[All-Russian Consensus on Diagnosis and Treatment of Celiac Disease in Children and Adults]. / Vserossiiskii konsensus po diagnostike i lecheniyu tseliakii u detei i vzroslykh.

The paper presents the All-Russian consensus on the diagnosis and treatment of celiac disease in children and adults, which has been elaborated by leading experts, such as gastroenterologists and pediatricians of Russia on the basis of the existing Russian and international guidelines. The consensus approved at the 42nd Annual Scientific Session of the Central Research Institute of Gastroenterology on Principles of Evidence-Based Medicine into Clinical Practice (March 2-3, 2016). The consensus is intended for practitioners engaged in the management and treatment of patients with celiac disease. Evidence for the main provisions of the consensus was sought in electronic databases. In making recommendations, the main source was the publications included in the Cochrane Library, EMBASE, MEDLINE, and PubMed. The search depth was 10 years. Recommendations in the preliminary version were reviewed by independent experts. Voting was done by the Delphic polling system.

[Nutritional management of kidney diseases in children].

The prevalence of various kidney diseases in children remains high in recent decades. Adequate nutrition management can enhance the effectiveness of drug treatment, slow the frequency of relapses andprevent the progression of the disease. The article is devoted to modern approaches to diet therapy in various kidney diseases in children with the defeat of tubular and glomerular appa ratus. For the first time the therapeutic diets for children with various kidney diseases are presented. Particular attention is paid to diet therapy in nephrotic syndrome (steroid-responsive and steroid-refractory). Dietary approaches with modern formulas for enteral nutrition in cases of steroid therapy complications in children with renal insufficiency (in predialysis stage and on dialysis) are described. Differentiated nutritional approaches for patients with different types of crystalluria are separately presented.

Characteristics of the nutrition organization and nutrient consumption in toddlers' living in the Central Federal District, Russian Federation (Moscow, Ivanovo, Yaroslavl).

A multicenter cross-sectional study with the participation of 356 healthy children aged 1 to 3 years from the cities of the Central Federal District: Moscow (n=106), Ivanovo (n=126), Yaroslavl (n=124) has been carried out. Questionnaire method, the method of 24-hour diet recall for 3 days (2 weekdays and 1 weekend) and the method of diary recording of food intake were used. The average daily volumes of food were consistent with the recommended age norms only in 31.2% of the surveyed children; after 1 year of life children actively started to transfer to nutrition from the family table and only 51.7% of children continued to receive individually prepared for baby food. The recommended dietary allowances were inadequate in macro- and micronutrients: 48.6% of all children received excessive amounts of protein and fat, and 74.1% were less in carbohydrates. The consumption of vitamins D and A and minerals (calcium, iron and zinc) did not correspond to existing recommendations in the majority of cases: only 10-30% of the examined children received physiological norms of vitamins from food and 13-34% - some minerals and trace elements. These results indicate that the nutrition of young children living in the Central Federal District is imbalanced on daily caloric, macro- and micronutrient composition, does not fully meet the requirements of the balanced diet and needs serious optimization.

[Nutritional epigenetics and epigenetic effects of human breast milk].

The article provides an overview of the current literature on nutritional epigenetics. There are currently actively studied hypothesis that nutrition especially in early life or in critical periods of the development, may have a role in modulating gene expression, and, therefore, have later effects on health in adults. Nutritional epigenetics concerns knowledge about the possible effects of nutrients on gene expression. Human breast milk is well-known for its ability in preventing necrotizing enterocolitis, infectious diseases, and also non-communicable diseases, such as obesity and related disorders. This paper discusses about presumed epigenetic effects of human breast milk and some its components. While evidence suggests that a direct relationship may exist of some components of human breast milk with epigenetic changes, the mechanisms involved are stillunclear.

[Evaluation of physical development in children with classical phenylketonuria].

