Использование низкобелковых обогащенных крахмаломучных продуктов в диетотерапии больных фенилкетонурией детей в возрасте старше 1 года

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.Обоснование. В питании детей с фенилкетонурией широко используют специализированные продукты на основе крахмалов, ассортимент которых постоянно расширяется.Цель исследования — изучить безопасность состава хлопьев крахмаломучных, обогащенных комплексом жирорастворимых витаминов, натуральными плодовыми и ягодными добавками, используемых в пище детей с фенилкетонурией.Методы. В исследование включали детей в возрасте до 14 лет, комплаентных к ранее проводимой гипофенилаланиновой диете, без острых инфекционных, тяжелых соматических или неврологических заболеваний. Исследуемые продукты — крахмалоржаные, пшеничные и пшеничные плодово-ягодные хлопья с комплексом провитамина А и витамина Е — назначали взамен применявшихся ранее низкобелковых кондитерских изделий в количестве 20–25 г/сут детям младше 6 лет, по 30–40 г — детям, достигших возраста или старше 6 лет. Продукты выдавали с рекомендацией использовать поочередно, продолжительностью не менее 10 сут, всего на 30 сут исследования. Безопасность продуктов оценивали по концентрации фенилаланина в крови (определяли флюориметрическим методом). Дополнительно оценивали органолептические качества продуктов и динамику физического развития детей.Результаты. В исследование включили 15 детей, средний возраст 4,4±1,9 года. Исходная концентрация фенилаланина в крови варьировала от 1,6 до 3,9 мг%, медиана — 2,2 мг% (2,0; 2,8). Через 30 сут после включения в рацион крахмаломучных хлопьев содержание фенилаланина в крови не изменилось и составило 2,5 мг% (2,2; 2,7); р=0,859. Органолептические свойства продуктов были оценены на «отлично» всеми пациентами и их родителями (у детей в возрасте до 6 лет — только согласно оценке родителей). Показатели физического развития не изменились. Нежелательные явления (аллергические реакции, диспепсии, отказ от приема продуктов) не зафиксированы.Заключение. Введение в рацион детей с фенилкетонурией новых функциональных продуктов — хлопьев крахмаломучных низкобелковых, обогащенных витаминным комплексом и натуральными плодовыми и ягодными добавками, позволяет сохранять уровень фенилаланина в крови на уровне референсных значений.ИСТОЧНИК ФИНАНСИРОВАНИЯ Работа выполнена при поддержке гранта Федерального государственного научного учреждения «Всероссийский научно-исследовательский институт крахмалопродуктов» Федерального агентства научных организаций (Московская область). Для целей исследования использовались продукты, безвозмездно предоставленные производителем (опытное производство ФГНУ «ВНИИК» ФАНО).КОНФЛИКТ ИНТЕРЕСОВ Т.Э. Боровик, Н.Н. Семёнова, О.Л. Лукоянова, Н.Г. Звонкова, Т.В. Бушуева, Т.Н. Степанова, В.А. Скворцова — проведение научно-исследовательских работ при поддержке компаний Heinz, Semper, Хипрока Нутришион Ист Лимитед. И.М. Гусева, Е.А. Рославцева, А.К. Геворкян, С.Т. Быкова, Т.Г. Калинина, С.Г. Калиненкова подтвердили отсутствие конфликта интересов.ВЫРАЖЕНИЕ ПРИЗНАТЕЛЬНОСТИ Выражаем благодарность к.м.н. С.Г. Калиненковой (Московский областной научно-исследовательский клинический институт им. М.Ф. Владимирского) за участие в выполнении лабораторной части данного исследования. 

