Dietary Patterns in a Nationwide Cohort of Patients with Hereditary Fructose Intolerance

<b>Background/Objectives</b>: Hereditary fructose intolerance (HFI) is an inherited metabolic disorder caused by a deficiency of the enzyme fructose-1,6-bisphosphate aldolase. Treatment consists of a lifelong diet restricted in fructose, sucrose, and sorbitol (FSS). The aim of this study was to determine dietary intake of FSS and to analyze the consumption patterns of vegetables, fruit, legumes, pulses, and dried fruit in a nationwide cohort of HFI patients. <b>Methods</b>: Overall, 36 HFI patients and 28 age-, sex- and BMI-matched healthy control subjects participated in this study. A self-administered three-day dietary record and an adapted quantitative food frequency questionnaire (FFQ) including frequency and portion sizes were collected. FSS intake was calculated using the DIAL Nutritional Calculation Program (ALCE INGENIERÍA). Total fructose intake was calculated as the sum of free fructose, 50% of sucrose, and sorbitol. <b>Results</b>: Protein intake was significantly higher in HFI patients compared to the controls (92.43 g/day [65.1-165.03] vs. 70.39 g/day [35.21-133.83]; <i>p</i> = 0.001). In most patients, total fructose intake was within the recommended limits (9.79 mg/kg bw/day [0.29-59.09]), with no significant differences between children and adults (<i>p</i> = 0.325). Although the established dietary recommendations did not always match the actual intake observed in a real-life setting, in general, foods with higher fructose content were consumed less frequently and in smaller quantities. <b>Conclusions</b>: Further research on the fructose content of various foods, particularly fruits and vegetables, and updated dietary recommendations for HFI patients are warranted to provide the best tools for the nutritional management of the disease.

Authors

Institutions

Publication Details

Journal
Nutrients
Published
2026-02-27
DOI
https://doi.org/10.3390/nu18050771
Primary Topic
Diet, Metabolism, and Disease
Type
article
Field-Weighted Citation Impact
0.00

Funders

Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Dietary Patterns in a Nationwide Cohort of Patients with Hereditary Fructose Intolerance

Estrella Petrina Jáuregui, Dolores García-Arenas, Silvia Chumillas-Calzada, Leticia Ceberio et al.
Nutrients
Diet, Metabolism, and Disease
article

Dietary Patterns in a Nationwide Cohort of Patients with Hereditary Fructose Intolerance

Estrella Petrina Jáuregui, Dolores García-Arenas, Silvia Chumillas-Calzada, Leticia Ceberio, Consuelo Pedrón‐Giner, Tomas Santos Hernandez, Igor Gómez, Arantza Arza, Juliana Serrano-Nieto, Teresa C. Delgado, Carlos Losilla Alcalde, Ainara Cano, Estrella Diego, Patricia Correcher, Marcello Bellusci, María-Luz Couce, Amaya Belanger-Quintana, Montserrat Morales, Elsa Izquierdo-García, Dámaris Martínez Chicano, Isidro Vitoria Miñana, Elvira Cañedo-Villarroya, María Soledad López-García, Paula Sánchez-Pintos, María Unceta Suarez, Javier de las Heras, Edorta Mora
article en

Abstract

<b>Background/Objectives</b>: Hereditary fructose intolerance (HFI) is an inherited metabolic disorder caused by a deficiency of the enzyme fructose-1,6-bisphosphate aldolase. Treatment consists of a lifelong diet restricted in fructose, sucrose, and sorbitol (FSS). The aim of this study was to determine dietary intake of FSS and to analyze the consumption patterns of vegetables, fruit, legumes, pulses, and dried fruit in a nationwide cohort of HFI patients. <b>Methods</b>: Overall, 36 HFI patients and 28 age-, sex- and BMI-matched healthy control subjects participated in this study. A self-administered three-day dietary record and an adapted quantitative food frequency questionnaire (FFQ) including frequency and portion sizes were collected. FSS intake was calculated using the DIAL Nutritional Calculation Program (ALCE INGENIERÍA). Total fructose intake was calculated as the sum of free fructose, 50% of sucrose, and sorbitol. <b>Results</b>: Protein intake was significantly higher in HFI patients compared to the controls (92.43 g/day [65.1-165.03] vs. 70.39 g/day [35.21-133.83]; <i>p</i> = 0.001). In most patients, total fructose intake was within the recommended limits (9.79 mg/kg bw/day [0.29-59.09]), with no significant differences between children and adults (<i>p</i> = 0.325). Although the established dietary recommendations did not always match the actual intake observed in a real-life setting, in general, foods with higher fructose content were consumed less frequently and in smaller quantities. <b>Conclusions</b>: Further research on the fructose content of various foods, particularly fruits and vegetables, and updated dietary recommendations for HFI patients are warranted to provide the best tools for the nutritional management of the disease.

NutrientsVol. 18(5)
Ikerbasque (ES), Universidad de Las Palmas de Gran Canaria (ES), Hospital Sant Joan de Déu Barcelona (ES), University of the Basque Country (ES), Universidade de Santiago de Compostela (ES), Instituto Cajal (ES), Hospital Universitario Insular de Gran Canaria (ES), Hospital Universitari i Politècnic La Fe (ES), Research Institute Hospital 12 de Octubre (ES), BioCruces Health research Institute (ES), Hospital Regional Universitario de Málaga (ES), Hospital Universitario Araba (ES), Centre for Biomedical Network Research on Rare Diseases (ES), Center for Research in Molecular Medicine and Chronic Diseases (ES), Hospital Infantil Universitario Niño Jesús (ES), Hospital de Cruces (ES), Instituto de Investigación Sanitaria de Santiago (ES), Hospital Universitario Río Hortega (ES), Hospital Universitario Ramón y Cajal (ES), Hospital Universitario Infanta Leonor (ES), University of Castilla-La Mancha (ES), Universidad de Navarra (ES)
Federación Española de Enfermedades Raras, Eusko Jaurlaritza, Ministerio de Ciencia e Innovación, European Regional Development Fund, Agencia Estatal de Investigación
Zero hunger
Openalex Percentile: Top 42%
Diet, Metabolism, and Disease
DOIOpen Access PDFOpenAlex
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.