Daily Fructose Traces Intake and Liver Injury in Children with Hereditary Fructose Intolerance
| Autor: | Giancarlo Parenti, Maria Grazia Caprio, Gianfranco Vallone, Raffaele Iorio, M.G. Puoti, Gabriella Esposito, Simona Fecarotta, Severo Pagliardini, Claudia Zuppaldi, Simona Spadarella, Maria Immacolata Spagnuolo, Fabiola Di Dato |
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| Přispěvatelé: | Di Dato, Fabiola, Spadarella, Simona, Puoti, Maria Giovanna, Caprio, Maria Grazia, Pagliardini, Severo, Zuppaldi, Claudia, Vallone, Gianfranco, Fecarotta, Simona, Esposito, Gabriella, Iorio, Raffaele, Parenti, Giancarlo, Spagnuolo, Maria Immacolata |
| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
Male Sucrose Hereditary fructose intolerance Gastroenterology Severity of Illness Index fructose chemistry.chemical_compound Diet Carbohydrate-Restricted Fructose-Bisphosphate Aldolase Child chemistry.chemical_classification Liver injury Aldolase B Fructose Liver steatosis Sialotransferrin profile Sorbitol Adolescent Alanine Transaminase Biomarkers Fatty Liver Female Fructose Intolerance Genetic Predisposition to Disease Humans Mutation Phenotype Retrospective Studies Sialoglycoproteins Transferrin Nutrition and Dietetics biology sucrose lcsh:Nutrition. Foods and food supply medicine.medical_specialty lcsh:TX341-641 Article liver steatosis liver steatosi 03 medical and health sciences Internal medicine medicine sorbitol Carbohydrate-Restricted 030102 biochemistry & molecular biology sialotransferrin profile business.industry aldolase B medicine.disease Diet 030104 developmental biology chemistry biology.protein Steatosis business Food Science |
| Zdroj: | Nutrients Volume 11 Issue 10 Nutrients 11 (2019). doi:10.3390/nu11102397 info:cnr-pdr/source/autori:Di Dato, Fabiola; Spadarella, Simona; Puoti, Maria Giovanna; Caprio, Maria Grazia; Pagliardini, Severo; Zuppaldi, Claudia; Vallone, Gianfranco; Fecarotta, Simona; Esposito, Gabriella; Iorio, Raffaele; Parenti, Giancarlo; Spagnuolo, Maria Immacolata/titolo:Daily Fructose Traces Intake and Liver Injury in Children with Hereditary Fructose Intolerance/doi:10.3390%2Fnu11102397/rivista:Nutrients/anno:2019/pagina_da:/pagina_a:/intervallo_pagine:/volume:11 Nutrients, Vol 11, Iss 10, p 2397 (2019) |
| ISSN: | 2072-6643 |
| DOI: | 10.3390/nu11102397 |
| Popis: | 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&rsquo 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. |
| Databáze: | OpenAIRE |
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