Breastfeeding in infants diagnosed with phenylketonuria

Chong, L.Y.; Kalvala, Jahnavi; Chadborn, Neil; Ojha, Shalini

doi:10.1002/14651858.cd015243

Cited by 2 publications

(4 citation statements)

References 15 publications

(8 reference statements)

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“…We performed this scoping review with a similar protocol as our review protocol for a systematic review 4 for the same research question, except in the plan to include all study types and not restrict the review to RCTs as was previously done. We included all studies where the participants were infants diagnosed with PKU (via newborn screening programme or later) in the first 6 months after birth and their mothers.…”

Section: Methodsmentioning

confidence: 99%

“…We followed a similar search strategy as performed for our systematic review with the Cochrane Collaboration. 4 We searched the following databases and trial registries: MEDLINE Ovid (1946 to present); Embase Ovid (1974 to present); US National Institutes of Health Ongoing Trials Register ClinicalTrials.gov ( www.clinicaltrials.gov ); WHO International Clinical Trials Registry Platform ( trialsearch.who.int/ ). We checked the bibliographies of included studies and any relevant systematic reviews identified for further references to relevant trials.…”

Section: Methodsmentioning

confidence: 99%

“…Our previous systematic review searched for randomised controlled trials (RCT) and found zero publications. 4 We now aim to conduct a scoping review inclusive of ‘non-RCT’ (or a broad range of study types) literature to assess the effects of breast feeding (exclusive or partial) compared with feeding low-Phe formula milk only in infants with PKU in the first 6 months after birth.…”

Section: Introductionmentioning

confidence: 99%

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Breast feeding in infants diagnosed with phenylketonuria (PKU): a scoping review

Kalvala,

Chong,

Chadborn

et al. 2023

bmjpo

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BackgroundPhenylketonuria (PKU) is the most common inherited disease of amino acid metabolism, characterised by elevated levels of phenylalanine (Phe). There is a lack of infant feeding guidance for those with PKU. From birth to 6 months of age, breast feeding is the optimal nutrition for an infant and continuing breast feeding for infants with PKU is recommended by European guidelines. However, human breast milk contains Phe in varying quantities, and therefore, the effects breast feeding might have on infants with PKU needs careful consideration.AimTo assess the effects of breast feeding (exclusive or partial) compared with low-Phe formula feeding in infants diagnosed with PKU, on blood Phe levels, growth and neurodevelopmental scores.MethodsThe Cochrane Inborn Errors of Metabolism Trials Register, MEDLINE and Embase were searched (date of latest search: 9 August 2022). Studies were included if they looked at the effects of breast feeding in infants diagnosed with PKU compared with formula feeding. Predetermined outcomes included blood Phe levels, growth in the first 2 years of life and neurodevelopmental scores.ResultsSeven observational studies (282 participants) met the inclusion criteria. All studies compared continuation of breast feeding with low-Phe formula versus formula feeding only. While most studies concluded that there was no difference in mean serum Phe levels in their follow-up period, two reported that breastfed infants were more likely to have a normal mean Phe level. Two studies described no difference in mean weight gain after birth, while one found that breastfed infants were more likely to have higher mean weight gain. Two studies commented that breastfed infants achieved higher developmental scores in childhood as compared with formula fed infants.ConclusionAlthough there are no randomised trials, observational evidence suggests that continuation of breast feeding and supplementation with low-Phe formula is safe and may be beneficial for infants diagnosed with PKU.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Breast feeding in infants diagnosed with phenylketonuria (PKU): a scoping review

Kalvala,

Chong,

Chadborn

et al. 2023

bmjpo

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“…Various studies on the subject have shown that medical foods with a low phenylalanine formula can improve the health status of children with PKU (Chen et al 2015;MacDonald et al 2020;Mtewa et al 2020). Specified medical food formulas that do not contain disease-causing protein derivatives should be used in other amino acid metabolism disorders such as non-ketonic hyperglycinemia, tyrosinemia, and maple syrup urine disease (McCandless et al 2021;Chong et al 2022).…”

Section: Medical Foods For Congenital Metabolic Disordersmentioning

confidence: 99%

Use of Medical Foods to Fight Chronic Diseases: A Narrative Review

Meral,

Demirdöven

2024

J Agr Sci-Tarim Bili

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Chronic diseases cannot be treated completely, and therefore often require repeated treatments. This situation leads to long-term drug utilization. Medical foods are as alternative natural drugs in the management and treatment of chronic diseases. Medical foods are specially formulated food to meet the particular nutritional requirements of patients affected by certain diseases. They play an important role in nutritional support for patients in clinical applications such as deglutition, dyspepsia or eating disorders. They considerably enhance the quality of living of patients by reducing drug usage, preventing complications with overuse of drug, and reducing the expenses of treatments. The nutritive value of medical foods can be regulated and personalized depend on disease. They are not drugs, and are exempt from regulations applying to drugs. Each medical food is formulated specifically according to relevant chronic disease. There are no studies on medical foods for different specific diseases with examples from the market in the literature. The examination and compilation of medical foods in an article with examples from the market is all important in terms of both creating new products and filling the gap in the relevant literature. Therefore, the aim of this review is to explain the use of medical foods for Alzheimer, Parkinson, anxiety and sleep disorder, pain syndrome, cancer, infants, inborn errors metabolism, diabetes mellitus, drug-resistant epilepsy, hypertension and indicate why should be preferred instead of drugs. Research on new medical foods should be increased and new formulations should be created.

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Breastfeeding in infants diagnosed with phenylketonuria

Abstract: To assess the e ects of breastfeeding (exclusive or partial) compared to low-Phe formula feeding in the first six months a er birth in infants diagnosed with PKU.

Cited by 2 publications

References 15 publications

Breast feeding in infants diagnosed with phenylketonuria (PKU): a scoping review

Breast feeding in infants diagnosed with phenylketonuria (PKU): a scoping review

Use of Medical Foods to Fight Chronic Diseases: A Narrative Review

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