Results of a Prospective Study Concerning the Clinical Efficiency of Recent Russian-Made Specialized Foods without Phenylalanine
Background: Specialized foods, based on aminoacids without phenilalanin, are the main source of protein for patients with phenilketonuria of all ages. Based on modern technologies, new Russian-made foods were created. They have an optimized aminoacid and micronutrient composition, which increases th...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | Russian |
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Union of pediatricians of Russia
2016-08-01
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| Series: | Педиатрическая фармакология |
| Subjects: | |
| Online Access: | https://www.pedpharma.ru/jour/article/view/1424 |
| _version_ | 1826570356457996288 |
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| author | T. V. Bushueva T. E. Borovik K. S. Ladodo L. M. Kuzenkova |
| author_facet | T. V. Bushueva T. E. Borovik K. S. Ladodo L. M. Kuzenkova |
| author_sort | T. V. Bushueva |
| collection | DOAJ |
| description | Background: Specialized foods, based on aminoacids without phenilalanin, are the main source of protein for patients with phenilketonuria of all ages. Based on modern technologies, new Russian-made foods were created. They have an optimized aminoacid and micronutrient composition, which increases their bioavailability.Objective: Our aim was to investigate the clinical efficiency of the new Russian-made specialized foods based on aminoacids without phenylalanine, in patients with phenylketonuria.Methods: Evaluating physical, somatic and neuropsychic development as well as measuring phenylalanine blood level were carried out twice: before the prescription and after 1 month of using the new Russian-made specialized foods. The chemical composition of the ration was controlled using dietology methods.Results: 57 children at the age of 14 days to 15 years with phenylketonuria (which was detected in neonatal screening) were picked for this study. A hypophenylalanine diet has been prescribed for all children no later than at the age of 3 months of life. In children of the first year (I group) (among which there were children with high phenylalanine blood levels) phenylalanine concentration decreased from 5,5 (4,0; 21,0) to 4,4 (3,7; 4,7) mg/dl (p = 0,014) while using Russian-made specialized foods. In this group of children the psychomotor and physical indices improved. In early childhood-, preschool- and school-aged patients phenylalanine blood level remained steady. The chemical composition of the ration with Russian-made foods, based on aminoacids without phenylalanine, corresponded to the reference intake of main nutrients and energy.Conclusion: Hypophenylalanine diet with new Russian-made specialized foods without phenylalanine (which is designed for phenylketonuria patients of various age groups) showed high clinical efficiency of these foods. |
| first_indexed | 2025-03-14T11:53:56Z |
| format | Article |
| id | doaj.art-76bac02d888b4e83975a08c89e6a2bc1 |
| institution | Directory Open Access Journal |
| issn | 1727-5776 2500-3089 |
| language | Russian |
| last_indexed | 2025-03-14T11:53:56Z |
| publishDate | 2016-08-01 |
| publisher | Union of pediatricians of Russia |
| record_format | Article |
| series | Педиатрическая фармакология |
| spelling | doaj.art-76bac02d888b4e83975a08c89e6a2bc12025-03-02T09:47:18ZrusUnion of pediatricians of RussiaПедиатрическая фармакология1727-57762500-30892016-08-0113325125810.15690/pf.v13i3.15751409Results of a Prospective Study Concerning the Clinical Efficiency of Recent Russian-Made Specialized Foods without PhenylalanineT. V. Bushueva0T. E. Borovik1K. S. Ladodo2L. M. Kuzenkova3Scientific Center of Children’s Health, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian Federation Sechenov First Moscow State Medical University, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian Federation Sechenov First Moscow State Medical University, Moscow, Russian FederationBackground: Specialized foods, based on aminoacids without phenilalanin, are the main source of protein for patients with phenilketonuria of all ages. Based on modern technologies, new Russian-made foods were created. They have an optimized aminoacid and micronutrient composition, which increases their bioavailability.Objective: Our aim was to investigate the clinical efficiency of the new Russian-made specialized foods based on aminoacids without phenylalanine, in patients with phenylketonuria.Methods: Evaluating physical, somatic and neuropsychic development as well as measuring phenylalanine blood level were carried out twice: before the prescription and after 1 month of using the new Russian-made specialized foods. The chemical composition of the ration was controlled using dietology methods.Results: 57 children at the age of 14 days to 15 years with phenylketonuria (which was detected in neonatal screening) were picked for this study. A hypophenylalanine diet has been prescribed for all children no later than at the age of 3 months of life. In children of the first year (I group) (among which there were children with high phenylalanine blood levels) phenylalanine concentration decreased from 5,5 (4,0; 21,0) to 4,4 (3,7; 4,7) mg/dl (p = 0,014) while using Russian-made specialized foods. In this group of children the psychomotor and physical indices improved. In early childhood-, preschool- and school-aged patients phenylalanine blood level remained steady. The chemical composition of the ration with Russian-made foods, based on aminoacids without phenylalanine, corresponded to the reference intake of main nutrients and energy.Conclusion: Hypophenylalanine diet with new Russian-made specialized foods without phenylalanine (which is designed for phenylketonuria patients of various age groups) showed high clinical efficiency of these foods.https://www.pedpharma.ru/jour/article/view/1424childrenphenylketonuriaspecialized foodsphenylalaninediet-therapy |
| spellingShingle | T. V. Bushueva T. E. Borovik K. S. Ladodo L. M. Kuzenkova Results of a Prospective Study Concerning the Clinical Efficiency of Recent Russian-Made Specialized Foods without Phenylalanine Педиатрическая фармакология children phenylketonuria specialized foods phenylalanine diet-therapy |
| title | Results of a Prospective Study Concerning the Clinical Efficiency of Recent Russian-Made Specialized Foods without Phenylalanine |
| title_full | Results of a Prospective Study Concerning the Clinical Efficiency of Recent Russian-Made Specialized Foods without Phenylalanine |
| title_fullStr | Results of a Prospective Study Concerning the Clinical Efficiency of Recent Russian-Made Specialized Foods without Phenylalanine |
| title_full_unstemmed | Results of a Prospective Study Concerning the Clinical Efficiency of Recent Russian-Made Specialized Foods without Phenylalanine |
| title_short | Results of a Prospective Study Concerning the Clinical Efficiency of Recent Russian-Made Specialized Foods without Phenylalanine |
| title_sort | results of a prospective study concerning the clinical efficiency of recent russian made specialized foods without phenylalanine |
| topic | children phenylketonuria specialized foods phenylalanine diet-therapy |
| url | https://www.pedpharma.ru/jour/article/view/1424 |
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