Extremely  low birthweight neonates with phenylketonuria
require special dietary management

J 2021

Extremely low birthweight neonates with phenylketonuria require special dietary management

ZEMANOVÁ, Markéta, Petr CHRASTINA, Václav SEBRON, Dagmar PROCHÁZKOVÁ, Helena JAHNOVÁ et. al.

Basic information

Original name

Extremely low birthweight neonates with phenylketonuria require special dietary management

Name in Czech

Dětí s extrémně nízkou porodní hmotností a fenylketonurií potřebují speciální dietní péči

Authors

ZEMANOVÁ, Markéta (203 Czech Republic), Petr CHRASTINA (203 Czech Republic), Václav SEBRON, Dagmar PROCHÁZKOVÁ (203 Czech Republic, guarantor, belonging to the institution), Helena JAHNOVÁ (203 Czech Republic), Petra SANAKOVA (203 Czech Republic), Lucie PROCHÁZKOVÁ (203 Czech Republic), Barbara TESAŘOVÁ (203 Czech Republic) and Jiří ZEMAN (203 Czech Republic)

Edition

Acta pediatrica, HOBOKEN, Wiley, 2021, 0803-5253

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30209 Paediatrics

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 4.056

RIV identification code

RIV/00216224:14110/21:00122006

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1111/apa.16035

UT WoS

000678910800001

Keywords (in Czech)

ELBW; dietní opatření; fenylalanine; fenylketonurie; tolerance

Keywords in English

ELBW; dietary management; phenylalanine; phenylketonuria; tolerance

Abstract

ORIG CZ

V originále

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.

In Czech

Autoři popisují péči o pacienty s PKU a extrémně nízkou porodní hmotností.

Citovat

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.

@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.

Detailed Information on Publication Record