55 research outputs found
ΠΠ¦ΠΠΠΠ ΠΠΠ§ΠΠ‘Π’ΠΠ ΠΠΠΠΠ ΠΠΠ’ΠΠ, ΠΠΠΠ¬ΠΠ«Π₯ Π€ΠΠΠΠΠΠΠ’ΠΠΠ£Π ΠΠΠ
Background: Phenylketonuria (PKU) β 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 β 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 (pΒ 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 (pΒ 0.001) was installed between the social functioning of PKU patients and timing of the dietary treatment. Also the statistically significant relationship (pΒ 0.05) was found between the role functioning and patientβs age and timing of the treatment. The assessment points of social and role functioning were higher (pΒ 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.Β Π€Π΅Π½ΠΈΠ»ΠΊΠ΅ΡΠΎΠ½ΡΡΠΈΡ β ΠΎΠ΄Π½ΠΎ ΠΈΠ· Π½Π°ΠΈΠ±ΠΎΠ»Π΅Π΅ ΡΠ°ΡΠΏΡΠΎΡΡΡΠ°Π½Π΅Π½Π½ΡΡ
Π² Π ΠΎΡΡΠΈΠΈ Π½Π°ΡΠ»Π΅Π΄ΡΡΠ²Π΅Π½Π½ΡΡ
Π½Π°ΡΡΡΠ΅Π½ΠΈΠΉ ΠΎΠ±ΠΌΠ΅Π½Π° Π°ΠΌΠΈΠ½ΠΎΠΊΠΈΡΠ»ΠΎΡ, Π²ΡΡΠ²Π»ΡΠ΅ΠΌΡΡ
ΠΏΡΡΠ΅ΠΌ Π½Π΅ΠΎΠ½Π°ΡΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΡΠΊΡΠΈΠ½ΠΈΠ½Π³Π°. ΠΠΈΠ΅ΡΠΎΡΠ΅ΡΠ°ΠΏΠΈΡ, Π½Π°Π·Π½Π°ΡΠ°Π΅ΠΌΠ°Ρ ΠΏΡΠΈ Π΄Π°Π½Π½ΠΎΠΉ ΠΏΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΠΈ, Π΄Π΅ΠΌΠΎΠ½ΡΡΡΠΈΡΡΠ΅Ρ ΠΏΠΎΠ»ΠΎΠΆΠΈΡΠ΅Π»ΡΠ½ΡΠΉ ΡΡΡΠ΅ΠΊΡ, ΠΎΠ΄Π½Π°ΠΊΠΎ ΠΊΠ°ΡΠ΅ΡΡΠ²ΠΎ ΠΆΠΈΠ·Π½ΠΈ Π±ΠΎΠ»ΡΠ½ΡΡ
ΡΠ΅Π½ΠΈΠ»ΠΊΠ΅ΡΠΎΠ½ΡΡΠΈΠ΅ΠΉ ΡΠ°Π·Π»ΠΈΡΠ½ΡΡ
Π²ΠΎΠ·ΡΠ°ΡΡΠ½ΡΡ
Π³ΡΡΠΏΠΏ ΠΈ Π² Π·Π°Π²ΠΈΡΠΈΠΌΠΎΡΡΠΈ ΠΎΡ ΡΡΠΎΠΊΠΎΠ² Π½Π°ΡΠ°Π»Π° Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΎΡΡΠ°Π΅ΡΡΡ Π½Π΅ΠΈΠ·ΡΡΠ΅Π½Π½ΡΠΌ. Π¦Π΅Π»Ρ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ: ΠΏΡΠΎΠΈΠ·Π²Π΅ΡΡΠΈ ΠΎΡΠ΅Π½ΠΊΡ ΠΊΠ°ΡΠ΅ΡΡΠ²Π° ΠΆΠΈΠ·Π½ΠΈ Π±ΠΎΠ»ΡΠ½ΡΡ
ΡΠ΅Π½ΠΈΠ»ΠΊΠ΅ΡΠΎΠ½ΡΡΠΈΠ΅ΠΉ Π΄Π΅ΡΠ΅ΠΉ Π² ΡΡΠ°Π²Π½Π΅Π½ΠΈΠΈ Ρ ΠΈΡ
Π·Π΄ΠΎΡΠΎΠ²ΡΠΌΠΈ ΡΠ²Π΅ΡΡΡΠ½ΠΈΠΊΠ°ΠΌΠΈ. ΠΠ΅ΡΠΎΠ΄Ρ: Π² ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΈ Π±ΡΠ»ΠΈ Π·Π°Π΄Π΅ΠΉΡΡΠ²ΠΎΠ²Π°Π½Ρ 64 ΠΏΠ°ΡΡ β ΡΠ΅Π±Π΅Π½ΠΎΠΊ Ρ ΡΠ΅Π½Π»ΠΈΠΊΡΠΎΠ½ΡΡΠΈΠ΅ΠΉ ΠΈ Π΅Π³ΠΎ ΡΠΎΠ΄ΠΈΡΠ΅Π»Ρ. ΠΠ»Ρ Π°Π½ΠΊΠ΅ΡΠΈΡΠΎΠ²Π°Π½ΠΈΡ ΠΈΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π»ΠΈ ΠΎΠ±ΡΠΈΠΉ ΠΎΠΏΡΠΎΡΠ½ΠΈΠΊ Pediatric Quality of Life Inventory (PedsQLΡΠΌ4.0, Varni ΠΈ ΡΠΎΠ°Π²Ρ., Π‘Π¨Π, 2001). Π‘ΡΠ°ΡΠΈΡΡΠΈΡΠ΅ΡΠΊΡΡ ΠΎΠ±ΡΠ°Π±ΠΎΡΠΊΡ ΠΏΠΎΠ»ΡΡΠ΅Π½Π½ΡΡ
ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΠΎΠ² ΠΎΡΡΡΠ΅ΡΡΠ²Π»ΡΠ»ΠΈ ΠΏΡΠΈ ΠΏΠΎΠΌΠΎΡΠΈ ΠΏΡΠΎΠ³ΡΠ°ΠΌΠΌΡ SPSS v. 14.0 (Π‘Π¨Π). Π Π΅Π·ΡΠ»ΡΡΠ°ΡΡ: ΡΡΡΠ°Π½ΠΎΠ²Π»Π΅Π½Ρ ΡΡΠ°ΡΠΈΡΡΠΈΡΠ΅ΡΠΊΠΈ Π·Π½Π°ΡΠΈΠΌΡΠ΅ (pΒ 0,001) ΡΠ°Π·Π»ΠΈΡΠΈΡ ΠΌΠ΅ΠΆΠ΄Ρ Π±ΠΎΠ»ΡΠ½ΡΠΌΠΈ ΡΠ΅Π½ΠΈΠ»ΠΊΠ΅ΡΠΎΠ½ΡΡΠΈΠ΅ΠΉ ΠΈ Π·Π΄ΠΎΡΠΎΠ²ΡΠΌΠΈ Π΄Π΅ΡΡΠΌΠΈ ΠΏΠΎ ΠΏΠ°ΡΠ°ΠΌΠ΅ΡΡΠ°ΠΌ ΡΠΈΠ·ΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΡΠΌΠΎΡΠΈΠΎΠ½Π°Π»ΡΠ½ΠΎΠ³ΠΎ, ΡΠΎΡΠΈΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΈ ΡΠΎΠ»Π΅Π²ΠΎΠ³ΠΎ ΡΡΠ½ΠΊΡΠΈΠΎΠ½ΠΈΡΠΎΠ²Π°Π½ΠΈΡ; Π½Π°ΠΈΠ±ΠΎΠ»Π΅Π΅ Π²ΡΡΠ°ΠΆΠ΅Π½Π½ΡΠ΅ ΠΎΡΠ»ΠΈΡΠΈΡ ΠΎΡΠΌΠ΅ΡΠ΅Π½Ρ Π² ΡΠΎΡΠΈΠ°Π»ΡΠ½ΠΎΠΌ (Π·Π½Π°ΡΠ΅Π½ΠΈΠ΅ ΠΌΠ΅Π΄ΠΈΠ°Π½Ρ 70 ΠΏΡΠΎΡΠΈΠ² 90 Ρ Π·Π΄ΠΎΡΠΎΠ²ΡΡ
) ΠΈ ΡΠΎΠ»Π΅Π²ΠΎΠΌ (57,5 ΠΏΡΠΎΡΠΈΠ² 80, ΡΠΎΠΎΡΠ²Π΅ΡΡΡΠ²Π΅Π½Π½ΠΎ) ΡΡΠ½ΠΊΡΠΈΠΎΠ½ΠΈΡΠΎΠ²Π°Π½ΠΈΠΈ. ΠΠ°ΠΊΡΠΈΠΌΠ°Π»ΡΠ½ΠΎ Π·Π½Π°ΡΠΈΠΌΠ°Ρ ΠΊΠΎΡΡΠ΅Π»ΡΡΠΈΡ (pΒ 0,001) ΡΡΡΠ°Π½ΠΎΠ²Π»Π΅Π½Π° ΠΌΠ΅ΠΆΠ΄Ρ ΡΠΎΡΠΈΠ°Π»ΡΠ½ΡΠΌ ΡΡΠ½ΠΊΡΠΈΠΎΠ½ΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅ΠΌ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΡΠ΅Π½ΠΈΠ»ΠΊΠ΅ΡΠΎΠ½ΡΡΠΈΠ΅ΠΉ ΠΈ ΡΡΠΎΠΊΠΎΠΌ Π½Π°ΡΠ°Π»Π° Π΄ΠΈΠ΅ΡΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ, Π° ΡΠ°ΠΊΠΆΠ΅ ΠΏΠΎΠ»ΡΡΠ΅Π½Π° ΡΡΠ°ΡΠΈΡΡΠΈΡΠ΅ΡΠΊΠΈ Π·Π½Π°ΡΠΈΠΌΠ°Ρ ΡΡΠ½ΠΊΡΠΈΠΎΠ½Π°Π»ΡΠ½Π°Ρ ΡΠ²ΡΠ·Ρ (pΒ 0,05) ΡΠΎΠ»Π΅Π²ΠΎΠ³ΠΎ ΡΡΠ½ΠΊΡΠΈΠΎΠ½ΠΈΡΠΎΠ²Π°Π½ΠΈΡ Ρ Π²ΠΎΠ·ΡΠ°ΡΡΠΎΠΌ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΠΈ ΡΡΠΎΠΊΠΎΠΌ Π½Π°ΡΠ°Π»Π° Π»Π΅ΡΠ΅Π½ΠΈΡ. ΠΠΎΠΊΠ°Π·Π°ΡΠ΅Π»ΠΈ ΡΠΎΡΠΈΠ°Π»ΡΠ½ΠΎΠΉ Π°Π΄Π°ΠΏΡΠ°ΡΠΈΠΈ (ΡΠΎΡΠΈΠ°Π»ΡΠ½ΠΎΠ΅ ΠΈ ΡΠΎΠ»Π΅Π²ΠΎΠ΅ ΡΡΠ½ΠΊΡΠΈΠΎΠ½ΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅) Π±ΡΠ»ΠΈ Π²ΡΡΠ΅ (pΒ 0,05) Π² ΠΏΠΎΠ΄Π³ΡΡΠΏΠΏΠ΅ Π±ΠΎΠ»ΡΠ½ΡΡ
ΡΠ΅Π½ΠΈΠ»ΠΊΠ΅ΡΠΎΠ½ΡΡΠΈΠ΅ΠΉ Π΄Π΅ΡΠ΅ΠΉ, ΡΠ²ΠΎΠ΅Π²ΡΠ΅ΠΌΠ΅Π½Π½ΠΎ Π²ΡΡΠ²Π»Π΅Π½Π½ΡΡ
ΠΈ ΡΠ°Π½ΠΎ Π½Π°ΡΠ°Π²ΡΠΈΡ
Π»Π΅ΡΠ΅Π½ΠΈΠ΅. ΠΠ°ΠΊΠ»ΡΡΠ΅Π½ΠΈΠ΅: ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΡ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ ΠΏΠΎΠ΄ΡΠ²Π΅ΡΠΆΠ΄Π°ΡΡ Π½Π΅ΠΎΠ±Ρ
ΠΎΠ΄ΠΈΠΌΠΎΡΡΡ ΡΠ°Π½Π½Π΅ΠΉ Π΄ΠΈΠ°Π³Π½ΠΎΡΡΠΈΠΊΠΈ ΡΠ΅Π½ΠΈΠ»ΠΊΠ΅ΡΠΎΠ½ΡΡΠΈΠΈ, ΡΠ²ΠΎΠ΅Π²ΡΠ΅ΠΌΠ΅Π½Π½ΠΎΠ³ΠΎ Π½Π°ΡΠ°Π»Π° Π΄ΠΈΠ΅ΡΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ, Π° ΡΠ°ΠΊΠΆΠ΅ ΠΎΡΠ³Π°Π½ΠΈΠ·Π°ΡΠΈΠΈ ΠΈΠ½ΡΠΎΡΠΌΠ°ΡΠΈΠΎΠ½Π½ΠΎΠΉ ΠΈ ΠΊΠΎΠ½ΡΡΠ»ΡΡΠ°ΡΠΈΠ²Π½ΠΎΠΉ ΠΏΠΎΠΌΠΎΡΠΈ ΡΠΎΠ΄ΠΈΡΠ΅Π»ΡΠΌ Π±ΠΎΠ»ΡΠ½ΡΡ
Π΄Π΅ΡΠ΅ΠΉ.
Π‘ΠΈΠ½Π΄ΡΠΎΠΌ Π‘Π°Π½ΡΠΈΠ»ΠΈΠΏΠΏΠΎ
