Extremely low birthweight neonates with phenylketonuria require special dietary management.
ELBW
dietary management
phenylalanine
phenylketonuria
tolerance
Journal
Acta paediatrica (Oslo, Norway : 1992)
ISSN: 1651-2227
Titre abrégé: Acta Paediatr
Pays: Norway
ID NLM: 9205968
Informations de publication
Date de publication:
Nov 2021
Nov 2021
Historique:
revised:
17
07
2021
received:
05
04
2021
accepted:
20
07
2021
pubmed:
22
7
2021
medline:
3
11
2021
entrez:
21
7
2021
Statut:
ppublish
Résumé
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. 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. 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. 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.
Substances chimiques
Phenylalanine
47E5O17Y3R
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
2994-2999Subventions
Organisme : Ministerstvo Zdravotnictví Ceské Republiky
ID : MH CZ RVO-VFN 64165
Organisme : Univerzita Karlova v Praze
ID : Q32/LF1
Informations de copyright
©2021 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.
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