Breastfeeding in Phenylketonuria: Changing Modalities, Changing Perspectives

Zuvadelli, Juri; Paci, Sabrina; Salvatici, E.; Giorgetti, Federica; Cefalo, Graziella; Dionigi, Alice Re; Rovelli, Valentina; Banderali, Giuseppe

doi:10.3390/nu14194138

Cited by 4 publications

(4 citation statements)

References 33 publications

(42 reference statements)

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“…All infant formulas must be safe and suitable to satisfy the nutritional requirements and promote appropriate growth and development. In infants with PKU, even if human milk or standard infant formulas can be used alongside infant Phe-free protein substitutes, to date, there are no specific indications about the proportions of the two modalities or the effects on disease control and infant growth [ 50 ]. For this reason, infant PS for PKU available on the Italian market should be the best possible alternative to exclusive maternal breastfeeding, providing the nutrient profile as well as the structural and functional effects are as similar as possible to those observed in exclusively breastfeeding or standard infant formulas.…”

Section: Discussionmentioning

confidence: 99%

Are Phe-Free Protein Substitutes Available in Italy for Infants with PKU All the Same?

Verduci,

Tosi,

Montanari

et al. 2023

Nutrients

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Breastfeeding or standard infant formulas, alongside phenylalanine (Phe)-free protein substitutes, constitute the dietary management for infants with PKU to guarantee protein requirements are met in compliance with metabolic tolerance. This work aims to analyse the nutritional composition of Phe-free infant protein substitutes, in terms of macronutrients, micronutrients and functional components, available for PKU dietary management in Italy. A total of seven infant Phe-free protein substitutes were included in this review, six powder and one liquid. A second analysis was conducted to compare them to the composition of formulas intended for healthy infants, taking into consideration the Commission Delegated Regulation (EU) 2016/127 and Commission Delegated Regulation (EU) 2016/128 for micronutrients. The analysis revealed heterogeneity among protein substitutes suitable for infants with PKU. The energy and protein equivalents (P.Eq.) content are different; all of the substitutes contain docosahexaenoic acid (DHA) and arachidonic acid (ARA), while eicosapentaenoic acid (EPA), fructo-oligosaccharides (FOS), galacto-oligosaccharides (GOS), human milk oligosaccharides (HMOs) and nucleotides are not present in all the substitutes. More attention should be paid to these infant products to ensure metabolic control of PKU, and also promote proper growth, cognitive neurodevelopment, favourable gut microbiota composition, and immune system health, while reducing the risk for non-communicable diseases (NCDs).

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Section: Discussionmentioning

confidence: 99%

Are Phe-Free Protein Substitutes Available in Italy for Infants with PKU All the Same?

Verduci,

Tosi,

Montanari

et al. 2023

Nutrients

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“…It is good practice to encourage parents to keep a record of daily feeds [ 32 , 33 ]. The method of administering SF-Phe before breastfeeding has shown that infants with PKU continue to breastfeed for an older age of HM compared to the alternative methods of alternating HM and SF-Phe or expressing milk and measuring the amount administered [ 34 , 35 ].…”

Section: Discussionmentioning

confidence: 99%

Breastfeeding and Inborn Errors of Amino Acid and Protein Metabolism: A Spreadsheet to Calculate Optimal Intake of Human Milk and Disease-Specific Formulas

et al. 2023

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Human milk (HM) offers important nutritional benefits. However, except for phenylketonuria (PKU), there are little data on optimal levels of consumption of HM and a special formula free of disease-related amino acids (SF-AA) in infants with inborn errors of metabolism of amino acids and proteins (IEM-AA-P). We designed a spreadsheet to calculate the amounts of SF-AA and HM required to cover amino acid, protein, and energy needs in patients with the nine main IEM-AA-P in infants aged under 6 months. Upon entering the infant’s weight and the essential amino acid or intact protein requirements for the specific IEM, the spreadsheet calculates the corresponding required volume of HM based on the amino acid concentration in HM. Next, the theoretical daily fluid intake (typical range, 120–200 mL/kg/day) is entered, and the estimated daily fluid intake is calculated. The required daily volume of SF-AA is calculated as the difference between the total fluid intake value and the calculated volume of HM. The spreadsheet allows for the introduction of a range of requirements based on the patient’s metabolic status, and includes the option to calculate the required volume of expressed HM, which may be necessary in certain conditions such as MMA/PA and UCD. In cases in which breastfeeding on demand is feasible, the spreadsheet determines the daily amount of SF-AA divided over 6–8 feeds, assuming that SF-AA is administered first, followed by HM as needed. Intake data calculated by the spreadsheet should be evaluated in conjunction with data from clinical and nutritional analyses, which provide a comprehensive understanding of the patient’s nutritional status and help guide individualized dietary management for the specific IEM.

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“…Acceptable blood Phe control and growth are possible with its use [6]. However, breastfeeding rates and duration of feeding in infants with PKU remain lower than in the general population [7]. There is limited research investigating the maternal experience of BF an infant with PKU.…”

Section: Introductionmentioning

confidence: 99%

A Retrospective Chart Review and Infant Feeding Survey in the Irish Phenylketonuria (PKU) Population (2016–2020)

Rice,

McNulty,

O’Shea

et al. 2023

Nutrients

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Phenylketonuria (PKU) is an inherited disorder of protein metabolism. It is generally treated using dietary management with limited intake of phenylalanine (Phe). Partial breastfeeding (BF) is encouraged among mothers of infants with PKU, together with a Phe-free mixture of synthetic amino acids. Our aim was to describe our current BF rates and complementary feeding practices, as well as examining parental experiences of infant feeding. The objective was to better understand the challenges faced by families so that improvements can be made to clinical care. A chart review was carried out on 39 PKU patients, examining the BF rate and duration, use of second stage synthetic protein (SP), and average complementary feeding age. A parental questionnaire on complementary feeding and BF experience was designed: 26% of babies were partially breastfed at three months of age; 70% of mums would like to have breastfed for longer and cited PKU as a reason for stopping; 52% of parents reported challenges during the complementary feeding process including food refusal, protein calculation, and anxiety around maintaining good Phe levels. Suggestions to improve BF continuation and duration include active promotion of the benefits and suitability, access to lactation consultant, and peer support. The delay in introducing a second stage SP may contribute to long-term bottle use for SP. Improved patient education, written resources, and support is necessary to improve food choices and long-term acceptance of SP.

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Breastfeeding in Phenylketonuria: Changing Modalities, Changing Perspectives

Cited by 4 publications

References 33 publications

Are Phe-Free Protein Substitutes Available in Italy for Infants with PKU All the Same?

Are Phe-Free Protein Substitutes Available in Italy for Infants with PKU All the Same?

Breastfeeding and Inborn Errors of Amino Acid and Protein Metabolism: A Spreadsheet to Calculate Optimal Intake of Human Milk and Disease-Specific Formulas

A Retrospective Chart Review and Infant Feeding Survey in the Irish Phenylketonuria (PKU) Population (2016–2020)

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