Sanfilippo syndrome (mucopolysaccharidosis type III) is a lysosomal disorder caused by a defect in the catabolism of heparan sulfate. Mucopolysaccharidosis type III is the most common type of all mucopolysaccharidoses. The pathogenic basis of the disease consists of the storage of undegraded substrate in the central nervous system. Progressive cognitive decline resulting in dementia and behavioural abnormalities are the main clinical characteristics of Sanfilippo syndrome. Mucopolysaccharidosis type III may be misdiagnosed as other forms of developmental delay, attention deficit/hyperactivity disorder and autistic spectrum disorders because of lack of somatic symptoms, presence of mild and atypical forms of the disease. Patients with Sanfilippo syndrome may have comparatively low urinary glycosaminoglycans levels resulting in false negative urinary assay. Definitive diagnosis is made by enzyme assay on leucocytes and cultured fibroblasts. There is currently no effective treatment of mucopolysaccharidosis type III, though ongoing researches of gene, substrate reduction and intrathecal enzyme replacement therapies expect getting curative method to alter devasting damage of central nervous system in near future.Π‘ΠΈΠ½Π΄ΡΠΎΠΌ Π‘Π°Π½ΡΠΈΠ»ΠΈΠΏΠΏΠΎ (ΠΌΡΠΊΠΎΠΏΠΎΠ»ΠΈΡΠ°Ρ
Π°ΡΠΈΠ΄ΠΎΠ· III ΡΠΈΠΏΠ°) β Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠ΅, ΠΎΡΠ½ΠΎΡΡΡΠ΅Π΅ΡΡ ΠΊ Π³ΡΡΠΏΠΏΠ΅ Π»ΠΈΠ·ΠΎΡΠΎΠΌΠ½ΡΡ
Π±ΠΎΠ»Π΅Π·Π½Π΅ΠΉ Π½Π°ΠΊΠΎΠΏΠ»Π΅Π½ΠΈΡ, ΠΎΠ±ΡΡΠ»ΠΎΠ²Π»Π΅Π½Π½ΠΎΠ΅ Π½Π°ΡΡΡΠ΅Π½ΠΈΠ΅ΠΌ ΠΊΠ°ΡΠ°Π±ΠΎΠ»ΠΈΠ·ΠΌΠ° Π³Π΅ΠΏΠ°ΡΠ°Π½ΡΡΠ»ΡΡΠ°ΡΠ°. ΠΡΠΊΠΎΠΏΠΎΠ»ΠΈΡΠ°Ρ
Π°ΡΠΈΠ΄ΠΎΠ· III ΡΠΈΠΏΠ° β Π½Π°ΠΈΠ±ΠΎΠ»Π΅Π΅ ΡΠ°ΡΠΏΡΠΎΡΡΡΠ°Π½Π΅Π½Π½ΡΠΉ ΡΡΠ΅Π΄ΠΈ Π²ΡΠ΅Ρ
ΠΌΡΠΊΠΎΠΏΠΎΠ»ΠΈΡΠ°Ρ
Π°ΡΠΈΠ΄ΠΎΠ·ΠΎΠ². ΠΠ°ΡΠΎΠ³Π΅Π½Π΅ΡΠΈΡΠ΅ΡΠΊΠΎΠΉ ΠΎΡΠ½ΠΎΠ²ΠΎΠΉ ΡΠΈΠ½Π΄ΡΠΎΠΌΠ° ΡΠ²Π»ΡΠ΅ΡΡΡ Π½Π°ΠΊΠΎΠΏΠ»Π΅Π½ΠΈΠ΅ ΠΏΡΠΎΠ΄ΡΠΊΡΠΎΠ² Π°Π½ΠΎΠΌΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΌΠ΅ΡΠ°Π±ΠΎΠ»ΠΈΠ·ΠΌΠ° Π² ΡΠ΅Π½ΡΡΠ°Π»ΡΠ½ΠΎΠΉ Π½Π΅ΡΠ²Π½ΠΎΠΉ ΡΠΈΡΡΠ΅ΠΌΠ΅. ΠΠ΅Π΄ΡΡΠΈΠ΅ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΠΏΡΠΈΠ·Π½Π°ΠΊΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ Π·Π°ΠΊΠ»ΡΡΠ°ΡΡΡΡ Π² Π½Π°ΡΡΡΠ΅Π½ΠΈΠΈ ΠΏΠΎΠ²Π΅Π΄Π΅Π½ΠΈΡ ΠΈ ΠΏΡΠΎΠ³ΡΠ΅ΡΡΠΈΡΡΡΡΠ΅ΠΌ ΡΠ΅Π³ΡΠ΅ΡΡΠ΅ ΠΏΡΠΈΡ
ΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΡΠ°Π·Π²ΠΈΡΠΈΡ Ρ ΠΈΡΡ
ΠΎΠ΄ΠΎΠΌ Π² Π΄Π΅ΠΌΠ΅Π½ΡΠΈΡ. ΠΡΡΡΡΡΡΠ²ΠΈΠ΅ Π²ΡΡΠ°ΠΆΠ΅Π½Π½ΡΡ
ΡΠΎΠΌΠ°ΡΠΈΡΠ΅ΡΠΊΠΈΡ
ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΠΉ, Π½Π°Π»ΠΈΡΠΈΠ΅ Π»Π΅Π³ΠΊΠΈΡ
ΠΈ Π°ΡΠΈΠΏΠΈΡΠ½ΡΡ
