Extremely low birthweight neonates with phenylketonuria require special dietary management

Kód výsledku v 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>

Nalezeny alternativní kódy

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

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

Extremely low birthweight neonates with phenylketonuria require special dietary management

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

Extremely low birthweight neonates with phenylketonuria require special dietary management

Popis výsledku anglicky

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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30209 - Paediatrics

Projekt

—

Návaznosti

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Rok uplatnění

2021

Kód důvěrnosti údajů

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

Název periodika

ACTA PAEDIATRICA

ISSN

0803-5253

e-ISSN

1651-2227

Svazek periodika

110

Číslo periodika v rámci svazku

11

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

6

Strana od-do

2994-2999

Kód UT WoS článku

000678910800001

EID výsledku v databázi Scopus

2-s2.0-85111444405