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Daily Fructose Traces Intake and Liver Injury in Children with Hereditary Fructose Intolerance

1
Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, 80131 Naples, Italy
2
Italian Society of Pediatric Gastroenterology and Nutrition (SIGENP), 20126 Milan, Italy
3
Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond St., Holborn, London WC1N 3JH, UK
4
Institute of Biostructure and Bioimaging National Research Council, 80145 Naples, Italy
5
Department of Pediatrics and Public Health and Pediatric Sciences, University of Torino, 10126 Torino, Italy
6
Pediatric Radiology, Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
7
Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, 80131 Naples, Italy
8
CEINGE Advanced Biotechnologies, 80145 Naples, Italy
9
Telethon Institute of Genetics and Medicine, 80078 Pozzuoli, Italy
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work as senior authors.
Nutrients 2019, 11(10), 2397; https://doi.org/10.3390/nu11102397
Received: 12 August 2019 / Revised: 23 September 2019 / Accepted: 2 October 2019 / Published: 7 October 2019
(This article belongs to the Special Issue Fructose and Glucose Intake and Human Health)
Background: Hereditary fructose intolerance (HFI) is a rare genetic disorder of fructose metabolism due to aldolase B enzyme deficiency. Treatment consists of fructose, sorbitol, and sucrose (FSS)-free diet. We explore possible correlations between daily fructose traces intake and liver injury biomarkers on a long-term period, in a cohort of young patients affected by HFI. Methods: Patients’ clinical data and fructose daily intake were retrospectively collected. Correlations among fructose intake, serum alanine aminotransferase (ALT) level, carbohydrate-deficient transferrin (CDT) percentage, liver ultrasonography, genotype were analyzed. Results: We included 48 patients whose mean follow-up was 10.3 ± 5.6 years and fructose intake 169 ± 145.4 mg/day. Eighteen patients had persistently high ALT level, nine had abnormal CDT profile, 45 had signs of liver steatosis. Fructose intake did not correlate with ALT level nor with steatosis severity, whereas it correlated with disialotransferrin percentage (R2 0.7, p < 0.0001) and tetrasialotransferrin/disialotransferrin ratio (R2 0.5, p = 0.0001). p.A150P homozygous patients had lower ALT values at diagnosis than p.A175D variant homozygotes cases (58 ± 55 IU/L vs. 143 ± 90 IU/L, p = 0.01). Conclusion: A group of HFI patients on FSS-free diet presented persistent mild hypertransaminasemia which did not correlate with fructose intake. Genotypes may influence serum liver enzyme levels. CDT profile represents a good marker to assess FSS intake. View Full-Text
Keywords: Hereditary fructose intolerance; fructose; sucrose; sorbitol; sialotransferrin profile; aldolase B; liver steatosis Hereditary fructose intolerance; fructose; sucrose; sorbitol; sialotransferrin profile; aldolase B; liver steatosis
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Di Dato, F.; Spadarella, S.; Puoti, M.G.; Caprio, M.G.; Pagliardini, S.; Zuppaldi, C.; Vallone, G.; Fecarotta, S.; Esposito, G.; Iorio, R.; Parenti, G.; Spagnuolo, M.I. Daily Fructose Traces Intake and Liver Injury in Children with Hereditary Fructose Intolerance. Nutrients 2019, 11, 2397.

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