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Extremely low birthweight neonates with phenylketonuria require special dietary management

M. Zemanova, P. Chrastina, V. Sebron, D. Prochazkova, H. Jahnova, P. Sanakova, L. Prochazkova, B. Tesarova, J. Zeman

. 2021 ; 110 (11) : 2994-2999. [pub] 20210726

Language English Country Norway

Document type Journal Article

Persistent link   https://www.medvik.cz/link/bmc22003254

Grant support
MH CZ RVO-VFN 64165 Ministerstvo Zdravotnictví Ceské Republiky
Q32/LF1 Univerzita Karlova v Praze

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.

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$a AIM: Extremely low birthweight (ELBW) neonates require a high protein intake, but this can be challenging in the very rare cases when they also have phenylketonuria (PKU). This is due to a lack of suitable parenteral nutrition or enteral formula. Our aim was to analyse tolerance to phenylalanine in these infants. MATERIAL: There are approximately 110 000 children born in the Czech Republic each year. A neonatal screening programme from 2005 to 2020 found that 320 neonates had PKU, including 30 premature neonates with a birth weight of less than 2500 g. RESULTS: This study focused on three neonates who were born with ELBWs of 720, 740 and 950 g, respectively. Phenylalanine levels normalised in ELBW neonates with PKU within 1 week of the introduction of low-phenylalanine parenteral or enteral nutrition. The tolerance to phenylalanine was very high (70-110 mg/kg) in the first months of life, due to a rapid weight gain, but significantly decreased during infancy. CONCLUSION: Extremely low birthweight neonates with PKU need special dietary management. Regular assessments of phenylalanine are necessary during the first weeks of life to allow prompt dietary adjustments that reflect rapid weight gain and transitory high tolerance to phenylalanine.
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$a Chrastina, Petr $u Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General Faculty Hospital, Prague, Czech Republic
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$a Sebron, Vaclav $u Department of Gynecology, First Faculty of Medicine, Charles University and General Faculty Hospital, Prague, Czech Republic
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$a Prochazkova, Dagmar $u Department of Paediatrics, Medical Genetics and Genomics, University Hospital and Faculty of Medicine, Masaryk University, Brno, Czech Republic
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$a Jahnova, Helena $u Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General Faculty Hospital, Prague, Czech Republic
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$a Sanakova, Petra $u Institute for Care of Mother and Child, Prague, Czech Republic
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$a Prochazkova, Lucie $u Department of Paediatrics, Regional Hospital Zlin, Zlin, Czech Republic
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$a Tesarova, Barbara $u Department of Paediatrics, Regional Hospital Zlin, Zlin, Czech Republic
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$a Zeman, Jiri $u Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General Faculty Hospital, Prague, Czech Republic
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