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.

UTILITY OF HEALTH STATE INDEX IN PEDIATRICS FOR THE EVALUATION OF EFFECTIVENESS OF MEDICAL INTERVENTIONS

So-called dynamical studies of health state including ?models of health status indexes? are actively performed in public health. Quality adjusted life year (QALY) is universal index of the result of treatment, and it can be used in estimation of all age groups and nosologic forms, it allows comparison between different diseases, strategies of health care and outcomes. Development of methods of QALY receipt in pediatrics is able to widen our opportunities and to improve an evaluation of effectiveness of treatment and prophylactic interventions. These measures may influence to the choice of more significant medical technologies for the treatment of diseases.Key words: quality adjusted life year, children, result of intervention, quality of life related to health.(Voprosy sovremennoi pediatrii — Current Pediatrics. – 2010;9(3):22-25)</p

UTILITY OF HEALTH STATE INDEX IN PEDIATRICS FOR THE EVALUATION OF EFFECTIVENESS OF MEDICAL INTERVENTIONS

So-called dynamical studies of health state including ?models of health status indexes? are actively performed in public health. Quality adjusted life year (QALY) is universal index of the result of treatment, and it can be used in estimation of all age groups and nosologic forms, it allows comparison between different diseases, strategies of health care and outcomes. Development of methods of QALY receipt in pediatrics is able to widen our opportunities and to improve an evaluation of effectiveness of treatment and prophylactic interventions. These measures may influence to the choice of more significant medical technologies for the treatment of diseases.Key words: quality adjusted life year, children, result of intervention, quality of life related to health.(Voprosy sovremennoi pediatrii — Current Pediatrics. – 2010;9(3):22-25

QUALITY OF LIFE OF CHILDREN WITH CHRONIC DISEASES

An urgency of studying of quality of life in clinical pediatrics is described in this article. Some results of trials, studying quality of life of children with chronic diseases was presented. These results include information of influence of chronic disease, peculiarities of quality of life in patients with different nosologic forms, long9term disorders of quality of life.Key words: children, quality of life, chronic diseases.</strong

QUALITY OF LIFE OF CHILDREN WITH CHRONIC DISEASES

An urgency of studying of quality of life in clinical pediatrics is described in this article. Some results of trials, studying quality of life of children with chronic diseases was presented. These results include information of influence of chronic disease, peculiarities of quality of life in patients with different nosologic forms, long9term disorders of quality of life.Key words: children, quality of life, chronic diseases

HEALTH STATE AND QUALITY OF LIFE IN GIRLS-ADOLESCENTS IN ORENBURG

The results of a trial studying health state and quality of life of girls-adolescents from Orenburg school of general education are presented in this article. The lowest rates of quality of life were detected in 14-year old girls. Estimation of some components of quality of life, given by parents and girls themselves differs. It was marked that girls-adolescents from III health group showed lower rates of quality of life than girls from I and II health groups. Low rates of functioning in school were marked in all girls-adolescents.Key words: girls-adolescents, morbidity, quality of life.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(5):9-13)</span

HEALTH STATE AND QUALITY OF LIFE IN GIRLS-ADOLESCENTS IN ORENBURG

The results of a trial studying health state and quality of life of girls-adolescents from Orenburg school of general education are presented in this article. The lowest rates of quality of life were detected in 14-year old girls. Estimation of some components of quality of life, given by parents and girls themselves differs. It was marked that girls-adolescents from III health group showed lower rates of quality of life than girls from I and II health groups. Low rates of functioning in school were marked in all girls-adolescents.Key words: girls-adolescents, morbidity, quality of life.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(5):9-13

QUALITY OF LIFE IN CHILDREN WITH CHRONIC DIARRHEA

The results of evaluation of quality of life in children from 1 to 3 years old with chronic diarrhea in exacerbation or remission with QUALIN questionnaire is presented in this article. Authors used two types of questionnaire: doctors' and parents'. Obtained data was compared to indices of healthy children. Proved, that children in early age with chronic diarrhea, as in exacerbation, as in remission, have significantly lower rates of quality of life compared to healthy coevals.Key words: children, quality of life, QUALIN questionnaire, chronic diarrhea.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(2):16-19)</span

QUALITY OF LIFE OF CHILDREN WITH GLOMERULAR KIDNEY DISEASES

The paper presents the results of a study of the life quality of children with chronic kidney diseases using a questionnaire Pediatric Quality of Life Inventory, which allows us to estimate the physical, emotional, social and role functioning. The authors included in the analysis the least studied in pediatric nephrology cohort of patients — children with intact renal function. According to the patients responses, regardless of the age, compared with healthy children, physical, emotional and social aspects were the lowest. In accordance with the parents responses the quality of children life have been reduced, mainly due to the physical component. The children of preschool age (5–7 years) showed the most marked reduction of all parameters of life quality. Key words: quality of life, chronic kidney disease, children. (Pediatric pharmacology. — 2011; 8 (6): 74–76)

QUALITY OF LIFE OF CHILDREN WITH GLOMERULAR KIDNEY DISEASES

The paper presents the results of a study of the life quality of children with chronic kidney diseases using a questionnaire Pediatric Quality of Life Inventory, which allows us to estimate the physical, emotional, social and role functioning. The authors included in the analysis the least studied in pediatric nephrology cohort of patients — children with intact renal function. According to the patients responses, regardless of the age, compared with healthy children, physical, emotional and social aspects were the lowest. In accordance with the parents responses the quality of children life have been reduced, mainly due to the physical component. The children of preschool age (5–7 years) showed the most marked reduction of all parameters of life quality. Key words: quality of life, chronic kidney disease, children. (Pediatric pharmacology. — 2011; 8 (6): 74–76)