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Open AccessArticle

Biomarkers of Micronutrients in Regular Follow-Up for Tyrosinemia Type 1 and Phenylketonuria Patients

1
Division of Metabolic Diseases, Groningen, Beatrix Children’s Hospital, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands
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Department of Endocrinology, Groningen, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands
3
Laboratory of Metabolic Diseases, Groningen, Department of Laboratory Medicine, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands
*
Author to whom correspondence should be addressed.
Nutrients 2019, 11(9), 2011; https://doi.org/10.3390/nu11092011
Received: 17 July 2019 / Revised: 16 August 2019 / Accepted: 21 August 2019 / Published: 27 August 2019
Phenylketonuria (PKU) is treated with dietary restrictions and sometimes tetrahydrobiopterin (BH4). PKU patients are at risk for developing micronutrient deficiencies, such as vitamin B12 and folic acid, likely due to their diet. Tyrosinemia type 1 (TT1) is similar to PKU in both pathogenesis and treatment. TT1 patients follow a similar diet, but nutritional deficiencies have not been investigated yet. In this retrospective study, biomarkers of micronutrients in TT1 and PKU patients were investigated and outcomes were correlated to dietary intake and anthropometric measurements from regular follow-up measurements from patients attending the outpatient clinic. Data was analyzed using Kruskal–Wallis, Fisher’s exact and Spearman correlation tests. Furthermore, descriptive data were used. Overall, similar results for TT1 and PKU patients (with and without BH4) were observed. In all groups high vitamin B12 concentrations were seen rather than B12 deficiencies. Furthermore, all groups showed biochemical evidence of vitamin D deficiency. This study shows that micronutrients in TT1 and PKU patients are similar and often within the normal ranges and that vitamin D concentrations could be optimized. View Full-Text
Keywords: Phenylketonuria; Tyrosinemia type 1; nutritional status; micronutrients; vitamin D; vitamin B12 Phenylketonuria; Tyrosinemia type 1; nutritional status; micronutrients; vitamin D; vitamin B12
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van Vliet, K.; Rodenburg, I.L.; van Ginkel, W.G.; Lubout, C.M.; Wolffenbuttel, B.H.; van der Klauw, M.M.; Heiner-Fokkema, M.R.; van Spronsen, F.J. Biomarkers of Micronutrients in Regular Follow-Up for Tyrosinemia Type 1 and Phenylketonuria Patients. Nutrients 2019, 11, 2011.

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