Метилмалоновая ацидурия у детей: клинические рекомендации

Methylmalonic acidemia (aciduria) is an inherited metabolic disturbance from the group of organic acidemias (acidurias). The article presents etiopathogenetic, epidemiological, diagnostic, and therapeutic aspects of the problem. The possibilities of laboratory and instrumental diagnostic methods the tactics of dietary correction of metabolic disorders in acute and interstitial periods of the disease are described in details; features of drug treatment are outlined. The necessary information for clinical practice and patients’ everyday life is given in the article.Метилмалоновая ацидемия (ацидурия) — генетически гетерогенное наследственное заболевание группы органических ацидемий (ацидурий). В статье представлены этиопатогенетические, эпидемиологические, диагностические и терапевтические аспекты данной проблемы. Подробно освещены возможности лабораторных и инструментальных методов диагностики и особенности медикаментозного лечения, изложена тактика диетической коррекции метаболических нарушений в острый и межприступный периоды заболевания. Дана необходимая информация для практических врачей и родителей пациентов

Reconsidered Concept of «Prebiotics» and Their Impact on the Child’s Organism

Detection of a large number of oligosaccharides in breast milk predetermined their use in the composition of infant formula. The most studied prebiotic simulating the unique properties of breast milk oligosaccharides is the scGOS/lcFOS mixture. Its efficacy is confirmed in clinical studies. In 2017, the experts of the  International Scientific Association of Probiotics and Prebiotics  (ISAPP) adopted a new definition for «prebiotics», which greatly  expanded the concept of their application. The World Allergy  Organization (WAO) recommends the use of prebiotics in allergy- prone children. The guidelines are based on the results of studies of the influence of prebiotics on the intestinal microbi, a violation of the composition and functions of which predisposes to the  development of allergy. Currently, the search for more promising  technologies for the production of prebiotics continues

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

Methylmalonic Aciduria in Children: Clinical Recommendations

Methylmalonic acidemia (aciduria) is an inherited metabolic disturbance from the group of organic acidemias (acidurias). The article presents etiopathogenetic, epidemiological, diagnostic, and therapeutic aspects of the problem. The possibilities of laboratory and instrumental diagnostic methods the tactics of dietary correction of metabolic disorders in acute and interstitial periods of the disease are described in details; features of drug treatment are outlined. The necessary information for clinical practice and patients’ everyday life is given in the article

Homocystinuria in Children

Homocystinuria is a genetically heterogeneous hereditary disease from the group of aminoacidopathies caused by a metabolic disorder of  sulphur-containing amino acids, primarily methionine. The article  presents the etiopathogenetic, diagnostic and therapeutic aspects of this disease and covers modern opportunities of biochemical and molecular  diagnostics. The approach to dietary and pharmacological correction of  metabolic disorders in homocystinuria and the general strategy of  patients’ management are described in detail. Important information is given for physicians of various disciplines and parents of patients

EFFICIENCY OF USING THE ADAPTED GOAT’S MILK FORMULA IN THE DIET OF HEALTHY YOUNG INFANTS: A MULTICENTER PROSPECTIVE COMPARATIVE STUDY

Background. There is no doubt that it is necessary to study the efficiency of milk formulas that are introduced into the Russian market of baby food. This applies to both new products and known brands of formulas whose composition is subject to change.Objective. Our aim was to assess the clinical efficacy of the adapted goat's milk formula in the diet of young infants.Methods. We conducted a prospective comparative study with healthy full-term  children aged 0–5 months being on a formula (main group) or breast feeding (comparison group). The tolerability of the adapted goat's milk formula, the dynamics of anthropometric indicators, changes in body composition as well as microscopic characteristics  of stool and general clinical and biochemical parameters  of peripheral blood were assessed after 1 month.Results. Good tolerability of the goat's milk formula was noted in 184 (96.8%) of 190 children in the main group. In the course of taking the product, the proportion of children with functional disorders of the gastrointestinal tract decreased significantly from 57 (30%) to 27 (14%) (p < 0.001). Physical development,  complete blood count results, the levels of ferritin, prealbumin and 25(OH)D in children of the main group and the comparison group (n = 71) were comparable and were within the mean age parameters. Qualitative analysis of the level of specific IgE to goat's milk proteins did not reveal any sensibilization in any of the children receiving the milk formula, either at the beginning of the study or after 1 month of taking the product.Conclusion. The studied adapted goat's milk formula can be used in nutrition of young infants in cases of lack or absence of mother's milk