Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Nov;110(11):2994-2999.
doi: 10.1111/apa.16035. Epub 2021 Jul 26.

Extremely low birthweight neonates with phenylketonuria require special dietary management

Marketa Zemanova  1 , Petr Chrastina  2 , Vaclav Sebron  3 , Dagmar Prochazkova  4 , Helena Jahnova  2 , Petra Sanakova  5 , Lucie Prochazkova  6 , Barbara Tesarova  6 , Jiri Zeman  2
Affiliations

Extremely low birthweight neonates with phenylketonuria require special dietary management

Marketa Zemanova et al. Acta Paediatr. 2021 Nov.

Abstract

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.

Keywords: ELBW; dietary management; phenylalanine; phenylketonuria; tolerance.

PubMed Disclaimer

References

REFERENCES

    1. Ballhausen D, Egli D, Bickle-Graz M, Bianchi N, Bonafé L. Born at 27 weeks of gestation with classical PKU: challenges of dietetic management in a very preterm infant. Pediatr Rep. 2011;3(4):103-107.
    1. Burton BK, Longo N, Vockley J, et al. Pegvaliase for the treatment of PKU: results of the phase 2 dose-finding studies with long-term follow-up. Mol Genet Metab. 2020;130(4):239-246.
    1. Bushati C, Chan B, Owen AH, et al. Challenges in implementing exclusive human milk diet to extremely low-birth-weight infants in a level III neonatal intensive care unit. [published online ahead of print February 23, 2021]. Nutr Clin Pract. 2021. https://doi.org/10.1002/ncp.10625
    1. Chu SS, White HO, Rindone SL, Tripp SA, Rhein LM. An initiative to reduce preterm infants pre-discharge growth failure through time-specific feeding volume increase. Pediatr Qual Saf. 2020;6(1):e366. https://doi.org/10.1097/pq9.0000000000000366
    1. Kaul YF, Johansson M, Månsson J, et al. Cognitive profiles of extremely preterm children: full-scale IQ hides strengths and weaknesses. Acta Paediatr. 2021;110(6):1817-1826.

LinkOut - more resources