ΡΠΎΡΠΌ ΡΠ΅ΡΠ΅Π½ΠΈΡ ΠΏΡΠΈΠ²ΠΎΠ΄ΡΡ ΠΊ ΡΡΡΠ΄Π½ΠΎΡΡΡΠΌ Π΄ΠΈΡΡΠ΅ΡΠ΅Π½ΡΠΈΠ°Π»ΡΠ½ΠΎΠΉ Π΄ΠΈΠ°Π³Π½ΠΎΡΡΠΈΠΊΠΈ Ρ Π΄ΡΡΠ³ΠΈΠΌΠΈ ΡΠΎΡΠΌΠ°ΠΌΠΈ Π·Π°Π΄Π΅ΡΠΆΠΊΠΈ ΡΠ΅ΡΠ΅Π²ΠΎΠ³ΠΎ ΠΈ ΠΏΡΠΈΡ
ΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΡΠ°Π·Π²ΠΈΡΠΈΡ, ΡΠΈΠ½Π΄ΡΠΎΠΌΠΎΠΌ Π΄Π΅ΡΠΈΡΠΈΡΠ° Π²Π½ΠΈΠΌΠ°Π½ΠΈΡ ΠΈ Π³ΠΈΠΏΠ΅ΡΠ°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈ ΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡΠΌΠΈ ΠΈΠ· Π³ΡΡΠΏΠΏΡ Π°ΡΡΠΈΡΡΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΡΠΏΠ΅ΠΊΡΡΠ°. ΠΠ»Ρ Π½Π΅ΠΊΠΎΡΠΎΡΡΡ
ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΡΠΈΠ½Π΄ΡΠΎΠΌ Π‘Π°Π½ΡΠΈΠ»ΠΈΠΏΠΏΠΎ Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠ½Ρ ΠΎΡΠ½ΠΎΡΠΈΡΠ΅Π»ΡΠ½ΠΎ Π½ΠΈΠ·ΠΊΠΈΠ΅ Π·Π½Π°ΡΠ΅Π½ΠΈΡ ΡΠΊΡΠΊΡΠ΅ΡΠΈΡΡΠ΅ΠΌΡΡ
Π³Π»ΠΈΠΊΠΎΠ·Π°ΠΌΠΈΠ½ΠΎΠ³Π»ΠΈΠΊΠ°Π½ΠΎΠ², Π² ΡΠ²ΡΠ·ΠΈ Ρ ΡΠ΅ΠΌ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎ ΠΏΠΎΠ»ΡΡΠ΅Π½ΠΈΠ΅ Π»ΠΎΠΆΠ½ΠΎΠΎΡΡΠΈΡΠ°ΡΠ΅Π»ΡΠ½ΡΡ
ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΠΎΠ² ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ ΠΌΠΎΡΠΈ Π½Π° Π³ΠΈΠΏΠ΅ΡΡΠΊΡΠΊΡΠ΅ΡΠΈΡ Π΄Π°Π½Π½ΡΡ
ΠΌΠ΅ΡΠ°Π±ΠΎΠ»ΠΈΡΠΎΠ². Π’ΠΎΡΠ½ΡΠΉ Π΄ΠΈΠ°Π³Π½ΠΎΠ· ΡΡΡΠ°Π½Π°Π²Π»ΠΈΠ²Π°ΡΡ ΠΏΡΠΈ Π°Π½Π°Π»ΠΈΠ·Π΅ ΡΠΎΠ΄Π΅ΡΠΆΠ°Π½ΠΈΡ ΡΠ΅ΡΠΌΠ΅Π½ΡΠ° Π² ΠΊΡΠ»ΡΡΡΡΠ΅ ΠΊΠΎΠΆΠ½ΡΡ
ΡΠΈΠ±ΡΠΎΠ±Π»Π°ΡΡΠΎΠ² ΠΈΠ»ΠΈ Π»Π΅ΠΉΠΊΠΎΡΠΈΡΠ°Ρ
ΠΊΡΠΎΠ²ΠΈ. ΠΡΡΠ΅ΠΊΡΠΈΠ²Π½ΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΌΡΠΊΠΎΠΏΠΎΠ»ΠΈΡΠ°Ρ
Π°ΡΠΈΠ΄ΠΎΠ·Π° III ΡΠΈΠΏΠ° Π² Π½Π°ΡΡΠΎΡΡΠ΅Π΅ Π²ΡΠ΅ΠΌΡ Π½Π΅ ΡΡΡΠ΅ΡΡΠ²ΡΠ΅Ρ, ΠΎΠ΄Π½Π°ΠΊΠΎ ΠΏΡΠΎΠ΄ΠΎΠ»ΠΆΠ°ΡΡΠΈΠ΅ΡΡ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ Π³Π΅Π½Π½ΠΎΠΉ, ΡΡΠ±ΡΡΡΠ°ΡΡΠ΅Π΄ΡΡΠΈΡΡΡΡΠ΅ΠΉ ΠΈ ΠΈΠ½ΡΡΠ°ΡΠ΅ΠΊΠ°Π»ΡΠ½ΠΎΠΉ ΡΠ΅ΡΠΌΠ΅Π½ΡΠΎΠ·Π°ΠΌΠ΅ΡΡΠΈΡΠ΅Π»ΡΠ½ΠΎΠΉ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ ΠΏΠΎΠ·Π²ΠΎΠ»ΡΡΡ Π½Π°Π΄Π΅ΡΡΡΡΡ Π½Π° Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎΡΡΡ ΠΏΠΎΠ»ΡΡΠ΅Π½ΠΈΡ Π² Π±Π»ΠΈΠΆΠ°ΠΉΡΠ΅ΠΌ Π±ΡΠ΄ΡΡΠ΅ΠΌ ΠΌΠ΅ΡΠΎΠ΄Π°, ΡΠΏΠΎΡΠΎΠ±Π½ΠΎΠ³ΠΎ ΠΏΡΠ΅Π΄ΠΎΡΠ²ΡΠ°ΡΠΈΡΡ ΠΏΡΠΎΠ³ΡΠ΅ΡΡΠΈΡΡΡΡΠ΅Π΅ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΡ ΡΠ΅Π½ΡΡΠ°Π»ΡΠ½ΠΎΠΉ Π½Π΅ΡΠ²Π½ΠΎΠΉ ΡΠΈΡΡΠ΅ΠΌΡ
ΠΠΠ«Π’ ΠΠΠΠΠΠ£Π ΠΠΠΠΠΠ«Π₯ ΠΠΠΠ’ΠΠ ΠΠ«Π₯ ΠΠΠͺΠΠΠ¦ΠΠ ΠΠΠ’Π£ΠΠΠΠΠ§ΠΠ‘ΠΠΠΠ Π’ΠΠΠ‘ΠΠΠ Π’ΠΠΠ Π (ABOBOTULINUM TOXIN A) ΠΠ Π Π‘ΠΠΠ‘Π’ΠΠ§ΠΠ‘ΠΠΠ₯ Π€ΠΠ ΠΠΠ₯ ΠΠΠ’Π‘ΠΠΠΠ Π¦ΠΠ ΠΠΠ ΠΠΠ¬ΠΠΠΠ ΠΠΠ ΠΠΠΠ§Π
