Nonalcoholic fatty liver disease in adolescents with phenylketonuria.

OBJECTIVE: This study aimed to investigate non-alcoholic fatty liver disease (NAFLD) occurrence and factors associated with the disease in phenylketonuria (PKU) patients undergoing exclusive dietary treatment. METHOD: This cross-sectional study included 101 adolescents 10 to < 20 years of age with PKU, who were undergoing exclusive dietary treatment and monitored since early diagnosis at a single reference service. Anthropometric and biochemical assessments were performed and food intake was documented, and an ultrasound diagnosis of NAFLD was established. Data were evaluated using the Student's t-test for continuous variables, the chi-square for categorical variables, and logistic regression using the Wald chi-squared test; differences with p < 0.05 were considered to be statistically significant. RESULTS: NAFLD was detected in 26 (25.7%) teenagers. There was no difference in prevalence between the sexes or nutritional status. The final logistic regression model revealed low sensitivity (26.1%) and high specificity (94.7%). The specificity suggested a lower likelihood of NAFLD in older adolescents, in the presence of normal or high levels of alkaline phosphatase, lower carbohydrate intake, and adequate protein and lipid intake. CONCLUSIONS: The prevalence of NAFLD in adolescents with PKU was higher than that found in healthy Brazilian adolescents and similar to that found in obese Brazilian children, suggesting a higher risk for NAFLD in patients with PKU treated exclusively by dietary modification.

Overweight/obesity in adolescents with phenylketonuria: protective and predisposing factors.

OBJECTIVE: To estimate the prevalence and factors associated with overweight/obesity development in adolescents with early diagnosed phenylketonuria treated exclusively by diet. METHODOLOGY: In this cross-sectional study anthropometric measurements, serum phenylalanine levels, and 10 metabolites associated with lipid and carbohydrate metabolism were analyzed in 101 adolescents aged 10-20 years. Adolescents were categorized into overweight/obesity and eutrophic/low body mass index groups. These patients were compared using Student's t-test, Pearson's chi-square test, Wald's chi-square test for multivariate analysis. Further, to verify whether the prevalence of overweight/obesity found in the study population was similar to that in the general population, the authors compared the nutritional status of 46 patients aged 13-17 years with that of healthy students of the same age from the National School Health Survey using the chi-square test for adherence. The significance threshold was p < 0.5. RESULTS: The prevalence of overweight/obesity in adolescents was 27.7%. There was no difference in prevalence between sexes. Older age was a protective factor and Increased Homeostasis Model Assessment Insulin Resistance index and high phenylalanine and low-density lipoprotein cholesterol levels were predictive factors for overweight/obesity. The equality hypothesis was not rejected in the comparison of nutritional states of 46 patients aged 13-17 years and healthy students of the same age. CONCLUSION: The prevalence of overweight/obesity in phenylketonuria adolescents was similar to what is found in healthy adolescents.

Nonalcoholic fatty liver disease in adolescents with phenylketonuria

Abstract Objective: This study aimed to investigate non-alcoholic fatty liver disease (NAFLD) occurrence and factors associated with the disease in phenylketonuria (PKU) patients undergoing exclusive dietary treatment. Method: This cross-sectional study included 101 adolescents 10 to < 20 years of age with PKU, who were undergoing exclusive dietary treatment and monitored since early diagnosis at a single reference service. Anthropometric and biochemical assessments were performed and food intake was documented, and an ultrasound diagnosis of NAFLD was established. Data were evaluated using the Student's t-test for continuous variables, the chi-square for categorical variables, and logistic regression using the Wald chi-squared test; differences with p < 0.05 were considered to be statistically significant. Results: NAFLD was detected in 26 (25.7%) teenagers. There was no difference in prevalence between the sexes or nutritional status. The final logistic regression model revealed low sensitivity (26.1%) and high specificity (94.7%). The specificity suggested a lower likelihood of NAFLD in older adolescents, in the presence of normal or high levels of alkaline phosphatase, lower carbohydrate intake, and adequate protein and lipid intake. Conclusions: The prevalence of NAFLD in adolescents with PKU was higher than that found in healthy Brazilian adolescents and similar to that found in obese Brazilian children, suggesting a higher risk for NAFLD in patients with PKU treated exclusively by dietary modification.

Overweight/obesity in adolescents with phenylketonuria: protective and predisposing factors

Abstract Objective: To estimate the prevalence and factors associated with overweight/obesity development in adolescents with early diagnosed phenylketonuria treated exclusively by diet. Methodology: In this cross-sectional study anthropometric measurements, serum phenylalanine levels, and 10 metabolites associated with lipid and carbohydrate metabolism were analyzed in 101 adolescents aged 10-20 years. Adolescents were categorized into overweight/obesity and eutrophic/low body mass index groups. These patients were compared using Student's t-test, Pearson's chi-square test, Wald's chi-square test for multivariate analysis. Further, to verify whether the prevalence of overweight/obesity found in the study population was similar to that in the general population, the authors compared the nutritional status of 46 patients aged 13-17 years with that of healthy students of the same age from the National School Health Survey using the chi-square test for adherence. The significance threshold was p < 0.5. Results: The prevalence of overweight/obesity in adolescents was 27.7%. There was no difference in prevalence between sexes. Older age was a protective factor and Increased Homeostasis Model Assessment Insulin Resistance index and high phenylalanine and low-density lipoprotein cholesterol levels were predictive factors for overweight/obesity. The equality hypothesis was not rejected in the comparison of nutritional states of 46 patients aged 13-17 years and healthy students of the same age. Conclusion: The prevalence of overweight/obesity in phenylketonuria adolescents was similar to what is found in healthy adolescents.

Significando a morte, através de redes sociais, em um contexto de vulnerabilidade social: um estudo com crianças pré-escolares, seus pais e professores / The meaning of death, through social networks, in a socially vulnerable context: a study with children, their parents and their teachers

O presente artigo resulta de uma pesquisa realizada durante 2004, em um bairro popular de Salvador, com o objetivo de compreender os processos de significação da morte, apresentados por um grupo de crianças pré-escolares, que se encontravam em uma situação de vulnerabilidade social. O intuito primordial não foi descrever tais significados, mas compreender como o contexto vivenciado, intermediado pelas relações com pais e outros educadores, promoviam o surgimento de determinadas falas e comportamentos por parte das crianças. No contexto investigado, as crianças se encontravam expostas a mortes decorrentes de conflitos relacionados ao tráfico de drogas. Para atingir tal objetivo, foi desenvolvido um estudo exploratório, em uma creche, na qual entrevistamos doze crianças, nove dos seus responsáveis e quatro educadores. Os instrumentos foram adequados de acordo com cada um dos grupos. Como resultado do estudo, observamos que, apesar das crianças conviverem com situações de violência e morte, os pais encontram grandes dificuldades em abordarem o tema com as mesmas que não obstante trazem, principalmente para a escola, através da fala e das brincadeiras, as experiências vividas em sua comunidade.

This paper is the result of a research carried out in 2004, in a poor area of the city of Salvador. The objective was to understand the process of the meaning of death to a group of socially vulnerable preschool children. The study focused on the children's social network of parents and other educators in order to understand how these relationships gave a social basis to the construction of death meanings. In the social context of investigation, the children were frequently exposed to deaths caused by drug related conflicts. The research was carried out as an exploratory study, in a day care center with twelve children, nine of their caretakers and four teachers. The instruments were adapted to each group. As a result of this study, we observed that the parents had a hard time approaching the subject of violence and death with the children, although they lived in a context of violence and death. Nevertheless, these children would bring to school, through play and talk, their experiences of violence and death in their community.