| ZEMANOVÁ, Markéta, Petr CHRASTINA, Václav SEBRON, Dagmar PROCHÁZKOVÁ, Helena JAHNOVÁ, Petra SANAKOVA, Lucie PROCHÁZKOVÁ, Barbara TESAŘOVÁ and Jiří ZEMAN. Extremely low birthweight neonates with phenylketonuria require special dietary management. Acta pediatrica. HOBOKEN: Wiley, 2021, vol. 110, No 11, p. 2994-2999. ISSN 0803-5253. Available from: https://dx.doi.org/10.1111/apa.16035. |
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@article{1784124,
author = {Zemanová, Markéta and Chrastina, Petr and Sebron, Václav and Procházková, Dagmar and Jahnová, Helena and Sanakova, Petra and Procházková, Lucie and Tesařová, Barbara and Zeman, Jiří},
article_location = {HOBOKEN},
article_number = {11},
doi = {http://dx.doi.org/10.1111/apa.16035},
keywords = {ELBW; dietary management; phenylalanine; phenylketonuria; tolerance},
language = {eng},
issn = {0803-5253},
journal = {Acta pediatrica},
title = {Extremely low birthweight neonates with phenylketonuria require special dietary management},
url = {https://onlinelibrary.wiley.com/doi/10.1111/apa.16035},
volume = {110},
year = {2021}
}
TY - JOUR
ID - 1784124
AU - Zemanová, Markéta - Chrastina, Petr - Sebron, Václav - Procházková, Dagmar - Jahnová, Helena - Sanakova, Petra - Procházková, Lucie - Tesařová, Barbara - Zeman, Jiří
PY - 2021
TI - Extremely low birthweight neonates with phenylketonuria require special dietary management
JF - Acta pediatrica
VL - 110
IS - 11
SP - 2994-2999
EP - 2994-2999
PB - Wiley
SN - 08035253
KW - ELBW
KW - dietary management
KW - phenylalanine
KW - phenylketonuria
KW - tolerance
UR - https://onlinelibrary.wiley.com/doi/10.1111/apa.16035
N2 - 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.
ER -
ZEMANOVÁ, Markéta, Petr CHRASTINA, Václav SEBRON, Dagmar PROCHÁZKOVÁ, Helena JAHNOVÁ, Petra SANAKOVA, Lucie PROCHÁZKOVÁ, Barbara TESAŘOVÁ and Jiří ZEMAN. Extremely low birthweight neonates with phenylketonuria require special dietary management. \textit{Acta pediatrica}. HOBOKEN: Wiley, 2021, vol.~110, No~11, p.~2994-2999. ISSN~0803-5253. Available from: https://dx.doi.org/10.1111/apa.16035.
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