Objective: Our aim was to analyze the dosages of Abobotulinum toxin A used for each muscle in the clinically effective and safe repeated multilevel injections in CP children, and the intervals between injections. Methods: Retrospective analysis of 229 injection sessions into 359 muscles of the upper and 361 muscles of the lower extremities in 133 children (2β18 years) with spastic CP. Analysis included only patients who were injected for the first time and demonstrated decrease of spasticity in injected muscles according to modified Ashworth and/or Tardieu scales without significant side effects 2β4 weeks after injections. Motor deficit according to GMFCS was: GMFCS I β 16 (12%) children, GMFCS II β 26 (19.6%), GMFCS III β 43 (32.3%), GMFCS IV β 30 (22.6%), GMFCS VΒ β 18 (13.5%). Repeated injections (up to 5 sessions) were done in 59 children. Maximum follow-up perion was 22 months. 40 patients (30.1%) had one-sided injections, 93 (69.9%) β two-sided, 125 (94%) β multilevel injections. Results: We presented minimal and maximal dosages, interquartile ranges for each injected muscle, also Β«off-labelΒ» and our proposition of per-segment calculation of dosages in multilevel injections in CP. We also demonstrated the stability of intervals between repeated injections and dosages per kg in a patient. These results are compared with the official Russian and international recommendations of BTX-A treatment for children. Conclusion: We presented our experience of BTX-A dosages calculation for the spastic CP treatment which could be used as a recommendation and guide for the multilevel injections treatment according to the aims of rehabilitation, spasticity level, muscle size and motor deficit of a concrete patient.Β Π¦Π΅Π»Ρ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ: ΠΏΡΠΎΠΈΠ·Π²Π΅ΡΡΠΈ Π°Π½Π°Π»ΠΈΠ· Π΄ΠΎΠ· ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ° Abobotulinum toxin A, ΠΏΡΠΈΠΌΠ΅Π½ΡΠ²ΡΠ΅Π³ΠΎΡΡ ΠΏΡΠΈ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈ ΡΡΡΠ΅ΠΊΡΠΈΠ²Π½ΡΡ
ΠΏΠΎΠ²ΡΠΎΡΠ½ΡΡ
ΠΌΠ½ΠΎΠ³ΠΎΡΡΠΎΠ²Π½Π΅Π²ΡΡ
ΠΈΠ½ΡΠ΅ΠΊΡΠΈΡΡ
Ρ Π΄Π΅ΡΠ΅ΠΉ ΡΠΎ ΡΠΏΠ°ΡΡΠΈΡΠ΅ΡΠΊΠΈΠΌΠΈ ΡΠΎΡΠΌΠ°ΠΌΠΈ ΠΠ¦Π, ΠΎΡΠ΅Π½ΠΈΡΡ ΡΡΠ°Π±ΠΈΠ»ΡΠ½ΠΎΡΡΡ ΠΈΡΠΏΠΎΠ»ΡΠ·ΡΠ΅ΠΌΡΡ
Π΄ΠΎΠ· ΠΈ ΠΈΠ½ΡΠ΅ΡΠ²Π°Π»ΠΎΠ² ΠΌΠ΅ΠΆΠ΄Ρ ΠΈΠ½ΡΠ΅ΠΊΡΠΈΡΠΌΠΈ. ΠΠ΅ΡΠΎΠ΄Ρ: Π²ΡΠΏΠΎΠ»Π½Π΅Π½ ΡΠ΅ΡΡΠΎΡΠΏΠ΅ΠΊΡΠΈΠ²Π½ΡΠΉ Π°Π½Π°Π»ΠΈΠ· 229 ΠΈΠ½ΡΠ΅ΠΊΡΠΈΠΎΠ½Π½ΡΡ
