87706 20210902121602.0 doi 10.1016/j.clnu.2019.02.019 sideral 111315 ART-2020-111315 eng Aldámiz-Echevarría, L. Non-alcoholic fatty liver in hereditary fructose intolerance 2020 Access copy available to the general public Unrestricted Background: Non-alcoholic fatty liver disease (NAFLD) is characterized by fat accumulation affecting >5% of the liver volume that is not explained by alcohol abuse. It is known that fructose gives rise to NAFLD and it has been recently described that the ingestion of fructose in low amounts in aldolase B deficient mice is associated with the development of fatty liver. Therefore, it is reasonable that patients with HFI (Hereditary Fructose Intolerance) present fatty liver at diagnosis, but its prevalence in patients treated and with adequate follow-up is not well documented in the literature. The aim of this study is to analyze the association between HFI and NAFLD in treated patients. Methods: A cross-sectional observational study was conducted. The population comprised 16 genetically diagnosed HFI patients aged from 3 years to 48 and in dietary treatment of fructose, sorbitol and sacarose exclusion at least for two years. Blood samples were obtained for analytical studies and anthropometric measurements of each patient were performed. Results: Patients presented a Body Mass Index (BMI) of 17.9 ± 2.9 kg/m 2 . The HOMA index and Quick index were in normal range for our population. The S-adenosyl-methionine (SAM)/S-adenosyl-L-homocysteine (SAH) ratio was increased in the patients in whom this analysis was performed. By imaging techniques it was observed that 9 of the 16 patients presented fatty liver (7 by hepatic MRI). Of these 9 patients, only 3 presented hepatomegaly. 7 of 9 patients affected by the c.448G > C mutation had fatty infiltration, of which three of them presented in addition hepatomegaly. Conclusions: There is a high prevalence of fatty liver in HFI patients and it is not related to obesity and insulin resistance. The diagnosis of fatty liver in HFI patients and, above all, the identification of new therapeutic approaches, can positively impact the quality of life of these patients. info:eu-repo/semantics/openAccess by-nc-nd http://creativecommons.org/licenses/by-nc-nd/3.0/es/ 7.324 2020 NUTRITION & DIETETICS 7 / 88 = 0.08 2020 Q1 T1 1.915 2020 Nutrition and Dietetics 2020 Q1 Critical Care and Intensive Care Medicine 2020 Q1 info:eu-repo/semantics/article info:eu-repo/semantics/acceptedVersion de las Heras, J. Couce, M.L. Alcalde, C. Vitoria, I. Bueno, M. Blasco-Alonso, J. Concepción García, M. Universidad de Zaragoza (orcid)0000-0002-5563-3177 Ruiz, M. Suárez, R. Andrade, F. Villate, O. 1011 670 Universidad de Zaragoza Dpto. Microb.Ped.Radio.Sal.Pú. Área Pediatría 39, 2 (2020), 455-459 Clin. nutr. Clinical Nutrition 0261-5614 141351 http://zaguan.unizar.es/record/87706/files/texto_completo.pdf Postprint 45133 http://zaguan.unizar.es/record/87706/files/texto_completo.jpg?subformat=icon icon Postprint oai:zaguan.unizar.es:87706 articulos driver 2021-09-02-08:35:22 ARTICLE