[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.

[Clinical evaluation of the usage of iodine-containing food products in the diet of children]. / Klinicheskaia otsenka ispol'zovaniia iodosoderzhashchikh produktov v pitanii detei.

On the basis of clinical supervision came to a conclusion, that the canned food meat of chickens with sea cabbage can be recommended for use in a dietotherapy of children 1.5-3.5 years with deficiency iodine by a condition.

[Role of polyunsaturated fatty acids in diet therapy of children with allergic diseases]. / Rol' polinenasyshchennykh zhirnykh kislot v lechebnom pitanii detei s allergicheskimi zabolevaniiami.

135 pediatric patients receiving hypoallergic diet were included into the study group. The control group consisted of 20 children. The impact of PUFA omega-3 biologically active supplements (polyen, prima-Oil) was studied in hypoallergic rations. Biochemical indices were simultaneously investigated. The revealed dynamic changes of fatty acid spectrum in plasma and red cell membranes, cellular and humoral immunity status and eicosanoids synthesis were followed by positive clinical changes. Diets enriched with biologically active supplementation (PUPA omega-3) can be recommended for application in pediatric practice.

[Effectiveness of beta-carotene for treatment of erosive ulcerative changes in gastric and duodenal mucosa in children with chronic glomerulonephritis]. / Otsenka éffektivnosti primeneniia beta-karotina pri érozivno-iazvennykh izmeneniiakh slizistoi obolochki zheludka i dvenadtsatiperstnoi kishki u detei s khronicheskim glomerulonefritom.

The peptic ulcer of stomach and duodenum are the more often occuring complications of steroid treatment in children with chronic glomerulonephrite. Dinamic investigation has shown that the using of beta-carotin oily solution in therapy of this complications increases the efficiency of traditional treatment. It has been shown that beta-carotene administration speeds the reparation of stomach and duodenum ulcers, saves adequate serum concentration of beta-carotene and retinol. The data obtained demonstrate that beta-carotene administration in the complex therapy of chronic glomerulonephrite patient with gastroenteropathy should be recommended.

[Selenium contents in human milk after term and preterm labor and in breast milk substitutes]. / Soderzhanie selena v zhenskom moloke posle srochnykh i prezhdevremennykh rodov, a takzhe v adaptirovannykh molochnykh produktakh.

Selenium contents in breast milk of Russia's and Vietnam's women after term and preterm childbirth and in breast milk substitutes were studied. The concentration of selenium in breast milk of Russia's women was normal after term and preterm childbirth. The level of selenium in breast milk of Vietnam's women was decreased and could not satisfy completely the need of breasted infants. Selenium contents in babyfood "Malutka" Istra' manufacturing company were lower than those in Sybaj' company.

[Content of toxic substances in breast milk after timely and premature labor]. / Soderzhanie toksichnykh veshchestv v zhenskom moloke posle srochnykh i prezhdevremennykh rodov.

Data on the contents of protein, fats and toxic compounds (PCB, DDT, gamma-Lindane) in breast milk Russian and Vietnamese women after timely and premature childbed are resulted. The contents of protein in transitive milk was higher than in mature milk, both timely childbed and premature one. The contents of protein in nature milk of the Vietnamese women was lower, than in mature milk of the Russian women (12 g/l against 16.5 g/l accordingly). The contents of fat in breast milk of the Russian women in lactation increased. Vietnamese women had very low fat level in mature milk (29 g/l against 42 g/l at the Russian women). The contents of toxic compounds (PCB, DDT, gamma-Lindane) in breast milk both, Vietnamese women and Russian women was rather high and significant frequency of detection of these compounds in milk was marked. The correlation contents of basic food components in breast milk and level of toxic compounds was absent.

[Use of the fish oil "Polyen" in pediatric practice]. / Opyt primeneniia ryb'ego zhira "polien" v pediatricheskoi praktike.

The effects of supplementation of basic diets with 'Polyen' in daily doses of 2.5-4.0 g during 30-40 days together with antioxidants were studied in patients with kidney diseases (diet No7, n = 14), different allergies (hypoallergenic diet, n = 37) and in control children (n = 12). It was shown positive dynamics in fatty acid contents in plasma and red blood cell membranes, in humoral and cell immunity indicators and in improving of clinical symptoms of diseases. 'Polyen' is recommended for wide use in pediatric practice.

[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.

[The characteristics of amino acid metabolism in premature infants on different types of feeding]. / Osobennosti aminokislotnogo obmena u nedonoshennykh detei pri razlichnykh vidakh vskarmlivaniia.

Analysis of the free amino acids (AA) in a blood plasma of premature infants receiving kinds of baby food has allowed to reveal a feature of AA metabolism. A significant increasing of sum of the free AA and stable contents of aromatic AA have been found in blood of premature infants fed Prepiltti [correction of 'Prepilti'] that testified to adequacy of protein in Prepiltti [correction of 'Prepiltu'] to requirements of premature infants. Also it has been established different changes of concentration methionine and cystine in this group of the babies that confirms a hypothesis about immature of liver enzymes catabolizing a methionine.

[Calcium homeostasis and calcium-regulating hormones in young children with phenylketonuria]. / Sostoianie kaltsievogo gomeostaza i kaltsiireguliruiushchikh gormonov u detei rannego vozrasta, bolnykh fenilketonuriei.

Calcium and calcium-regulating hormones were investigated in 19 infants with phenylketonuria. Parathyroid hormone, osteocalcin, dehydrocholecalciferol were found elevated while calcitonin reduced in the serum of the infants prior to the diet. Total calcium and phosphorus remained stable in the course of the study. Element diet resulted in reduced serum parathormone levels though surpassing those of healthy subjects. This fact is attributed to primary metabolic disorders responsible for the onset of hyperparathyroidism. The conclusion is made that element diet is unable to change calcium homeostasis and that of calcium-regulating hormones in infants with phenylketonuria.