000087706 001__ 87706 000087706 005__ 20210902121602.0 000087706 0247_ $$2doi$$a10.1016/j.clnu.2019.02.019 000087706 0248_ $$2sideral$$a111315 000087706 037__ $$aART-2020-111315 000087706 041__ $$aeng 000087706 100__ $$aAldámiz-Echevarría, L. 000087706 245__ $$aNon-alcoholic fatty liver in hereditary fructose intolerance 000087706 260__ $$c2020 000087706 5060_ $$aAccess copy available to the general public$$fUnrestricted 000087706 5203_ $$aBackground: 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. 000087706 540__ $$9info:eu-repo/semantics/openAccess$$aby-nc-nd$$uhttp://creativecommons.org/licenses/by-nc-nd/3.0/es/ 000087706 590__ $$a7.324$$b2020 000087706 591__ $$aNUTRITION & DIETETICS$$b7 / 88 = 0.08$$c2020$$dQ1$$eT1 000087706 592__ $$a1.915$$b2020 000087706 593__ $$aNutrition and Dietetics$$c2020$$dQ1 000087706 593__ $$aCritical Care and Intensive Care Medicine$$c2020$$dQ1 000087706 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/acceptedVersion 000087706 700__ $$ade las Heras, J. 000087706 700__ $$aCouce, M.L. 000087706 700__ $$aAlcalde, C. 000087706 700__ $$aVitoria, I. 000087706 700__ $$aBueno, M. 000087706 700__ $$aBlasco-Alonso, J. 000087706 700__ $$0(orcid)0000-0002-5563-3177$$aConcepción García, M.$$uUniversidad de Zaragoza 000087706 700__ $$aRuiz, M. 000087706 700__ $$aSuárez, R. 000087706 700__ $$aAndrade, F. 000087706 700__ $$aVillate, O. 000087706 7102_ $$11011$$2670$$aUniversidad de Zaragoza$$bDpto. Microb.Ped.Radio.Sal.Pú.$$cÁrea Pediatría 000087706 773__ $$g39, 2 (2020), 455-459$$pClin. nutr.$$tClinical Nutrition$$x0261-5614 000087706 8564_ $$s141351$$uhttps://zaguan.unizar.es/record/87706/files/texto_completo.pdf$$yPostprint 000087706 8564_ $$s45133$$uhttps://zaguan.unizar.es/record/87706/files/texto_completo.jpg?subformat=icon$$xicon$$yPostprint 000087706 909CO $$ooai:zaguan.unizar.es:87706$$particulos$$pdriver 000087706 951__ $$a2021-09-02-08:35:22 000087706 980__ $$aARTICLE