Classical phenylketonuria (PKU) is hereditary disease, which is based on the disturbance of phenylalanine conversion to tyrosine. The basic treatment of PKU is low phenylalanine diet. Prolonged restriction of natural protein may have a negative impact to PKU patient growth and physical development. The objective was to evaluate the physical development of patients with classical PKU at birth and on the diet based on the products with different chemical composition without phenylalanine. 257 PKU patients have been examined with the computer program "WHO Anthroplus 2009". All patient were born at term. Z-score of body weight, height and body mass index (BMI) to age has been retrospectively estimated. Patients were divided into 2 groups: group 1--101 children born in 1980-1993 were fed by unadapted specialty products based on protein hydrolyzate with restricted phenylalanine, and group 2--156 children born in 1995-2012 were fed by contemporary amino acid mixtures without phenylalanine. All newborn PKU patients had the middle for age Z-score of weight and BMI, 21% of neonates had high Z-score growth. Before the diet therapy BMI Z-score was normal in 84.1% patients in group 1 and 87.2% patients of group 2. After 6 mo of treatment with low phenylalanine diet the number of patient with normal BMI Z-score was 71.3% in group 1 against 95.6% in group 2. Thus, using of modern amino acid mixtures without phenylalanine, enriched with essential nutrients can promote the normal physical development of PKU patients.

[Assessment of the life quality in children with phenylketonuria].

BACKGROUND: Phenylketonuria (PKU) - the most common inherited disorder of amino acid metabolism, identified in Russia by neonatal screening. The results of dietary treatment demonstrate a positive effect. However, the quality of PKU patients life remains unknown. OBJECTIVE: The aim of the study was to assess the quality of PKU children life in comparison with their healthy peers, also depending on the treatment onset and the patient's age. METHODS: The study involved 64 pairs - PKU child and one of his parents. It was used the common questionnaire survey Pediatric Quality of Life Inventory (PedsQLtm4. 0, Varni et al., USA, 2001) and the program SPSS v. 14.0 (US) for statistical processing of the results. RESULTS: The statistically significant (p <0.001) differences between PKU patients and healthy children in the physical, emotional, social and role functioning were found. The most pronounced differences were observed in the social (70 points vs 90 respectively) and role (57.5 vs 80 respectively) functioning. Maximum significant correlation (p <0.001) was installed between the social functioning of PKU patients and timing of the dietary treatment. Also the statistically significant interaction (p <0.05) was found between the role functioning, patient's age and treatment onset. The assessment points of social and role functioning were higher (p <0.05) in the PKU patients subgroup with early treatment initiation. CONCLUSION: These results confirmed the need of early diagnostics of PKU and initiation of dietary treatment, as well as the organization of timely psychological support for parents of sick children.

[Open, non-comparative phase III clinical study to evaluate the efficacy and safety of sapropterin in patients with phenylketonuria and hyperphenylalaninemia].

BACKGROUND: Phenylketonuria (PKU) is an autosomal recessive inherited disease associated with impaired metabolism of the amino acids phenylalanine (Phe) and tyrosine. The main criterion for diagnosis of PKU is high blood Phe level determined during neonatal screening. In case where PKU patient is responsive to tetrahydrobiopterin treatment, sapropterin restores the impaired activity of the enzyme phenylalanine hydroxylase, resulting in the stimulation of normal Phe metabolism and thereby enhancing patient tolerance to natural products. AIM: The present open, non-comparative clinical study was initiated to assess the degree and frequency of response after 8-day sapropterin administration and assess the safety of 6-week sapropterin treatment in patients with PKU and hyperphenylalaninemia. PATIENTS AND METHODS: The study enrolled 90 patients with PKU. The criterion of response to 8-day sapropterin therapy was the reduction of Phe blood levels ≥ 30% compared with the baseline value. RESULTS: Positive response to treatment was observed in 30 (33.3%) patients (95% CI 23.7-44.1). The mean percentage change in Phe blood levels after the 8-day response test period compared to Phe levels prior to dosing was 14.1 ± 28.4% in the overall subject population (95% CI 8.2-20.1) and 44.3 ± 15.1% in the subpopulation of patients with a positive response (95% CI 38.6-49.9). During the study, adverse events were reported in 24 (26.7%) patients in the overall population in 16 (53.3%) patients in the subpopulation who had a response. CONCLUSION: The study results confirmed the efficacy and safety of sapropterin therapy in patients with PKU, which is consistent with international clinical trials data.

[Lactase deficiency: somatoneurology and neurodietology].

Neurologic and somatoneurologic aspects of lactose intolerance are considered in the article. Authors stress the role of adequate diet therapy in this form of food intolerance.

[Use of dietetic canned meats in the nutrition of infants with food allergy]. / Ispol'zovanie dieticheskikh miasnykh konservov v pitanii detei rannego vozrasta s pishchevoi allergiei.

