Biomarkers of Micronutrients in Regular Follow-Up for Tyrosinemia Type 1 and Phenylketonuria Patients.
Adolescent
Adult
Aged
Amino Acids
/ administration & dosage
Biomarkers
/ blood
Biopterins
/ analogs & derivatives
Child
Child, Preschool
Cyclohexanones
/ therapeutic use
Diet, Protein-Restricted
/ adverse effects
Dietary Supplements
/ adverse effects
Enzyme Inhibitors
/ therapeutic use
Female
Humans
Infant
Male
Micronutrients
/ blood
Middle Aged
Nitrobenzoates
/ therapeutic use
Nutritional Status
Phenylketonurias
/ blood
Retrospective Studies
Risk Assessment
Risk Factors
Tyrosinemias
/ blood
Vitamin B 12 Deficiency
/ blood
Vitamin D Deficiency
/ blood
Young Adult
Phenylketonuria
Tyrosinemia type 1
micronutrients
nutritional status
vitamin B12
vitamin D
Journal
Nutrients
ISSN: 2072-6643
Titre abrégé: Nutrients
Pays: Switzerland
ID NLM: 101521595
Informations de publication
Date de publication:
27 Aug 2019
27 Aug 2019
Historique:
received:
17
07
2019
revised:
16
08
2019
accepted:
21
08
2019
entrez:
30
8
2019
pubmed:
30
8
2019
medline:
11
2
2020
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 31461828
pii: nu11092011
doi: 10.3390/nu11092011
pmc: PMC6769775
pii:
doi:
Substances chimiques
Amino Acids
0
Biomarkers
0
Cyclohexanones
0
Enzyme Inhibitors
0
Micronutrients
0
Nitrobenzoates
0
Biopterins
0
sapropterin
EGX657432I
nitisinone
K5BN214699
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
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