ΡΠ΅ΡΡΠΈΠΉ Π² 359 ΠΌΡΡΡ Π²Π΅ΡΡ
Π½ΠΈΡ
ΠΈ 361 ΠΌΡΡΡΡ Π½ΠΈΠΆΠ½ΠΈΡ
ΠΊΠΎΠ½Π΅ΡΠ½ΠΎΡΡΠ΅ΠΉ Ρ 133 Π΄Π΅ΡΠ΅ΠΉ ΡΠΎ ΡΠΏΠ°ΡΡΠΈΡΠ΅ΡΠΊΠΈΠΌΠΈ ΡΠΎΡΠΌΠ°ΠΌΠΈ ΠΠ¦Π Π² Π²ΠΎΠ·ΡΠ°ΡΡΠ΅ ΠΎΡ 2 Π΄ΠΎ 18Β Π»Π΅Ρ, Π²ΠΏΠ΅ΡΠ²ΡΠ΅ ΠΏΡΠΎΡ
ΠΎΠ΄ΠΈΠ²ΡΠΈΡ
Π±ΠΎΡΡΠ»ΠΈΠ½ΠΎΡΠ΅ΡΠ°ΠΏΠΈΡ Ρ ΠΏΠΎΡΠ»Π΅Π΄ΡΡΡΠ΅ΠΉ ΠΊΠΎΠΌΠΏΠ»Π΅ΠΊΡΠ½ΠΎΠΉ ΡΠ΅Π°Π±ΠΈΠ»ΠΈΡΠ°ΡΠΈΠ΅ΠΉ ΠΈ ΠΏΡΠΎΠ΄Π΅ΠΌΠΎΠ½ΡΡΡΠΈΡΠΎΠ²Π°Π²ΡΠΈΡ
ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ ΡΠΎΠ½ΡΡΠ° ΠΈ ΡΠΏΠ°ΡΡΠΈΡΠ½ΠΎΡΡΠΈ ΠΏΠΎ ΠΌΠΎΠ΄ΠΈΡΠΈΡΠΈΡΠΎΠ²Π°Π½Π½ΠΎΠΉ ΡΠΊΠ°Π»Π΅ ΠΡΠ²ΠΎΡΡΠ° ΠΈ/ΠΈΠ»ΠΈ ΠΏΠΎ ΡΠΊΠ°Π»Π΅ Π’Π°ΡΠ΄ΡΠ΅ Π±Π΅Π· ΡΡΡΠ°ΡΡ ΡΡΠ½ΠΊΡΠΈΠΈ ΠΈ ΠΈΠ½ΡΡ
Π½Π΅ΠΆΠ΅Π»Π°ΡΠ΅Π»ΡΠ½ΡΡ
ΡΠ²Π»Π΅Π½ΠΈΠΉ ΡΠ΅ΡΠ΅Π· 2β4Β Π½Π΅Π΄ ΠΏΠΎΡΠ»Π΅ ΠΈΠ½ΡΠ΅ΠΊΡΠΈΠΈ. Π Π°ΡΠΏΡΠ΅Π΄Π΅Π»Π΅Π½ΠΈΠ΅ Π΄Π΅ΡΠ΅ΠΉ ΠΏΠΎ ΡΡΠΎΠ²Π½ΡΠΌ ΠΌΠΎΡΠΎΡΠ½ΠΎΠ³ΠΎ ΡΠ°Π·Π²ΠΈΡΠΈΡ: GMFCS I β 16 (12%), GMFCS II β 26 (19,6%), GMFCS III β 43 (32,3%), GMFCS IV β 30 (22,6%), GMFCS V β 18 (13,5%) ΡΠ΅Π»ΠΎΠ²Π΅ΠΊ. ΠΠΎΠ²ΡΠΎΡΠ½ΡΠ΅ ΠΈΠ½ΡΠ΅ΠΊΡΠΈΠΈ (Π΄ΠΎ 5) ΠΠ’Π ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½Ρ Ρ 59 Π΄Π΅ΡΠ΅ΠΉ, ΠΌΠ°ΠΊΡΠΈΠΌΠ°Π»ΡΠ½ΡΠΉ ΡΡΠΎΠΊ Π½Π°Π±Π»ΡΠ΄Π΅Π½ΠΈΡ β 1Β Π³ΠΎΠ΄ 10Β ΠΌΠ΅Ρ. ΠΠ΄Π½ΠΎΡΡΠΎΡΠΎΠ½Π½ΠΈΠ΅ ΠΈΠ½ΡΠ΅ΠΊΡΠΈΠΈ ΠΏΠΎΠ»ΡΡΠΈΠ»ΠΈ 40 (30,1%) ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΠΎΠ΄Π½ΠΎ- ΠΈ Π΄Π²ΡΡΡΠΎΡΠΎΠ½Π½ΠΈΠΌΠΈ ΡΠΎΡΠΌΠ°ΠΌΠΈ ΠΠ¦Π, Π΄Π²ΡΡΡΠΎΡΠΎΠ½Π½ΠΈΠ΅ ΠΈΠ½ΡΠ΅ΠΊΡΠΈΠΈ β 93 (69,9%) ΠΏΠ°ΡΠΈΠ΅Π½ΡΠ°, ΠΌΠ½ΠΎΠ³ΠΎΡΡΠΎΠ²Π½Π΅Π²ΡΠ΅ ΠΈΠ½ΡΠ΅ΠΊΡΠΈΠΈ β 125 (94%) Π΄Π΅ΡΠ΅ΠΉ. Π Π΅Π·ΡΠ»ΡΡΠ°ΡΡ: ΡΠ°ΡΡΡΠΈΡΠ°Π½Ρ ΠΌΠΈΠ½ΠΈΠΌΠ°Π»ΡΠ½ΡΠ΅ ΠΈ ΠΌΠ°ΠΊΡΠΈΠΌΠ°Π»ΡΠ½ΡΠ΅ ΠΈΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π½Π½ΡΠ΅ Π΄ΠΎΠ·Ρ, ΠΈΠ½ΡΠ΅ΡΠΊΠ²Π°ΡΡΠΈΠ»ΡΠ½ΡΠ΅ Π΄ΠΈΠ°ΠΏΠ°Π·ΠΎΠ½Ρ Π΄ΠΎΠ· ΠΠ’Π Π΄Π»Ρ ΠΊΠ°ΠΆΠ΄ΠΎΠΉ ΠΌΡΡΡΡ, Π² Ρ.Ρ. ΠΏΠΎ Β«off-labelΒ» ΠΏΠΎΠΊΠ°Π·Π°Π½ΠΈΡΠΌ. ΠΠ±ΠΎΡΠ½ΠΎΠ²Π°Π½ ΠΏΠΎΡΠ΅Π³ΠΌΠ΅Π½ΡΠ½ΡΠΉ ΠΏΠΎΠ΄Ρ
ΠΎΠ΄ ΠΏΡΠΈ ΡΠ°ΡΡΠ΅ΡΠ΅ ΠΌΠ½ΠΎΠ³ΠΎΡΡΠΎΠ²Π½Π΅Π²ΡΡ
ΠΈΠ½ΡΠ΅ΠΊΡΠΈΠΉ ΠΠ’Π ΠΏΡΠΈ ΠΠ¦Π. ΠΠΎΠΊΠ°Π·Π°Π½Π° ΡΡΠ°Π±ΠΈΠ»ΡΠ½ΠΎΡΡΡ ΠΈΠ½ΡΠ΅ΡΠ²Π°Π»ΠΎΠ² ΠΌΠ΅ΠΆΠ΄Ρ ΠΏΠΎΠ²ΡΠΎΡΠ½ΡΠΌΠΈ ΠΈΠ½ΡΠ΅ΠΊΡΠΈΡΠΌΠΈ ΠΈ ΠΎΡΠ½ΠΎΡΠΈΡΠ΅Π»ΡΠ½ΠΎΠ΅ ΠΏΠΎΡΡΠΎΡΠ½ΡΡΠ²ΠΎ Π΄ΠΎΠ· Abobotulinum toxin A Π² ΡΠ°ΡΡΠ΅ΡΠ΅ Π½Π° ΠΊΠΈΠ»ΠΎΠ³ΡΠ°ΠΌΠΌ ΠΌΠ°ΡΡΡ ΡΠ΅Π»Π° Π΄Π»Ρ ΠΊΠΎΠ½ΠΊΡΠ΅ΡΠ½ΠΎΠ³ΠΎ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠ°. ΠΠΎΠ»ΡΡΠ΅Π½Π½ΡΠ΅ ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΡ ΡΠΎΠΏΠΎΡΡΠ°Π²Π»Π΅Π½Ρ Ρ ΡΡΡΠ΅ΡΡΠ²ΡΡΡΠΈΠΌΠΈ ΠΎΡΠΈΡΠΈΠ°Π»ΡΠ½ΡΠΌΠΈ ΠΈΠ½ΡΡΡΡΠΊΡΠΈΡΠΌΠΈ, ΠΎΡΠ΅ΡΠ΅ΡΡΠ²Π΅Π½Π½ΡΠΌΠΈ ΠΈ ΠΌΠ΅ΠΆΠ΄ΡΠ½Π°ΡΠΎΠ΄Π½ΡΠΌΠΈ ΡΠ΅ΠΊΠΎΠΌΠ΅Π½Π΄Π°ΡΠΈΡΠΌΠΈ ΠΏΠΎ ΠΏΡΠΈΠΌΠ΅Π½Π΅Π½ΠΈΡ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠ² ΠΠ’Π Ρ Π΄Π΅ΡΠ΅ΠΉ. ΠΡΠ²ΠΎΠ΄Ρ: ΡΠΊΠ°Π·Π°Π½Π½ΡΠ΅ Π΄ΠΎΠ·Ρ Π½ΠΎΡΡΡ ΡΠ΅ΠΊΠΎΠΌΠ΅Π½Π΄Π°ΡΠ΅Π»ΡΠ½ΡΠΉ Ρ
