis (ALS) remains unclear. We examined whether blood lactate is associated with survival and weight loss in ALS. Methods
This retrospective study included an Australian exploratory cohort and a Japanese validation cohort with clinical ...Ryutaro Nakamura, Frederik J. Steyn, Shuhei Kobashi, Nobuhiro Ogawa, Akihiro Kitamura, Isamu Yamakawa, Robert D. Henderson, Shyuan T. Ngo, Makoto Urushitani +8 morewiley +1 more sourceTemporal Modeling of Amyloid and Tau Trajectories in Alzheimer's Disease Using PET and Plasma Biomarkers
Annals of Neurology, EarlyView.Objective
This study aimed to compare positron emission tomography (PET) and plasma‐based temporal modeling of amyloid and tau biomarkers in Alzheimer's disease. Methods
Longitudinal amyloid PET (n = 1,097, mean age ± SD = 72.5 ± 7.38 year, 51.4% male), 18F‐flortaucipir tau‐PET (n = 230, 74.3 ± 7.18 year, 52.2% female), and Fujirebio Lumipulse plasma p‐Christopher A. Brown, Katheryn A.Q. Cousins, Magdalena Korecka, Emily McGrew, Alice Chen‐Plotkin, John A. Detre, Corey T. McMillan, Edward B. Lee, Sandhitsu R. Das, Dawn Mechanic‐Hamilton, Paul A. Yushkevich, Ilya M. Nasrallah, Leslie M. Shaw, for the Alzheimer's Disease Neuroimaging Initiative, David A. Wolk +14 morewiley +1 more sourceHigh Prevalence of SOD1 Pathogenic Variants in the UK Biobank: Implications for Early Intervention in Amyotrophic Lateral Sclerosis
Annals of Neurology, EarlyView.Objective
SOD1 is the second most frequently mutated gene in European patients with amyotrophic lateral sclerosis (ALS). Given the recent authorization of SOD1‐targeted antisense oligonucleotides for SOD1‐ALS, prompt screening for SOD1 mutations in patients with ALS patients is highly recommended.Delia Gagliardi, Chiara Villella, Matteo Zanovello, Virginia Iacobelli, Stefania Corti, Giacomo Pietro Comi, Pietro Fratta, Henry Houlden, Arianna Tucci, Dario Ronchi +9 morewiley +1 more sourceThe Dynamics of Neurofilament Light Chain in Spinal Muscular Atrophy
Annals of Neurology, EarlyView.Objective
Newborn screening (NBS) for spinal muscular atrophy (SMA) facilitates early diagnosis and treatment for affected individuals. However, fluid biomarkers that provide early insights into disease activity and outcomes in a neonatal cohort and those unable to access (due to reimbursement criteria) or deferring immediate treatment are lacking ...Arlene D'Silva, Karen Herbert, Lakshmi Balaji, Jia Mei He, Tejaswi Kandula, Hugo A. Sampaio, Hooi‐Ling Teoh, Esther Tantsis, Jihee Sohn, Nancy Briggs, Nickson Ning, Matthew C. Kiernan, Didu S. Kariyawasam, Michelle A. Farrar +13 morewiley +1 more sourceRecurrent Genomic Alterations in BCRi‐Experienced CLL Patients Treated With Venetoclax: Extended Phase 2 Follow‐Up
American Journal of Hematology, EarlyView.Jennifer A. Woyach, Relja Popovic, Emily L. Rossi, Sanjana Singh, Toshihiko Oki, Tanya S. Rosenberg, Chen Qian, Michael Moran, Zihuan Liu, Michelle Boyer, Jeffrey Jones, Richard R. Furman, William Wierda, Jason Gotlib, Stephen J. Schuster, Brenda Chyla, Matthew S. Davids +16 morewiley +1 more sourceCerebrospinal Fluid Amyloid‐β Biomarkers Predict Future Hemorrhage in Patients with Cerebral Amyloid Angiopathy
Annals of Neurology, EarlyView.Objective
Accurately predicting future hemorrhagic events in patients with cerebral amyloid angiopathy (CAA) remains a major clinical challenge. It is unknown whether cerebrospinal fluid (CSF) biomarkers of amyloid‐beta (Aβ) pathology are associated with increased hemorrhage risk in this population.Philipp Arndt, Malte Pfister, Valentina Perosa, Hendrik Mattern, Melis Tas, Lara Holzheimer, Johanna Engel, Marc Dörner, Marwa Al‐Dubai, Cornelia Garz, Eya Khadhraoui, Sebastian J. Müller, Patrick Müller, Sven G. Meuth, Katja Neumann, Stefanie Schreiber +15 morewiley +1 more sourceSURVIVAL ANALYSIS AND LENGTH-BIASED SAMPLING
Croatian Operational Research Review, 2010 When survival data are colleted as part of a prevalent cohort study, the recruited cases have already experienced their initiating event. These prevalent cases are then followed for a fixed period of time at the end of which the subjects will either have Masoud Asghariandoaj
