Results 41 to 50 of about 656 (241)
ALK1 controls hepatic vessel formation, angiodiversity, and angiocrine functions in hereditary hemorrhagic telangiectasia of the liver
Hepatology, EarlyView., 2022 Hepatic endothelial Alk1 signaling protects from development of vascular malformations while maintaining organ‐specific endothelial differentiation and angiocrine portmanteau of the names Wingless and Int‐1 signaling. Abstract Background and Aims
In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with ...Christian David Schmid, Victor Olsavszky, Manuel Reinhart, Vanessa Weyer, Felix A. Trogisch, Carsten Sticht, Manuel Winkler, Sina W. Kürschner, Johannes Hoffmann, Roxana Ola, Theresa Staniczek, Joerg Heineke, Beate K. Straub, Jens Mittler, Kai Schledzewski, Peter ten Dijke, Karsten Richter, Steven Dooley, Cyrill Géraud, Sergij Goerdt, Philipp‐Sebastian Koch +20 morewiley +1 more sourceGenetic predisposition to porto‐sinusoidal vascular disorder: A functional genomic‐based, multigenerational family study
Hepatology, EarlyView., 2022 A deleterious variant of FCHSD1 results in mTOR pathway overactivation and may cause porto‐sinusoidal vascular disorder (PSVD). The pedigree of the family demonstrated an autosomal dominant disease with variable expressivity. Whole‐genome sequencing and Sanger sequencing both validated the existence of the FCHSD1 variant and the heterozygosity of c ...Jingxuan Shan, André Megarbane, Aziz Chouchane, Deepak Karthik, Ramzi Temanni, Atilio Reyes Romero, Huiying Hua, Chun Pan, Xixi Chen, Murugan Subramanian, Chadi Saad, Hamdi Mbarek, Cybel Mehawej, Eliane Chouery, Sirin W. Abuaqel, Alexander Dömling, Sami Remadi, Cesar Yaghi, Pu Li, Lotfi Chouchane +19 morewiley +1 more sourceNatural 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 sourcePharmacokinetics and efficacy of oral versus intravenous mixed-micellar phylloquinone (vitamin K-1) in severe acute liver disease
, 2005 Background/Aims: In patients with severe acute liver dysfunction, i.v. phylloquinone (vitamin K-1) may be given to exclude vitamin K deficiency, rather than impaired hepatic synthesis of coagulation factors alone, as the cause of the coagulopathy ...Pereira, SP, David Rowbotham, Stephen P. Pereira, Sarah Fitt, Fitt, S, Pereira, S P, Wendon, J, Roger Williams, Williams, R, Julia Wendon, Rowbotham, D, Martin J. Shearer, Shearer, MJ, Shearer, M J +13 morecore +1 more sourceSerum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy
Hepatology, EarlyView., 2022 Serum bile acid levels predict outcomes in patients with biliary atresia who achieve normalized bilirubin levels after Kasai portoenterostomy. Abstract Background and Aims
In biliary atresia, serum bilirubin is commonly used to predict outcomes after Kasai portoenterostomy (KP).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, for Childhood Liver Disease Research Network (ChiLDReN) +22 morewiley +1 more sourceTemporal association between serious bleeding and immunization: vitamin K deficiency as main causative factor
BMC Pediatrics, 2020 Background Bleeding as an adverse event following immunization (AEFI) that is rarely reported in children, although it can be a parental concern. Bleeding episodes ranging in severity from mild to severe and defined as any external and/or internal ...Susi Susanah, Eddy Fadlyana, Meita Dhamayanti, Rodman Tarigan, Eko Fuji Ariyanto, Yunisa Pamela, Yuzar I. B. Ismoetoto, Rita Verita Sri, Monika Hasna, Kusnandi Rusmil +9 moredoaj +1 more sourcePrevention of vitamin K deficiency bleeding in breastfed infants: lessons from the Dutch and Danish biliary atresia registries. [PDF]
, 2008 Contains fulltext :
71354.pdf (Publisher’s version ) (Open Access)OBJECTIVE: Newborns routinely receive vitamin K to prevent vitamin K deficiency bleeding. The efficacy of oral vitamin K administration may be compromised in infants with de Koning, Tom J., Jorgensen, Marianne Horby, Ruud Berger, Henkjan J. Verkade, Verkade, Henkjan J.; id_orcid, Peeters, P.M., Verkade, H.J., van den Neucker, A.M., Heurn, L.W.E. van, Vries, E. De, Aronson, D.C., Elsemieke de Vries, Koning, T.J., Zee, D.C. van der, Berger, R., van Hasselt, Peter M., Langen, Z.J. de, de Koning, T.J., Houwen, R.H., van Hasselt, P.M., Houwen, R.H.J., Nina Kvist, Kvist, Nina Eva, Peter M. van Hasselt, Escher, J.H., Verkade, Henkjan J., Koning, T.J. de, Kvist, Nina, Hasselt, P.M. van, Madern, G.C., Neucker, A.M., Kimpen, J.L., Kvist, N., Berger, Ruud, van Heurn, L.W.E., Kneepkens, C.M., Kimpen, Jan L. L., Kimpen, J.L.L., Jan L. L. Kimpen, Lundin, C.R., Rieu, P.N.M.A., Christina Rydahl Lundin, Roderick H. J. Houwen, de Vries, E., Tolboom, J.J.M., Lundin, Christina Rydahl, Jorgensen, M.H., de Vries, Elsemieke, Marianne Horby Jorgensen, Houwen, Roderick H. J., Tom J. de Koning, Kindermann, A. +51 morecore +1 more source