Π°ΡΠ°ΠΊΡΠ΅Ρ ΠΈ ΠΌΠΎΠ³ΡΡ Π±ΡΡΡ ΠΈΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π½Ρ Π² ΠΊΠ°ΡΠ΅ΡΡΠ²Π΅ ΠΎΡΠΈΠ΅Π½ΡΠΈΡΠ° ΠΏΡΠΈ ΠΏΠ»Π°Π½ΠΈΡΠΎΠ²Π°Π½ΠΈΠΈ ΠΌΠ½ΠΎΠ³ΠΎΡΡΠΎΠ²Π½Π΅Π²ΠΎΠΉ Π±ΠΎΡΡΠ»ΠΈΠ½ΠΎΡΠ΅ΡΠ°ΠΏΠΈΠΈ Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΠΠ¦Π Ρ ΡΡΠ΅ΡΠΎΠΌ ΡΠ΅Π»Π΅ΠΉ Π»Π΅ΡΠ΅Π½ΠΈΡ, ΡΡΠΎΠ²Π½Ρ ΡΠΏΠ°ΡΡΠΈΡΠ½ΠΎΡΡΠΈ, ΡΠ°Π·ΠΌΠ΅ΡΠΎΠ² ΠΌΡΡΡ ΠΈ ΡΡΠ΅ΠΏΠ΅Π½ΠΈ ΡΡΠ½ΠΊΡΠΈΠΎΠ½Π°Π»ΡΠ½ΡΡ
Π½Π°ΡΡΡΠ΅Π½ΠΈΠΉ.
LONG TERM NEUROLOGICAL OUTCOMES OF NEWBORNS REANIMATION
The complex evaluation of infants with perinatal lesions of central nervous system was carried out in reconstructive period. The first group (n = 33) consisted of infants sustained reanimation in maternity department. The second group (n = 21) were composed by infants sustained reanimation in maternity department including artificial pulmonary ventilation for more than twenty four hours. The third group (n = 25) was control and were formed from newborns with perinatal lesions of central nervous system which didn't require reanimation. The severe long term neurological outcomes in the first and the second groups were detected. The pathology of nervous system in these groups consisted of motor disorders and severe arrested development. Ventriculodilatation had widespread character in these groups.Key words: reanimation, neurological outcomes, infants, motor disorders, ventriculodilatation
PSYCHONEUROLOGICAL ASPECTS OF JUVENILE RHEUMATOID ARTHRITIS
This review describes basic aspects of neurological pathology in children with juvenile rheumatoid arthritis. Results of reviewed trials show that neurological and cognitive disorders, presenting in patients with juvenile rheumatoid arthritis, worsen patientβs quality of life and need to be corrected with medicaments.Key words: children, juvenile rheumatoid arthritis, neurological patology.(Voprosy sovremennoi pediatrii βΒ Current Pediatrics. 2009;8(1):46-51
MULTIVITAMINOUS COMPLEXIS IN NEUROPEDIATRICS
