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Kidney and vascular function in adult patients with hereditary fructose intolerance

Objective: Previous studies have shown that patients with hereditary fructose intolerance (HFI) are characterized by a greater intrahepatic triglyceride content, despite a fructose-restricted diet. The present study aimed to examine the long-term consequences of HFI on other aldolase-B-expressing or...

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Autores principales: Simons, Nynke, Debray, François-Guillaume, Schaper, Nicolaas C., Feskens, Edith J.M., Hollak, Carla E.M., Bons, Judith A.P., Bierau, Jörgen, Houben, Alfons J.H.M., Schalkwijk, Casper G., Stehouwer, Coen D.A., Cassiman, David, Brouwers, Martijn C.G.J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7225396/
https://www.ncbi.nlm.nih.gov/pubmed/32426234
http://dx.doi.org/10.1016/j.ymgmr.2020.100600
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author Simons, Nynke
Debray, François-Guillaume
Schaper, Nicolaas C.
Feskens, Edith J.M.
Hollak, Carla E.M.
Bons, Judith A.P.
Bierau, Jörgen
Houben, Alfons J.H.M.
Schalkwijk, Casper G.
Stehouwer, Coen D.A.
Cassiman, David
Brouwers, Martijn C.G.J.
author_facet Simons, Nynke
Debray, François-Guillaume
Schaper, Nicolaas C.
Feskens, Edith J.M.
Hollak, Carla E.M.
Bons, Judith A.P.
Bierau, Jörgen
Houben, Alfons J.H.M.
Schalkwijk, Casper G.
Stehouwer, Coen D.A.
Cassiman, David
Brouwers, Martijn C.G.J.
author_sort Simons, Nynke
collection PubMed
description Objective: Previous studies have shown that patients with hereditary fructose intolerance (HFI) are characterized by a greater intrahepatic triglyceride content, despite a fructose-restricted diet. The present study aimed to examine the long-term consequences of HFI on other aldolase-B-expressing organs, i.e. the kidney and vascular endothelium. Methods: Fifteen adult HFI patients were compared to healthy control individuals matched for age, sex and body mass index. Aortic stiffness was assessed by carotid-femoral pulse wave velocity (cf-PWV) and endothelial function by peripheral arterial tonometry, skin laser doppler flowmetry and the endothelial function biomarkers soluble E-selectin [sE-selectin] and von Willebrand factor. Serum creatinine and cystatin C were measured to estimate the glomerular filtration rate (eGFR). Urinary glucose and amino acid excretion and the ratio of tubular maximum reabsorption of phosphate to GFR (TmP/GFR) were determined as measures of proximal tubular function. Results: Median systolic blood pressure was significantly higher in HFI patients (127 versus 122 mmHg, p = .045). Pulse pressure and cf-PWV did not differ between the groups (p = .37 and p = .49, respectively). Of all endothelial function markers, only sE-selectin was significantly higher in HFI patients (p = .004). eGFR was significantly higher in HFI patients than healthy controls (119 versus 104 ml/min/1.73m(2), p = .001, respectively). All measurements of proximal tubular function did not differ significantly between the groups. Conclusions: Adult HFI patients treated with a fructose-restricted diet are characterized by a higher sE-selectin level and slightly higher systolic blood pressure, which in time could contribute to a greater cardiovascular risk. The exact cause and, hence, clinical consequences of the higher eGFR in HFI patients, deserves further study.
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spelling pubmed-72253962020-05-18 Kidney and vascular function in adult patients with hereditary fructose intolerance Simons, Nynke Debray, François-Guillaume Schaper, Nicolaas C. Feskens, Edith J.M. Hollak, Carla E.M. Bons, Judith A.P. Bierau, Jörgen Houben, Alfons J.H.M. Schalkwijk, Casper G. Stehouwer, Coen D.A. Cassiman, David Brouwers, Martijn C.G.J. Mol Genet Metab Rep Research Paper Objective: Previous studies have shown that patients with hereditary fructose intolerance (HFI) are characterized by a greater intrahepatic triglyceride content, despite a fructose-restricted diet. The present study aimed to examine the long-term consequences of HFI on other aldolase-B-expressing organs, i.e. the kidney and vascular endothelium. Methods: Fifteen adult HFI patients were compared to healthy control individuals matched for age, sex and body mass index. Aortic stiffness was assessed by carotid-femoral pulse wave velocity (cf-PWV) and endothelial function by peripheral arterial tonometry, skin laser doppler flowmetry and the endothelial function biomarkers soluble E-selectin [sE-selectin] and von Willebrand factor. Serum creatinine and cystatin C were measured to estimate the glomerular filtration rate (eGFR). Urinary glucose and amino acid excretion and the ratio of tubular maximum reabsorption of phosphate to GFR (TmP/GFR) were determined as measures of proximal tubular function. Results: Median systolic blood pressure was significantly higher in HFI patients (127 versus 122 mmHg, p = .045). Pulse pressure and cf-PWV did not differ between the groups (p = .37 and p = .49, respectively). Of all endothelial function markers, only sE-selectin was significantly higher in HFI patients (p = .004). eGFR was significantly higher in HFI patients than healthy controls (119 versus 104 ml/min/1.73m(2), p = .001, respectively). All measurements of proximal tubular function did not differ significantly between the groups. Conclusions: Adult HFI patients treated with a fructose-restricted diet are characterized by a higher sE-selectin level and slightly higher systolic blood pressure, which in time could contribute to a greater cardiovascular risk. The exact cause and, hence, clinical consequences of the higher eGFR in HFI patients, deserves further study. Elsevier 2020-05-11 /pmc/articles/PMC7225396/ /pubmed/32426234 http://dx.doi.org/10.1016/j.ymgmr.2020.100600 Text en © 2020 The Authors http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Research Paper
Simons, Nynke
Debray, François-Guillaume
Schaper, Nicolaas C.
Feskens, Edith J.M.
Hollak, Carla E.M.
Bons, Judith A.P.
Bierau, Jörgen
Houben, Alfons J.H.M.
Schalkwijk, Casper G.
Stehouwer, Coen D.A.
Cassiman, David
Brouwers, Martijn C.G.J.
Kidney and vascular function in adult patients with hereditary fructose intolerance
title Kidney and vascular function in adult patients with hereditary fructose intolerance
title_full Kidney and vascular function in adult patients with hereditary fructose intolerance
title_fullStr Kidney and vascular function in adult patients with hereditary fructose intolerance
title_full_unstemmed Kidney and vascular function in adult patients with hereditary fructose intolerance
title_short Kidney and vascular function in adult patients with hereditary fructose intolerance
title_sort kidney and vascular function in adult patients with hereditary fructose intolerance
topic Research Paper
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7225396/
https://www.ncbi.nlm.nih.gov/pubmed/32426234
http://dx.doi.org/10.1016/j.ymgmr.2020.100600
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