Extremely low birthweight neonates with phenylketonuria require special dietary management
| Autor: | Marketa Zemanova, Petra Sanakova, Petr Chrastina, Helena Jahnová, Barbara Tesarova, Jiri Zeman, Lucie Prochazkova, Dagmar Procházková, Vaclav Sebron |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Parenteral Nutrition
Pediatrics medicine.medical_specialty Phenylalanine Birth weight Enteral administration Screening programme 03 medical and health sciences Neonatal Screening 0302 clinical medicine Phenylketonurias 030225 pediatrics Birth Weight Humans Medicine 030212 general & internal medicine business.industry Infant Newborn Dietary management Infant General Medicine Protein intake 3. Good health Parenteral nutrition Pediatrics Perinatology and Child Health medicine.symptom business Weight gain |
| Zdroj: | Acta Paediatrica. 110:2994-2999 |
| ISSN: | 1651-2227 0803-5253 |
| DOI: | 10.1111/apa.16035 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text