Challenges to Breastfeeding Infants With Phenylketonuria

Banta-Wright, Sandra A.; Kodadek, Sheila M.; Steiner, Robert D.; Houck, Gail M.

doi:10.1016/j.pedn.2014.05.003

Cited by 19 publications

(8 citation statements)

References 29 publications

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“…33,34 This suggests that mothers who are breastfeeding infants with PKU may need more support during the early weeks after diagnosis and again when lowPhe table foods are introduced at around 6 months. Although some barriers and challenges to breastfeeding infants with PKU 35 have been identified, the role that metabolic clinics play in the continuation or discontinuation of breastfeeding infants with PKU should be investigated further. This study has several limitations and strengths.…”

Section: Discussionmentioning

confidence: 99%

Breastfeeding Infants with Phenylketonuria in the United States and Canada

Banta-Wright

Press

Knafl

et al. 2014

Breastfeeding Medicine

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Objective: This study described the prevalence and duration of mothers' breastfeeding infants with phenylketonuria (PKU) and explored factors related to duration of breastfeeding as a surrogate for breastfeeding success. Subjects and Methods: Descriptive analysis as performed from an international Internet survey of mothers (n = 103) who met the inclusion criteria: (1) at least 21 years of age, (2) able to read and write in English, (3) child with PKU, and (4) living in the United States or Canada. Results: Of the 103 mothers, 89 (86%) initiated breastfeeding immediately following delivery, whereas 14 (14%) chose bottle feeding. In comparison to breastfeeding after delivery, significantly fewer mothers breastfed after diagnosis (McNemar's v 2 = 30.33, p < 0.001; n = 72 vs. n = 89). Breastfeeding duration ranged from less than 1 month to 24 months with one modal duration category (n = 20, 22%) at less than 1 month. The timing of the addition of commercial infant formula to supplement breastfeeding or expressed mothers' milk was associated with a shorter duration of breastfeeding among infants with PKU: v 2 (42, n = 73) = 88.13, p < 0.001. Conclusions: PKU is treated with phenylalanine (Phe) restriction. Breastfeeding infants with PKU is challenging in part because Phe intake is difficult to determine precisely. We studied breastfeeding duration in infants with PKU and factors associated with success. Further research should identify the unique needs of mothers' breastfeeding infants with PKU to guide the development of interventions specific to these mothers to support their efforts to continue breastfeeding after the diagnosis of PKU.

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Section: Discussionmentioning

confidence: 99%

Breastfeeding Infants with Phenylketonuria in the United States and Canada

Banta-Wright

Press

Knafl

et al. 2014

Breastfeeding Medicine

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“…This enzyme is defective in people with phenylketonuria. It leads to an increase of phenylalanine in the blood and the brain (1). High levels of phenylalanine in the plasma cause mental retardation, seizures, behavioural difficulties, motor delay and slow language development.…”

Section: Introductionmentioning

confidence: 99%

Health-related quality of life of parents of children with phenylketonuria in Tehran Province, Islamic Republic of Iran

Etemad¹,

Heidari²,

Setoodeh³

et al. 2020

East Mediterr Health J

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Background: Parents of children with phenylketonuria are at risk of reduced quality of life. Aims: This study determined the quality of life of parents of children with phenylketonuria in Tehran Province. Methods: The study was conducted in 2015 and included parents of children with phenylketonuria referred to three government children's hospitals in Tehran Province that provide phenylketonuria services. Data were collected using the Farsi version of the World Health Organization Quality of Life-Bref questionnaire. Analysis of variance, t-test, Pearson correlation coefficient and multiple linear regression were used to assess the relationship between quality of life domains and sociodemographic characteristics of the parent and child. Results: The study included 240 parents; 55% were mothers. Quality of life of parents in psychological, social relationships and environment domains was low. Significant relationships were found between: physical domain and age of child at phenylketonuria diagnosis (P = 0.044); psychological domain and parent's age (P = 0.019), child's age (P = 0.007) and parent's education (P = 0.015); social relationships domain and parent's age (P = 0.003), and education (P = 0.002), household income (P = 0.025) and child's age (P = 0.004; and environmental domain and residence (P = 0.034), parent's education (P = 0.007), household income (P = 0.002) and child's age (P = 0.049). In the multivariable analysis, parent's age and education, child's age, and household income were significantly associated with parent's quality of life. Conclusion: Given the low levels of quality of life in the parents, education and more financial support are recommended.

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“…En los primeros meses de vida, la alimentación del paciente se realizará con alimento o fórmula médica sin PHE más una fuente de PHE, la cual será leche materna como primera opción y si esto no fuera posible, se optará por una fórmula infantil de inicio. 12 El alimento médico sin PHE para PKU El tratamiento nutricional requiere del uso de una fórmula sin PHE o también llamada alimento médico, que proporcione al paciente las proteínas que necesita además de otros macro y micro nutrimentos. 5 Existen nuevas opciones de aporte proteico sin PHE o con pocas cantidades de éste, como es el glicomacropéptido y los aminoácidos largos neutros; los cuales son terapias actualmente utilizadas en otros países con buenos resultados y que generalmente se usan en niños y adolescentes.…”

Section: Tratamiento De La Fenilcetonuria Al Diagnósticounclassified

¿Qué aspectos considerar al iniciar el tratamiento nutricional para Fenilcetonuria?

Mejía¹,

Vázquez²,

Guillén-López³

2018

Acta Pediatr Mex

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La fenilcetonuria clásica (PKU) es una enfermedad genética en la cual el metabolismo del aminoácido esencial fenilalanina (PHE), se ve obstaculizado por la deficiencia de una enzima llamada fenilalanina hidroxilasa, como consecuencia de este bloqueo los niveles de PHE se acumulan en sangre y otros tejidos, causando daños graves e irreversibles a nivel del sistema nervioso central. El objetivo del tratamiento nutricional consiste en proporcionar una cantidad específica de PHE y al mismo tiempo dar la cantidad de proteína que requiere el paciente para crecer y evitar deficiencias asociadas a la eliminación de alimentos altos en proteína, además de mantener un funcionamiento mental adecuado. El tratamiento nutricional inicialmente, requiere del uso de una fórmula sin PHE, que provea al paciente las proteínas que necesita además de vitaminas y nutrimentos inorgánicos. En los pacientes con PKU es necesario ofrecer lactancia materna, sin embargo no puede ser de forma exclusiva ya que debe combinarse con una fórmula libre PHE.PALABRAS CLAVE: Fenilcetonuria, terapia nutricional, tamiz neonatal, lactancia materna.

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Challenges to Breastfeeding Infants With Phenylketonuria

Cited by 19 publications

References 29 publications

Breastfeeding Infants with Phenylketonuria in the United States and Canada

Breastfeeding Infants with Phenylketonuria in the United States and Canada

Health-related quality of life of parents of children with phenylketonuria in Tehran Province, Islamic Republic of Iran

¿Qué aspectos considerar al iniciar el tratamiento nutricional para Fenilcetonuria?

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