Formulas have been developed for special canned pig meat "Pig meat puree" and "Cheburashka" and horse meat "Konek-gorbunok", and a combination of pig and horse meat "Vinni Pukh", without extractives. The above canned meat produced a high therapeutic effect when it was included into the diet of infants suffering from food allergy because of sensitization to cow milk and beef proteins. The canned meat permits correcting the ration by protein component and providing 25-30% of daily protein requirement of an infant.

[Basic principles of diet therapy in food allergy in young infants]. / Osnovnye printsipy dietoterapii pri pishchevoi allergii u detei rannego vozrasta.

Altogether 101 infants aged 3 weeks to 1 year with exudative-catarrhal diathesis and apotic dermatitis were examined. Sensitization of the infants with different protein fractions of cow's milk and egg protein was studied by the passive hemagglutination test with the use of antigenic erythrocyte diagnostic agents. In the majority of exudative-catarrhal diathesis cases and in medium-severe apotic dermatitis, sensitization with 2-3 antigens was noted, while in severe atopic dermatitis, sensitization with 4-5 antigens was recorded in over 40% of cases. On the basis of the data obtained there have been developed scientifically validated principles of dietetics for infants suffering from food allergy.

[A clinical and experimental study of the food and biological value of dietetic canned chicken meat for children]. / Kliniko-éksperimental'noe izuchenie pishchevoi i biologicheskoi tsennosti detskikh dieteticheskikh konservov iz miasa tsypliat.

The results are presented of clinico-experimental investigations of 4 different variants of dietetic canned chicken meat for children. The products were enriched with blood stimulants. Certain clinical and hematological parameters were used for the evaluation of the effectiveness of the canned chicken meat. The results obtained have evidenced that the products studied are tolerated well and produce a pronounced antianemic effect.

[Specialized products for nutrition of children with mucoviscidosis]. / Spetsializirovannye produkty dlia pitaniia detei, bol'nykh mukovistsidozom.

Mucoviscidosis is serious inherited disease with lesions of almost all secretory cells of mucosa and exocrine glands, following by disturbances of metabolism, digestion and nutritional status. Using of modern high effective pancreatic enzymes does not allow to reach a normalization of protein metabolism and digestion especially of fats. For success treatment of this disease the effective dietetic methods should be used including dietary intake of special food products which can supply enough energy and nutrients in small volume. The nutrient contents of a few foreign substitution of breast milk and food product for older children are given. The results of treatment of 86 children suffering from mucoviscidosis are presented. Milk proteins (casein, whey proteins), hydrolysates of milk protein and soy protein isolates are main resources of protein component in special food. Fat components are produced from middle-chain triglycerides (60-70%) and vegetable oils. Carbohydrates in special food are made from glucose polymers, glucose, fructose and sucrose. Positive results of clinical trials of foreign special food have been an example for creation of two home-produced food for treatment of children with mucoviscidosis: 1) adapted version "A" for children in age under 1 year, and 2) version "B" for older children. The products were created on basis of native milk protein with ratio casein to whey protein as 60:40. Middle-chain triglycerides compose 70-75% of fats. Carbohydrates are presented by dextrin-maltose (85%), glucose and sucrose. In present time these special products are under clinical trial in scientific medico-genetic center of Russia' Academy of Medical Science.

Microecology and local immune and nonspecific defensive proteins depending on different nutrition.

Breast-feeding is of high importance for the development of intestinal eubiosis. Before beginning with breast-feeding the coprofiltrates of newborns lack of IgA. Following the first feeding IgA concentration in the faeces sharply increases (up to 200 mg/100 g faeces). Comparable high values can be found in the coprofiltrates of breast-fed sick prematures. In the coprofiltrates of artificially fed healthy newborns and sick prematures no IgA is provable, within the first two weeks of age. Afterwards both the frequency of its evidence and its concentration gradually rise. This can be regarded as a sign of an increasing local production of immune proteins. Starting with the second year of life, only, the values of all the immunoglobulins fall again. It happens a microbial degradation. Increased concentrations of immunoglobulins in the coprofiltrates of children over 3 years must be evaluated as a sign of subclinical dysbacteriosis.

Microecology of the gastrointestinal tract and the immunological status under food allergy.

In children suffering from food allergy the microecology of the gut and the immune status were examined. As a rule, sensitization due to the penetration of the epithelial barrier of the intestinum by macromolecules of the food is not accompanied by deficiency of secretory IgA. In all cases dysbacteriosis of different degree was observed with a characteristic picture of deficiency of Bifidobacteria and Lactobacilli combined with rising numbers of Enterobacteriaceae. Correlations to an altered immune response are obvious.