Results 11 to 20 of about 5,925 (200)

Extrahepatic manifestations of progressive familial intrahepatic cholestasis syndromes: Presentation of a case series and literature review

Liver International, Volume 42, Issue 5, Page 1084-1096, May 2022., 2022
Abstract Background and Aims Progressive familial intrahepatic cholestasis (PFIC) is a collective term for a heterogenous group of rare, inherited cholestasis syndromes. The number of genes underlying the clinical PFIC phenotype is still increasing.
Eva‐Doreen Pfister, Carola Dröge, Roman Liebe, Amelie Stalke, Nicole Buhl, Antje Ballauff, Tobias Cantz, Eva Bueltmann, Jan Stindt, Tom Luedde, Ulrich Baumann, Verena Keitel   +11 more
wiley   +1 more source

Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency

Hepatology, Volume 74, Issue 2, Page 892-906, August 2021., 2021
Background and Aims Mutations in ATPase phospholipid transporting 8B1 (ATP8B1) can lead to familial intrahepatic cholestasis type 1 (FIC1) deficiency, or progressive familial intrahepatic cholestasis type 1. The rarity of FIC1 deficiency has largely prevented a detailed analysis of its natural history, effects of predicted protein truncating mutations (
Daan B.E. van Wessel, Richard J. Thompson, Emmanuel Gonzales, Irena Jankowska, Benjamin L. Shneider, Etienne Sokal, Tassos Grammatikopoulos, Agustina Kadaristiana, Emmanuel Jacquemin, Anne Spraul, Patryk Lipiński, Piotr Czubkowski, Nathalie Rock, Mohammad Shagrani, Dieter Broering, Talal Algoufi, Nejat Mazhar, Emanuele Nicastro, Deirdre Kelly, Gabriella Nebbia, Henrik Arnell, Björn Fischler, Jan B.F. Hulscher, Daniele Serranti, Cigdem Arikan, Dominique Debray, Florence Lacaille, Cristina Goncalves, Loreto Hierro, Gema Muñoz Bartolo, Yael Mozer‐Glassberg, Amer Azaz, Jernej Brecelj, Antal Dezsőfi, Pier Luigi Calvo, Dorothee Krebs‐Schmitt, Steffen Hartleif, Wendy L. van der Woerd, Jian‐She Wang, Li‐ting Li, Özlem Durmaz, Nanda Kerkar, Marianne Hørby Jørgensen, Ryan Fischer, Carolina Jimenez‐Rivera, Seema Alam, Mara Cananzi, Noémie Laverdure, Cristina Targa Ferreira, Felipe Ordonez, Heng Wang, Valerie Sency, Kyung Mo Kim, Huey‐Ling Chen, Elisa Carvalho, Alexandre Fabre, Jesus Quintero Bernabeu, Estella M. Alonso, Ronald J. Sokol, Frederick J. Suchy, Kathleen M. Loomes, Patrick J. McKiernan, Philip Rosenthal, Yumirle Turmelle, Girish S. Rao, Simon Horslen, Binita M. Kamath, Maria Rogalidou, Wikrom W. Karnsakul, Bettina Hansen, Henkjan J. Verkade, on behalf of Natural Course and Prognosis of PFIC and Effect of Biliary Diversion Consortium   +71 more
wiley   +1 more source

Systematic Review and Meta‐analysis

Journal of Pediatric Gastroenterology and Nutrition, Volume 71, Issue 2, Page 176-183, August 2020., 2020
ABSTRACT Objectives: We assessed available data on impact of partial external biliary diversion (PEBD) surgery on clinical outcomes in patients with progressive familial intrahepatic cholestasis (PFIC). Methods: We performed a systematic literature review (PubMed) and meta‐analysis to evaluate relationships between liver biochemistry parameters (serum ...
Henkjan J. Verkade, Richard J. Thompson, Henrik Arnell, Björn Fischler, Per‐Göran Gillberg, Jan P. Mattsson, Kristina Torfgård, Erik Lindström   +7 more
wiley   +1 more source

Potential of ileal bile acid transporter inhibition as a therapeutic target in Alagille syndrome and progressive familial intrahepatic cholestasis

