Extremely low birthweight neonates with phenylketonuria require special dietary management
Language English Country Norway Media print-electronic
Document type Journal Article
Grant support
MH CZ RVO-VFN 64165
Ministerstvo Zdravotnictví Ceské Republiky
Q32/LF1
Univerzita Karlova v Praze
PubMed
34289149
DOI
10.1111/apa.16035
Knihovny.cz E-resources
- Keywords
- ELBW, dietary management, phenylalanine, phenylketonuria, tolerance,
- MeSH
- Phenylalanine MeSH
- Phenylketonurias * diagnosis MeSH
- Infant MeSH
- Humans MeSH
- Infant, Newborn MeSH
- Neonatal Screening MeSH
- Parenteral Nutrition MeSH
- Birth Weight MeSH
- Check Tag
- Infant MeSH
- Humans MeSH
- Infant, Newborn MeSH
- Publication type
- Journal Article MeSH
- Names of Substances
- Phenylalanine MeSH
AIM: Extremely low birthweight (ELBW) neonates require a high protein intake, but this can be challenging in the very rare cases when they also have phenylketonuria (PKU). This is due to a lack of suitable parenteral nutrition or enteral formula. Our aim was to analyse tolerance to phenylalanine in these infants. MATERIAL: There are approximately 110 000 children born in the Czech Republic each year. A neonatal screening programme from 2005 to 2020 found that 320 neonates had PKU, including 30 premature neonates with a birth weight of less than 2500 g. RESULTS: This study focused on three neonates who were born with ELBWs of 720, 740 and 950 g, respectively. Phenylalanine levels normalised in ELBW neonates with PKU within 1 week of the introduction of low-phenylalanine parenteral or enteral nutrition. The tolerance to phenylalanine was very high (70-110 mg/kg) in the first months of life, due to a rapid weight gain, but significantly decreased during infancy. CONCLUSION: Extremely low birthweight neonates with PKU need special dietary management. Regular assessments of phenylalanine are necessary during the first weeks of life to allow prompt dietary adjustments that reflect rapid weight gain and transitory high tolerance to phenylalanine.
Department of Biochemistry Faculty Hospital Motol Prague Czech Republic
Department of Paediatrics Regional Hospital Zlin Zlin Czech Republic
Institute for Care of Mother and Child Prague Czech Republic
See more in PubMed
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