{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T12:29:47Z","timestamp":1786364987311,"version":"build-2736575974"},"reference-count":40,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T00:00:00Z","timestamp":1786320000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"DMF PHARMA FoodAR"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Background\/Objectives: Hereditary fructose intolerance (HFI) is a rare autosomal recessive metabolic disorder caused by variants in the ALDOB gene, resulting in aldolase B deficiency. Symptoms occur after the ingestion of fructose, sucrose, or sorbitol, leading to metabolic toxicity. Management is exclusively dietary, but clinical practice remains highly variable. This survey aimed to investigate current Italian dietary management practices for patients with HFI. Methods: A cross-sectional centre-level survey was conducted using a 29-item questionnaire distributed to metabolic dietitians within the Italian Society for the Study of Inherited Metabolic Diseases network between April and May 2026. Results: Data from 27 dietitians across 15 Italian inherited metabolic disease centres were analysed. Over half of the centres (53.3%) provided care for both paediatric and adult patients, and all centres (100%) supplied structured educational materials for the fructose-, sucrose-, and sorbitol-restricted diet. Marked heterogeneity was observed in dietary practices, particularly in the classification of sugars and sweeteners, recommendations for cereal-based products, and fibre supplementation strategies. Cereal-based dietary guidance was provided by 10\/15 centres (66.7%), ranging from no specific recommendations (5\/15, 33.3%) to a preference for refined grains (7\/15, 46.7%) or fibre-based restrictions (2\/15, 13.3%). With regard to sugars and sweeteners, consensus was observed only for maltitol and sorbitol, which were classified as prohibited by all centres (15\/15, 100%). Mannitol and isomalt were prohibited in 14\/15 centres (93.3%). All other compounds showed variable recommendations across centres, including xylitol (prohibited 46.7%, permitted 40.0%), erythritol (permitted 60.0%), and sucralose (prohibited 33.3%, permitted 20.0%, restricted 20.0%, unspecified 26.7%). Vitamin C (100%) and folic acid (86.6%) were the most commonly prescribed supplements. One-third (33%) of the centres required the use of fibre supplements or osmotic laxatives to manage chronic constipation, with wide variability in the specific type of fibre recommended. Conclusions: These findings highlight substantial heterogeneity in dietary management of HFI across specialised Italian centres and support the development of evidence-based, standardised nutritional guidelines to improve the consistency of care, nutritional adequacy, and patient outcomes.<\/jats:p>","DOI":"10.3390\/nu18162609","type":"journal-article","created":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T11:38:09Z","timestamp":1786361889000},"page":"2609","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Current Dietary Practices in Hereditary Fructose Intolerance: Results from a National Survey Across 15 Italian Metabolic Centres"],"prefix":"10.3390","volume":"18","author":[{"given":"Giulia","family":"Bruni","sequence":"first","affiliation":[{"name":"Dietetic Unit, Meyer Children University Hospital IRCCS, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juri","family":"Zuvadelli","sequence":"additional","affiliation":[{"name":"Clinical Department of Paediatrics, ASST Santi Paolo e Carlo, San Paolo Hospital, University of Milan, 20142 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia Maria","family":"Bernabei","sequence":"additional","affiliation":[{"name":"Nutritional Rehabilitation Unit, SITRA, Bambino Ges\u00f9 Children\u2019s Hospital IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-4415-0324","authenticated-orcid":false,"given":"Cristina","family":"Bonfanti","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Fondazione IRCCS San Gerardo Hospital, University Milano Bicocca, 20900 Monza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0750-9451","authenticated-orcid":false,"given":"Barbara","family":"Borsani","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Vittore Buzzi Children\u2019s Hospital, University of Milan, 20154 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-9539-2649","authenticated-orcid":false,"given":"Alice","family":"Dianin","sequence":"additional","affiliation":[{"name":"Regional Center for Screening, Diagnosis, and Treatment of Inherited Metabolic Diseases, Pediatrics C Unit, University Hospital of Verona, 37126 Verona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annalisa","family":"Finizii","sequence":"additional","affiliation":[{"name":"Clinical Department of Paediatrics, ASST Santi Paolo e Carlo, San Paolo Hospital, University of Milan, 20142 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurora","family":"Favaro","sequence":"additional","affiliation":[{"name":"Regional Center for Screening, Diagnosis, and Treatment of Inherited Metabolic Diseases, Pediatrics C Unit, University Hospital of Verona, 37126 Verona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Giorda","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Metabolic Diseases, University of Turin, 10124 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3557-8078","authenticated-orcid":false,"given":"Giorgia","family":"Gugelmo","sequence":"additional","affiliation":[{"name":"Division of Metabolic Diseases, Department of Medicine, University Hospital of Padua, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Paola","family":"Ierardi","sequence":"additional","affiliation":[{"name":"Nutritional Center, Giannina Gaslini Pediatric Institute IRCCS, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Luciano","sequence":"additional","affiliation":[{"name":"Nutritional Center, Giannina Gaslini Pediatric Institute IRCCS, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-6203-5940","authenticated-orcid":false,"given":"Marta","family":"Muscarella","sequence":"additional","affiliation":[{"name":"Dietetic Unit, Meyer Children University Hospital IRCCS, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Pancaldi","sequence":"additional","affiliation":[{"name":"Clinical Dietietics Unit, IRCCS AOUBO, 40138 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-1644-6237","authenticated-orcid":false,"given":"Sara","family":"Parolisi","sequence":"additional","affiliation":[{"name":"UOSD Metabolic Diseases, AORN Santobono-Pausilipon, 80129 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7741-0874","authenticated-orcid":false,"given":"Veronica","family":"Perico","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Vittore Buzzi Children\u2019s Hospital, University of Milan, 20154 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Pierattini","sequence":"additional","affiliation":[{"name":"Dietetic Unit, Meyer Children University Hospital IRCCS, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cinzia","family":"Pistolesi","sequence":"additional","affiliation":[{"name":"Dietetic Unit, Careggi University Hospital, 50134 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lidia","family":"Pontillo","sequence":"additional","affiliation":[{"name":"Nutritional Center, Giannina Gaslini Pediatric Institute IRCCS, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-6713-8030","authenticated-orcid":false,"given":"Roberta","family":"Pretese","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Fondazione IRCCS San Gerardo Hospital, University Milano Bicocca, 20900 Monza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela Ira","family":"Pozzoli","sequence":"additional","affiliation":[{"name":"Pediatrics and Neonatology Unit, Regional Referral Clinical Center for Inborn Errors of Metabolism, Guglielmo da Saliceto Hospital, 29121 Piacenza, 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