Liver International, Volume 40, Issue 8, Page 1812-1822, August 2020., 2020
Abstract Alagille syndrome (ALGS) and progressive familial intrahepatic cholestasis (PFIC) are rare, inherited cholestatic liver disorders that manifest in infants and children and are associated with impaired bile flow (ie cholestasis), pruritus and potentially fatal liver disease.
Binita M. Kamath, Philip Stein, Roderick H. J. Houwen, Henkjan J. Verkade   +3 more
wiley   +1 more source

CRAQUEAMENTO TERMOCATALÍTICO DE POLIETILENO (PEBD E PEAD) EM ESCALA DE LABORATÓRIO [PDF]

Anais do XX Congresso Brasileiro de Engenharia Química, 2015
RESUMO – A ampla utilizacao dos plasticos gera uma grande quantidade de residuos que devem ser descartados ou, preferencialmente, reciclados de forma adequada. O presente trabalho investigou, em escala de laboratorio, o processo de craqueamento de polietileno de baixa densidade (PEBD) virgem e de polietileno de alta densidade (PEAD) proveniente de ...
M. S. PEREIRA, L. E. P. BORGES, N. T. MACHADO   +2 more
openaire   +1 more source

Efeito da modificação atmosférica em goiabas var. Paluma na redução de danos mecânicos em pós-colheita

Agro@mbiente On-line, 2011
No manejo pós-colheita, os frutos podem estar expostos a injúrias que depreciam sua qualidade e vida útil. Assim, avaliou-se o efeito da atmosfera modificada em goiabas var. Paluma submetidas a diferentes tipos de danos mecânicos.
Maria Luiza Grigio, Leandro Camargo Neves, Jessica Milanez Tosin, Cássia Rejane Nascimento, Edvan Alves Chagas, Rogério Lopes Vieites   +5 more
doaj   +1 more source

Radiação gama e atmosfera modificada passiva na qualidade de goiabas 'Pedro Sato'

Revista Brasileira de Fruticultura, 2011
O objetivo deste trabalho foi avaliar o efeito da irradiação gama associada à atmosfera modificada passiva na qualidade pós-colheita de goiabas 'Pedro Sato', verificando suas características físico-químicas.
André José de Campos, Érika Fujita, Leandro Camargo Neves, Rogério Lopes Vieites, Edvan Alves Chagas   +4 more
doaj   +1 more source

Recyclable and non-recyclable packaging films with different barrier properties: Effect of processing and storage time on quality of mashed potato and ground carrot. [PDF]

J Food Sci
Abstract The aim of this study was to evaluate if high barrier recyclable material polyethylene/ethylene vinyl alcohol (PE/EVOH) can be an alternative non‐recyclable polyamides (PA)/PE laminate and also if high barrier is required or recyclable PE material with low barrier properties is good enough to maintain the quality of thermally processed mashed ...
Sharmin N, Rotabakk BT, Grøvlen MS, Larsen H, Skåra T, Pettersen MK.   +5 more
europepmc   +2 more sources

Caracterização pós-colheita de mamão armazenado em atmosfera modificada Postharvest characterization of stored papaya in modified atmosphere

Revista Brasileira de Engenharia Agrícola e Ambiental, 2006
Através deste trabalho objetivou-se avaliar a vida pós-colheita de mamão, estudando-se o efeito da atmosfera modificada e absorvedor de etileno na qualidade dos frutos em três diferentes formas de armazenamento: frutos com embalagem de polietileno de ...
Luiz F. G. Oliveira Jr., Enilce M. Coelho, Fábio C. Coelho   +2 more
doaj   +1 more source

Estabilidade de farinha de batata-doce biofortificada Stability of biofortified sweet potato flour

Brazilian Journal of Food Technology, 2012
A farinha de batata-doce, quando elaborada a partir de raízes com alto teor de β-caroteno, é fonte de pró-vitamina A, que apresenta efeitos benéficos na saúde humana.
Rosa Maria Vercelino Alves, Danielle Ito, Jose Luiz Viana de Carvalho, Werito Fernandes de Melo, Ronoel Luiz de Oliveira Godoy   +4 more
doaj   +1 more source