A5024371731- Glycogen Storage Diseases and Myoclonus
- Dialysis and Renal Disease Management
- Lysosomal Storage Disorders Research
- Neonatal Health and Biochemistry
- Metabolism and Genetic Disorders
- Folate and B Vitamins Research
- Nutritional Studies and Diet
- Diabetes and associated disorders
- Diet, Metabolism, and Disease
- Digestive system and related health
- Restless Legs Syndrome Research
- Aldose Reductase and Taurine
- Diet and metabolism studies
- Prenatal Substance Exposure Effects
- Amino Acid Enzymes and Metabolism
- Genetics and Neurodevelopmental Disorders
- Muscle and Compartmental Disorders
University Medical Center Groningen
2019-2024
University of Groningen
2019-2024
Patients dependent on chronic hemodialysis treatment are prone to malnutrition, at least in part due insufficient nutrient intake, metabolic derangements, and inflammation. Losses of amino acids during may be an important additional contributor. In this study, we assessed changes plasma acid concentrations hemodialysis, quantified intradialytic losses, investigated whether losses by urinary excretion associated with fatigue. The study included a total 59 patients (65 ± 15 years, 63% male) 33...
Dried blood spot succinylacetone (SA) is often used as a biomarker for newborn screening (NBS) tyrosinemia type 1 (TT1). However, false-positive SA results are observed. Elevated may also be due to maleylacetoacetate isomerase deficiency (MAAI-D), which appears clinically insignificant. This study investigated whether urine organic acid (uOA) and quantitative maleic (Q-uMA) analyses can distinguish between TT1 MAAI-D. We reevaluated/measured uOA (GC-MS) and/or Q-uMA (LC-MS/MS) in available...
It is unknown whether meat intake beneficial for long-term patient and graft survival in kidney transplant recipients (KTR).
The urinary metabolite tetraglucoside (Glc4) is a potential biomarker for hepatic glycogen storage diseases (GSDs). Glc4 believed to reflect body content and/or turnover. However, dietary polysaccharide intake may influence excretion, potentially limiting the utility of as monitoring in GSDs. We aimed investigate association with excretion. Urinary excretion (mmol/mmol creatinine and mmol/24 h) was analyzed using validated LC-MS/MS method. Data from 65 kidney transplant recipients 58 healthy...
Taurine is a sulfur containing nutrient that has been shown to protect against oxidative stress, which implicated in the pathophysiology leading late graft failure after renal transplantation. We prospectively investigated whether high urinary taurine excretion, reflecting intake, associated with low risk for development of transplant recipients (RTR). Urinary excretion was measured longitudinal cohort 678 stable RTR. Prospective associations were assessed using Cox regression analyses....