ОЦЕНКА КАЧЕСТВА ЖИЗНИ ДЕТЕЙ, БОЛЬНЫХ ФЕНИЛКЕТОНУРИЕЙ

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 compared to their healthy peers, also depending on the timing of the treatment start 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: It was found the statistically significant (p 0.001) differences between PKU patients and healthy children in the physical, emotional, social and role functioning. 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 relationship (p 0.05) was found between the role functioning and patient’s age and timing of the treatment. 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. Фенилкетонурия — одно из наиболее распространенных в России наследственных нарушений обмена аминокислот, выявляемых путем неонатального скрининга. Диетотерапия, назначаемая при данной патологии, демонстрирует положительный эффект, однако качество жизни больных фенилкетонурией различных возрастных групп и в зависимости от сроков начала лечения остается неизученным. Цель исследования: произвести оценку качества жизни больных фенилкетонурией детей в сравнении с их здоровыми сверстниками. Методы: в исследовании были задействованы 64 пары — ребенок с фенликтонурией и его родитель. Для анкетирования использовали общий опросник Pediatric Quality of Life Inventory (PedsQLтм4.0, Varni и соавт., США, 2001). Статистическую обработку полученных результатов осуществляли при помощи программы SPSS v. 14.0 (США). Результаты: установлены статистически значимые (p 0,001) различия между больными фенилкетонурией и здоровыми детьми по параметрам физического эмоционального, социального и ролевого функционирования; наиболее выраженные отличия отмечены в социальном (значение медианы 70 против 90 у здоровых) и ролевом (57,5 против 80, соответственно) функционировании. Максимально значимая корреляция (p 0,001) установлена между социальным функционированием пациентов с фенилкетонурией и сроком начала диетического лечения, а также получена статистически значимая функциональная связь (p 0,05) ролевого функционирования с возрастом пациентов и сроком начала лечения. Показатели социальной адаптации (социальное и ролевое функционирование) были выше (p 0,05) в подгруппе больных фенилкетонурией детей, своевременно выявленных и рано начавших лечение. Заключение: результаты исследования подтверждают необходимость ранней диагностики фенилкетонурии, своевременного начала диетического лечения, а также организации информационной и консультативной помощи родителям больных детей.

MODERN PRINCIPLES OF DIET ORGANIZATION IN CHILDREN IN DIFFERENT AGE WITH PHENYLKETONURIA

The article reports questions of diet organization in children with phenylketonuria in different age with the use of modern specialized food. Authors give examples of calculation of diet based on new norms of needs in energy and food for different groups of population in Russian Federation, and present recommendations on optimization of nutrition in schoolchildren with phenylketonuria.Key words: children, schoolchildren, phenylketonuria, phenylalanine, dietotherapy.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(2):124-129

INTESTINAL DYSBIOSIS IN CHILDREN WITH FOOD ALLERGY: PATHOGENETIC ASPECTS AND MODERN CORRECTION METHODS

Background paper analyses the role of intestinal micro-flora at the stage of forming immunity, the importance of intestinal microflora abnormalities during the periods of allergic diseases development (primarily food allergies), as well as mechanisms of dysbiosis effect on the allergic processes in child's body. The study discusses the mechanisms of treatment and prevention effect of probiotics for cases of child allergic diseases. The work also specifies modern approaches to correcting dysbiotic abnormalities for children with food allergies, reviews the options of diet and medication treatment of food allergy, suggests a new algorithm of gradual treatment that targets correction of dysbiosis in this patient category.Key words: children, food allergy, dysbiosis, probiotics, prebiotics, diet therapy.</strong

DIET CORRECTION OF GASTROINTESTINAL TRACT DYSFUNCTIONS OF INFANTS AFTER CEREBRAL ISCHEMIA

The article analyzes mechanisms of occurrence of gastrointestinal tract dysfunctions of infants after cerebral ischemia. It describes the clinical symptomatology of gastrointestinal impairment, particularly associated with vegetovisceral impairment, lactase deficiency, cow's milk protein intolerance. It gives recommendations about diet correction of gastrointestinal tract dysfunctions of infants.Key words: infants, gastrointestinal tract dysfunctions, dietotherapy.</strong

