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Extremely low birthweight neonates with phenylketonuria require special dietary management

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023698%3A_____%2F21%3AN0000013" target="_blank" >RIV/00023698:_____/21:N0000013 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11110/21:10429502 RIV/65269705:_____/21:00075607 RIV/00064203:_____/21:10429502 RIV/00064165:_____/21:10429502 RIV/00216224:14110/21:00122006

  • Result on the web

    <a href="https://doi.org/10.1111/apa.16035" target="_blank" >https://doi.org/10.1111/apa.16035</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1111/apa.16035" target="_blank" >10.1111/apa.16035</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Extremely low birthweight neonates with phenylketonuria require special dietary management

  • Original language description

    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.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30209 - Paediatrics

Result continuities

  • Project

  • Continuities

    V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Others

  • Publication year

    2021

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    ACTA PAEDIATRICA

  • ISSN

    0803-5253

  • e-ISSN

    1651-2227

  • Volume of the periodical

    110

  • Issue of the periodical within the volume

    11

  • Country of publishing house

    US - UNITED STATES

  • Number of pages

    6

  • Pages from-to

    2994-2999

  • UT code for WoS article

    000678910800001

  • EID of the result in the Scopus database

    2-s2.0-85111444405