Kidney and vascular function in adult patients with hereditary fructose intolerance
| Autor: | Carla E. M. Hollak, Edith J. M. Feskens, Casper G. Schalkwijk, David Cassiman, François-Guillaume Debray, Jörgen Bierau, Nynke Simons, Alfons J.H.M. Houben, Judith A P Bons, Coen D.A. Stehouwer, Martijn C. G. J. Brouwers, Nicolaas C. Schaper |
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| Přispěvatelé: | Interne Geneeskunde, RS: Carim - V01 Vascular complications of diabetes and metabolic syndrome, RS: Carim - V02 Hypertension and target organ damage, RS: CAPHRI - R2 - Creating Value-Based Health Care, MUMC+: MA Endocrinologie (9), MUMC+: DA CDL Algemeen (9), RS: NUTRIM - R3 - Respiratory & Age-related Health, MUMC+: DA KG Lab Centraal Lab (9), MUMC+: Centrum voor Chronische Zieken (3), MUMC+: MA Med Staf Artsass Interne Geneeskunde (9), MUMC+: HVC Pieken Maastricht Studie (9), MUMC+: MA Interne Geneeskunde (3), Endocrinology, AGEM - Inborn errors of metabolism |
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
glutamic acid
(Glu) Hereditary fructose intolerance leucine (Leu) BLOOD-PRESSURE CKD-EPI equation based on serum creatinine (eGFRcr) MICROVASCULAR DYSFUNCTION tyrosine (Tyr) Kidney UP-REGULATION chemistry.chemical_compound soluble E-selectin (sE-selectin) Endocrinology perfusion units (PU) isoleucine (Ile) methionine (Met) cysteine (Cys) threonine (Thr) von willebrand factor (vWF) phenylalanine (Phe) Pulse wave velocity lcsh:QH301-705.5 lcsh:R5-920 CKD-EPI equation based on cystatin c (eGFRcys) biology difference (Δ) citrulline (Cit) GLOMERULAR HYPERFILTRATION asparagine (Asn) Case-control study estimated glomerular filtration rate (eGFR) 95% confidence interval (95% CI) aldolase B (ALDOB) glutamine (Gln) Pulse pressure lysine (Lys) Blood hereditary fructose intolerance (HFI) histidine (His) CKD-EPI equation based on creatinine and cystatin c combined (eGFRcr-cys) lcsh:Medicine (General) alanine (Ala) glycine (Gly) ARTERIAL STIFFNESS reactive hyperemia index (RHI) Research Paper medicine.medical_specialty valine (Val) ALDOLASE-B ratio of tubular maximum reabsorption of phosphate to GFR (TmP/GFR) BODY-FLUIDS Urology Renal function DIAGNOSIS ornithine (Orn) Genetics medicine arginine (Arg) tubular reabsorption of phosphate (TRP) serine (Ser) intrahepatic triglyceride (IHTG) Molecular Biology reactive hyperemia peripheral arterial tonometry (RH-PAT) VLAG chronic kidney disease epidemiology collaboration (CKD-EPI) Global Nutrition Creatinine Wereldvoeding proline (Pro) business.industry DIETARY-SODIUM laser doppler flowmetry (LDF) medicine.disease glucokinase regulatory protein (GKRP) taurine (Tau) Fanconi syndrome SERUM CYSTATIN C Blood pressure chemistry Cystatin C lcsh:Biology (General) Arterial stiffness biology.protein tryptophan (Try) Vessels carotid-femoral pulse wave velocity (cf-PWV) business statistical package of social sciences (SPSS) |
| Zdroj: | Molecular Genetics and Metabolism Reports, 23:100600. Elsevier Science Molecular Genetics and Metabolism Reports, 23 Molecular Genetics and Metabolism Reports Molecular Genetics and Metabolism Reports 23 (2020) Molecular Genetics and Metabolism Reports, Vol 23, Iss, Pp-(2020) Molecular genetics and metabolism reports, 23:100600. Elsevier BV |
| ISSN: | 2214-4269 |
| Popis: | 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/1.73m2, 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. ispartof: MOLECULAR GENETICS AND METABOLISM REPORTS vol:23 ispartof: location:United States status: published |
| Databáze: | OpenAIRE |
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