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Lipidomics uncovers metabolic manifestations related to liver steatosis and low-grade systemic inflammation in diet-treated hereditary fructose intolerance patients.

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第一作者: Javier de Las,Heras
第一单位: Congenital Metabolic Disorders and Metabolic Liver Disease Laboratory, Biobizkaia Health Research Institute, 48093 Barakaldo, Spain; Division of Paediatric Metabolism, CIBERER, MetabERN, Cruces University Hospital, 48093 Barakaldo, Spain; Department of Paediatrics, University of the Basque Country (UPV/EHU), 48940 Leioa, Spain; AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain. Electronic address: javier.delasherasmontero@osakidetza.eus.
作者单位: Congenital Metabolic Disorders and Metabolic Liver Disease Laboratory, Biobizkaia Health Research Institute, 48093 Barakaldo, Spain; Division of Paediatric Metabolism, CIBERER, MetabERN, Cruces University Hospital, 48093 Barakaldo, Spain; Department of Paediatrics, University of the Basque Country (UPV/EHU), 48940 Leioa, Spain; AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain. Electronic address: javier.delasherasmontero@osakidetza.eus. [1] Congenital Metabolic Disorders and Metabolic Liver Disease Laboratory, Biobizkaia Health Research Institute, 48093 Barakaldo, Spain; AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Biochemistry Laboratory, Metabolism Area, Cruces University Hospital, 48903, Barakaldo, Spain. [2] Liver Disease Lab, Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), 48160 Derio, Spain. [3] Congenital Metabolic Disorders and Metabolic Liver Disease Laboratory, Biobizkaia Health Research Institute, 48093 Barakaldo, Spain. [4] Department of Physiology, Faculty of Medicine and Nursing, University of the Basque Country UPV/EHU, 48940 Leioa, Spain. [5] OWL Metabolomics (Rubió Metabolomics S.L.U.), Bizkaia Technology Park, 48160, Derio, Spain. [6] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Paediatrics Unit, Río Hortega University Hospital, 47012, Valladolid, Spain. [7] Congenital Metabolic Disorders and Metabolic Liver Disease Laboratory, Biobizkaia Health Research Institute, 48093 Barakaldo, Spain; Food Research, AZTI, Basque Research and Technology Alliance (BRTA), Parque Tecnológico de Bizkaia, Astondo Bidea, Edifício 609, 48160 Derio, Spain. [8] Liver Disease Lab, Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), 48160 Derio, Spain; Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Carlos III National Health Institute, 28029, Madrid, Spain. [9] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Metabolic Diseases Unit, Department of Paediatrics, Ramón y Cajal Hospital, 28034, Madrid, Spain. [10] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Department of Metabolism Diseases and Nutrition, Niño Jesús University Children´s Hospital, 28009, Madrid, Spain. [11] Congenital Metabolic Disorders and Metabolic Liver Disease Laboratory, Biobizkaia Health Research Institute, 48093 Barakaldo, Spain; AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Internal Medicine Service, Cruces University Hospital, 48903, Barakaldo, Spain. [12] 12 de Octubre University Hospital, CIBERER, MetabERN, 28041, Madrid, Spain. [13] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Nutrition and Metabolic Diseases Unit. La Fe University Hospital, 46026, Valencia, Spain. [14] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Department of Paediatric Gastroenterology, Hepatology and Nutrition, Sant Joan de Déu Hospital, 08950, Barcelona, Spain. [15] Araba University Hospital, 01009, Vitoria-Gasteiz, Spain. [16] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Paediatric Service, Albacete University Hospital, 02006, Castilla-La Mancha, Spain. [17] Pharmacy Department, Infanta Leonor University Hospital, 28031, Madrid, Spain; Asociación afectados Intolerancia Hereditária a la Fructosa, 28037, Madrid, Spain. [18] Department of Paediatric Gastroenterology, Hepatology and Nutrition, Sant Joan de Déu Hospital, 08950, Barcelona, Spain. [19] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; 12 de Octubre University Hospital, CIBERER, MetabERN, 28041, Madrid, Spain. [20] Clinical Nutrition Section, Navarra University Hospital, 31008, Pamplona, Spain. [21] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Paediatric Gastroenterology, Hepatology and Nutrition Unit, Mother and Child Insular University Hospital complex, Asociación Canaria para la Investigación Pediátrica (ACIP), CIBEROBN. University Institute for Research in Biomedical and Health Sciences, University of Las Palmas de Gran Canaria, 35016, Las Palmas de Gran Canaria, Spain. [22] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Department of Forensic Sciences, Pathology, Gynecology and Obstetrics, Pediatrics, Metabolic Unit, Health Research Institute of Santiago de Compostela (IDIS), Hospital Clínico Universitario de Santiago de Compostela, CIBERER, MetabERN, Santiago de Compostela, Spain. [23] AECOM Asociación Española para el Estudio de los Errores Congénitos del Metabolismo, Spain; Paediatric Service, Málaga Regional University Hospital (HRU), 29010, Málaga, Spain. [24] Fundación Española para el Estudio e Terapéutica de la Enfermedad de Gaucher y otras lisosomales (FEETEG), GIIS-012 group, Aragon Health Research Institute (IIS Aragón), 50009 Zaragoza, Spain. [25] Osatek, Hospital Galdakao-Usansolo, 48960, Galdakao, Spain. [26] Research into the genetics and control of diabetes and other endocrine disorders, Biobizkaia Health Research Institute, 48093 Barakaldo, Spain; Department of Physiology, Faculty of Medicine and Nursing, University of the Basque Country UPV/EHU, 48940 Leioa, Spain; Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Carlos III National Health Institute, 28029, Madrid, Spain. [27] Congenital Metabolic Disorders and Metabolic Liver Disease Laboratory, Biobizkaia Health Research Institute, 48093 Barakaldo, Spain; Liver Disease Lab, Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), 48160 Derio, Spain; IKERBASQUE, Basque Foundation for Science, 48009 Bilbao, Spain. Electronic address: tcardosodelgado@gmail.com. [28]
DOI 10.1016/j.clnu.2026.106608
PMID 41806524
发布时间 2026-03-28
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