Use of Low-Protein Enriched Starch Products in Diet Therapy of Children With Phenylketonuria Aged Over One Year

Background. The nutrition of children with phenylketonuria includes specialized starch-based products, the range of which is constantly expanding. Our aim was to study the safety of the composition of starchy flakes enriched with a complex of fat-soluble vitamins, natural fruit and berry additives used in the food of children with phenylketonuria. Methods. The study included children under the age of 14 years who were compliant with the previously conducted hypophenylalanine diet, without acute infectious, severe somatic or neurological diseases. The investigated products (starch-rye, wheat, and wheat fruit flakes with a complex of provitamin A and vitamin E) were prescribed instead of previously used low-protein confectionery products in the amount of 20–25 g/day for children under 6 years, 30–40 g — for children aged 6 years and over. The products were given with the recommendation to use alternately, with a duration of at least 10 days, totally for 30 days of the study. The safety of the products was assessed by phenylalanine concentration in the blood (determined by the fluorimetric method). In addition, we assessed the organoleptic qualities of the products and the dynamics of physical development of children. Results. The study included 15 children, mean age 4.4 ± 1.9 years. The initial concentration of phenylalanine in the blood varied from 1.6 to 3.9 mg%, the median — 2.2 mg% (2.0; 2.8). In 30 days after inclusion of starchy flakes in the diet, the content of phenylalanine in the blood did not change and was 2.5 mg% (2.2; 2.7); p = 0.859. The organoleptic properties of the products were rated «excellent» by all patients and their parents (in children under 6 years, only according to the parents’ assessment). The indicators of physical development did not change. There was no adverse events (allergic reactions, dyspepsia, refusal to take food). Conclusion. Introduction of new functional products — low-protein starchy flakes enriched with a vitamin complex and natural fruit and berry additives — in the diet of children with phenylketonuria allows to maintain the level of phenylalanine in the blood at the level of reference values.

1. Fenilketonuriya i narusheniya obmena tetragidrobiopterina u detei. Klinicheskie rekomendatsii. Moscow: Soyuz pediatrov Rossii, Assotsiatsiya meditsinskikh genetikov; 2017. (In Russ).] Доступно по: http://pediatr-russia.ru/news/recomend

2. Novikov PV. Neonatal’nyi skrining nasledstvennykh boleznei obmena veshchestv i ego perspektivy v Rossiiskoi Federatsii. Spravochnik zaveduyushchego KDL. 2014;(2):24–36. (In Russ).

3. Dietoterapiya pri nasledstvennykh boleznyakh aminokislotnogo obmena. Metodicheskoe pis’mo. Moscow: 2013; 97 p. (In Russ).

4. Methodical recommendations MP 2.3.1.2432-08 «Normy fiziologicheskikh potrebnostei v energii i pishchevykh veshchestvakh dlya razlichnykh grupp naseleniya Rossiiskoi Federatsii» (utv. Glavnym gosudarstvennym sanitarnym vrachom RF 18 dekabrya 2008). (In Russ).

5. Spetsializirovannye produkty lechebnogo pitaniya dlya detei s fenilketonuriei. (Metodicheskoe pis’mo). 3 rd ed, updated and revised. Moscow; 2012. 85 p. (In Russ).

6. Bykova ST, Buravleva TN, Kalinina TG, et al. Use of inulin in dietary baby nutrition. Food processing industry. 2014;(2):32–33. (In Russ).

7. Bykova ST, Buravleva TN, Kalinina TG, et al. Nutritional composition for baby food preparation. Food processing industry. 2013;(12):11–14. (In Russ).

8. Borovik TE, Bushueva TV, Voznesenskaya TS, et al. Klinicheskaya dietologiya. Rukovodstvo dlya vrachei. 2nd ed, updated and revised. Moscow: MIA; 2015. 720 p. (In Russ).

9. Bushueva TV, Freidlin EV, Kuzenkova LM, et al. Osobennosti immunnogo statusa limfotsitov perifericheskoi krovi u bol’nykh fenilketonuriei. Russ J Immunol. 2015;9(2):136–138. (In Russ).

10. Bushueva TV, Vinyarskaya IV, Chernikov VV, et al. Assessment of the life quality in children with phenylketonuria. Annals of the Russian academy of medical sciences. 2014;69(11–12):39–45. (In Russ).] doi: 10.15690/ vramn.v69i11-12.1181.