Российская газета. Экономика Уральского округа. 2015. № 255

<p><em>The article provides information on healthy eating and discusses the issues in reducing the consumption by the population of essential and functional food components, insufficient adaption of humans to aggressive environmental factors and the need to enrich the diet with micronutrients, omega-3 and omega-6 polyunsaturated fatty acids, and prebiotics that have a control impact on the body. The article contains information on the biological role of polyunsaturated fatty acids and prebiotics, their ability to enrich children’s diets. </em><br /><strong><em>Key words: healthy eating, child, micronutrients, omega-3 and omega-6 polyunsaturated fatty acids, prebiotics, diet enrichment. </em></strong></p><p><strong><em></em></strong><em>(Pediatric Pharmacology. – 2010; 7(6):86-92)</em></p

EFFICIENCY OF THE AMINOACID-BASED MIXTURES IN BRINGING RELIEF FOR THE SYMPTOMS OF THE COW'S MILK PROTEIN ALLERGY: SYSTEMATIC REVIEW

In the given systematic review, the authors evaluated the efficiency of the amino acid based mixtures among the patients, suffering from the cow's milk protein allergy. They have studied the electronic and bibliographic databases. The researchers accounted for the patients of any age, suffering from the cow's milk protein allergy or similar symptoms. The task was to compare the aminoacid based mixtures with highly hydrolyzed mixtures, soy mixtures and cow's milk/cow's milk based mixtures. They showed that the use of the amino acid based mixtures is safe and efficient among the infants with the confirmed or suspected cow's milk protein allergy. The data of the randomized controlled studies, comparing amino acid based mixtures with highly hydrolyzed mixtures, witnesses that both types of mixtures are equally efficient in reducing the symptoms of the cow's milk protein allergy in the confirmed or alleged allergy cases. This review demonstrates the clinical impact of the amino acid based mixtures both upon certain symptoms and growth of the infants and children with the cow's milk protein allergy, who have zero tolerance towards the highly hydrolyzed mixtures.Key words: aminoacid-based mixtures, cow's milk protein allergy, highly hydrolyzed mixtures, soy mixtures, systematic review

COMBINED SKIN AND RESPIRATORY SYMPTOMS OF ALIMENTARY ALLERGY IN CHILDREN: ETIOLOGY, CLINICS, COMPLEX TREATMENT

The article describes peculiarities of skin and respiratory symptoms of alimentary allergy, main etiological factors and pathogenetical stages of pathology’s formation. Authors present a description of alimentary allergy in different age periods, methods of its diagnostics, treatment of skin and respiratory signs of alimentary allergy.Key words: children, alimentary allergy, atopic dermatitis, respiratory allergy.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(2):74-81

USE OF FOODSTUFFS WITH PRO-AND PREBIOTIC ACTIONS IN INFANT FOOD

The article deals with the data about opportunity to enrich the modern infant foodstuffs with pro and prebiotics. The authors name the contents of the foodstuffs with pro and prebiotic properties, findings of the multicentric research according to the estimate of their clinical efficiency among healthy and ill infants. They show the safety and high efficiency of the use of the given healthful and dietary foodstuffs.Key words: infant food, foodstuffs, probiotics, prebiotics, bifidus bacteria, lactic acid bacilli, oligosaccharides, lactulose, inulin

BALANCED DIET IN CHILDHOOD — THE FOUNDATION OF A HEALTHY LIFE

The article illustrates current views on the balanced diet concept and the healthy diet notion. It discusses the issues related to the shortage of nutrients in children and adults, its causes, symptoms, possibility of preventive measures and correction using specialized enriched foodstuffs, vitamin and mineral supplements, dietary supplements, provides information about key food sources of vitamins and minerals, new age norms for children’s consumption of micronutrients. Key words: balanced and healthy diet, vitamins and minerals, norms for consumption of micronutrients, vitamin and mineral supplements, dietary supplements, enriched foodstuffs, children. (Pediatric Pharmacology. – 2010; 7(3):82-87

BALANCED DIET IN CHILDHOOD — THE FOUNDATION OF A HEALTHY LIFE

The article illustrates current views on the balanced diet concept and the healthy diet notion. It discusses the issues related to the shortage of nutrients in children and adults, its causes, symptoms, possibility of preventive measures and correction using specialized enriched foodstuffs, vitamin and mineral supplements, dietary supplements, provides information about key food sources of vitamins and minerals, new age norms for children’s consumption of micronutrients. Key words: balanced and healthy diet, vitamins and minerals, norms for consumption of micronutrients, vitamin and mineral supplements, dietary supplements, enriched foodstuffs, children. (Pediatric Pharmacology. – 2010; 7(3):82-87