A 3 Year Longitudinal Prospective Review Examining the Dietary Profile and Contribution Made by Special Low Protein Foods to Energy and Macronutrient Intake in Children with Phenylketonuria

Abstract

The nutritional composition of special low protein foods (SLPFs) is controlled under EU legislation for ‘Foods for Special Medical Purposes (FSMP)’. They are designed to meet the energy needs of patients unable to eat a normal protein containing diet. In phenylketonuria (PKU), the macronutrient contribution of SLPFs has been inadequately examined. Aim: A 3-year longitudinal prospective study investigating the contribution of SLPFs to the macronutrient intake of children with early treated PKU. Methods: 48 children (27 boys) with a mean recruitment age of 9.3 y were studied. Semi-quantitative dietary assessments and food frequency questionnaires (FFQ) were collected three to four times/year for 3 years. Results: The mean energy intake provided by SLPFs was 33% (SD ± 8), and this figure was 42% (SD ± 13) for normal food and 21% (SD ± 5) for protein substitutes (PS). SLPFs supplied a mean intake of 40% carbohydrate (SD ± 10), 51% starch (SD ± 18), 21% sugar (SD ± 8), and 38% fat (SD ± 13). Fibre intake met 83% of the Scientific Advisory Committee on Nutrition (SACN) reference value, with 50% coming from SLPFs with added gums and hydrocolloids. Low protein bread, pasta and milk provided the highest energy contribution, and the intake of sweet SLPFs (e.g., biscuits, cakes, and chocolate) was minimal. Children averaged three portions fruit/vegetable daily, and children aged ≥ 12 y had irregular meal patterns. Conclusion: SLPFs provide essential energy in phenylalanine restricted diets. Optimising the nutritional quality of SLPFs deserves more attention.

Exploring the Barriers and Motivators to Dietary Adherence among Caregivers of Children with Disorders of Amino Acid Metabolism (AAMDs): A Qualitative Study

“…It is now well established from a variety of studies that individualized dietary therapy plays an important role in the treatment of AAMD, as it is characterized by a disruption of the metabolism of amino acids, which are the main constituents of the daily diet [6,7]. The current treatment for this group of disorders includes (a) the provision of a suitable protein substitute free of the offending substrate; (b) a lifelong protein restriction of natural protein intake through the exclusion of all high protein food, such as eggs, milk, cheese, meat, poultry, fish, dried beans, and legumes; and (c) the provision of special low-protein food to meet the energy requirement [8,9]. The daily requirement and allocation of natural protein for each individual are calculated and titrated according to the longitudinal metabolic control and the plasma essential amino acid level over time [10].…”

The Impact of the Quality of Nutrition and Lifestyle in the Reproductive Years of Women with PKU on the Long-Term Health of Their Children

“…The habitual intake of meat, fish, dairy products, wholegrain cereals, and nuts and seeds is unknown but thought to be minimal. It is established that teenagers commonly eat high amounts of carbohydrates with a limited intake of fruit and vegetables [ 44 ], despite extensive dietary education.…”

“…In a Phe-restricted diet, many of the carbohydrate sources allowed are based on simple sugars, e.g., sucrose and fructose, and this may cause rapid deregulation in the composition of the gut microbiota and, hence, metabolic dysfunction in the host [ 91 ]. Although some SPLFS contain added fibre, it is usually in the form of hydrocolloids to help their structure rather than provide nutritional benefits [ 11 , 44 ]. There is evidence that patients with PKU may have dysbiosis with less variety of bacteria, which may interfere with an optimal metabolism [ 92 ].…”

Breast Milk: A Source of Functional Compounds with Potential Application in Nutrition and Therapy

“…Κ-casein, as well as its glycomacropeptide hydrolyzate (GMP), are bound to complex oligosaccharides that, similar to mucosal glucans, can bind to pathogens [ 105 ]. GMP contains very low levels of phenylalanine and is used in food products for the nutritional management of children with phenylketonuria [ 107 , 108 ]. Beyond its nutritional utility, GMP has been tested to reduce glycemic response with good results in adults [ 109 ].…”