Therapeutic effect of multivitaminous complexis has been assessed in the open controlled study included 50 patients with psychoneurological disorders, aged from 4 to 16 years for 28 days. No pharmacological therapy was conducted in group of a control which included 15 patients with same disorders. Utilising the computer testing systems, basic of parameters cognitive functions (CF) were analyzed before and after the course of therapy. Statistically relevant positive effect of the vitaminis on cognitive functions was demonstrated.Key words: multivitaminous complexis, children, treatment, psychoneurological disorders
MULTIVITAMINOUS COMPLEXIS IN NEUROPEDIATRICS
Therapeutic effect of multivitaminous complexis has been assessed in the open controlled study included 50 patients with psychoneurological disorders, aged from 4 to 16 years for 28 days. No pharmacological therapy was conducted in group of a control which included 15 patients with same disorders. Utilising the computer testing systems, basic of parameters cognitive functions (CF) were analyzed before and after the course of therapy. Statistically relevant positive effect of the vitaminis on cognitive functions was demonstrated.Key words: multivitaminous complexis, children, treatment, psychoneurological disorders
LONG TERM NEUROLOGICAL OUTCOMES OF NEWBORNS REANIMATION
The complex evaluation of infants with perinatal lesions of central nervous system was carried out in reconstructive period. The first group (n = 33) consisted of infants sustained reanimation in maternity department. The second group (n = 21) were composed by infants sustained reanimation in maternity department including artificial pulmonary ventilation for more than twenty four hours. The third group (n = 25) was control and were formed from newborns with perinatal lesions of central nervous system which didn't require reanimation. The severe long term neurological outcomes in the first and the second groups were detected. The pathology of nervous system in these groups consisted of motor disorders and severe arrested development. Ventriculodilatation had widespread character in these groups.Key words: reanimation, neurological outcomes, infants, motor disorders, ventriculodilatation
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