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. Objective nonalcoholic non alcoholic (NAFLD (PKU Method crosssectional cross sectional 1 2 service documented established Students Student s ttest t test variables chisquare chi square chisquared squared 005 0 05 0.0 significant Results 25.7% 257 25 7 (25.7% teenagers status 26.1% 261 (26.1% 94.7%. 947 94.7% . 94 (94.7%) phosphatase Conclusions children modification 00 0. 25.7 (25.7 26.1 (26.1 94.7 9 (94.7% 25. (25. 26. (26. 94. (94.7 (25 (26 (94. (2 (94 ( (9

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.

Resumo Objetivos Apresentar a prevalência geral e caracterizar as deficiências de tetrahidrobiopterina - BH4 - com hiperfenilalaninemia, identificadas pelo Programa de Triagem Neonatal do Estadode Minas Gerais. Métodos Estudo descritivo de pacientes com deficiência de BH4 do Programa de Triagem Neonatal do Estado de Minas Gerais. Resultados A prevalência encontrada foi de 2,1 para 1.000.000 recém-nascidos vivos e a frequência de 1,71%, dentre as hiperfenilalaninemias. Quatro casos (40%) com deficiência de 6-piruvoil-tetrahidropterina sintase, três com deficiência de GTP ciclohidrolase I e três com deficiência de dihidropteridina redutase (30% cada um). Seis pacientes foram diagnosticadospor suspeita clínica e quatro pela pesquisa sistemática na triagem neonatal. Após o início do tratamento, os pacientes identificados pela triagem neonatal tiveram melhora rápida e melhor desenvolvimento neuropsicomotor em comparação com aqueles diagnosticados pela história clínica. Conclusões A prevalência das deficiências de BH4 em Minas Gerais foi um pouco maior que a encontrada na literatura, mas a frequência, entre as hiperfenilalaninemias, foi semelhante. Embora raras, são graves e, se não tratadas, levam a atraso de desenvolvimento, movimentos anormais, convulsões e morte precoce. O tratamento precoce (início antes dos 5 meses) mostrou bons resultados na prevenção de deficiência intelectual, justificando a pesquisa dessas deficiências nos recém-nascidos com hiperfenilalaninemia pelos programas de triagem neonatalpara fenilcetonúria.

Abstract Objectives To show the general prevalence and to characterize tetrahydrobiopterin (BH4) deficiencies with hyperphenylalaninemia, identified by the Neonatal Screening Program of the State of Minas Gerais. Methods Descriptive study of patients with BH4 deficiency identified by the Neonatal Screening Program of the State of Minas Gerais. Results The prevalence found was 2.1 for 1,000,000 live births, with a frequency of 1.71% among hyperphenylalaninemias. There were four cases (40%) with 6-pyruvoyl-tetrahydropterin synthase deficiency, three with GTP cyclohydrolase I - autosomal recessive form deficiency, and three with dihydropteridine reductase deficiency (30% each). Six patients were diagnosed due to clinical suspicion and four cases due to systematic screening in neonatal screening. After the start of the treatment, patients identified by neonatal screening had rapid improvement and improved neuropsychomotor development compared to those diagnosed by the medical history. Conclusions The prevalence of BH4 deficiencies in Minas Gerais was slightly higher than that found in the literature, but the frequency among hyperphenylalaninemias was similar. Although rare, they are severe diseases and, if left untreated, lead to developmental delays, abnormal movements, seizures, and premature death. Early treatment onset (starting before 5 months of age) showed good results in preventing intellectual disability, justifying the screening of these deficiencies in newborns with hyperphenylalaninemia identified at the neonatal screening programs for phenylketonuria.

OBJETIVO: Investigar o aleitamento materno exclusivo em bebês com fissura de lábio e/ou palato e sua associação com o tipo de fissura. MÉTODOS: Foram coletados dados de 137 prontuários, obtidos pela pediatra na primeira consulta. A amostra constituiu-se das fichas de pediatria de bebês de ambos os gêneros, com fissura de lábio e/ou palato, nascidos a termo e sem outras anomalias associadas, que chegaram para a primeira consulta entre zero e 12 meses. Os dados foram analisados estatisticamente. RESULTADOS: Houve predomínio da fissura transforame incisivo em bebês do gênero masculino. Os bebês apresentavam média de idade de 53 dias e mediana de 33 dias na primeira consulta. Quanto ao aleitamento materno exclusivo, este ocorreu em 7,3% do total da amostra, sendo 6,57% no grupo pré-forame incisivo e 0,73% no grupo pós-forame incisivo, mas não ocorreu no grupo transforame incisivo. Houve associação significativa entre o tipo de fissura e o aleitamento. CONCLUSÃO: O aleitamento exclusivo está presente em pequena parte da amostra total de bebês com fissura de lábio e/ou palato, sendo frequente na fissura pré-forame incisivo.

PURPOSE: To investigate exclusive breastfeeding in infants with cleft lip and/or palate and its association with the type of cleft. METHODS: Data recorded by the pediatrician during the first consultation were obtained from 137 clinical charts. The sample consisted of the pediatric records of infants of both genders, with cleft lip and/or palate, born full term and without any other associated anomalies, who arrived for the first consultation between zero and 12 months of age. Data were statistically analyzed. RESULTS: There was predominance of complete cleft lip and palate in male infants. Subjects' mean age was 53 days, with median of 33 days on the date of first consultation. Exclusive breastfeeding occurred in 7.3% of the total sample - 6.57% in cleft lip group, 0.73% in the cleft palate, and none in the complete cleft lip and palate group. There was significant association between type of cleft and breastfeeding. CONCLUSION: Exclusive breastfeeding is present in only a small part of the total sample of infants with cleft lip and/or palate, and is frequent in infants with incomplete cleft lip.

OBJETIVO: Investigar a prevalência do uso de sonda nasogástrica em bebês com fissura labiopalatina, sua correlação com tipo de fissura, maternidade e cidade de origem, e a idade na primeira consulta. MÉTODOS: A amostra constituiu-se de 137 bebês de ambos os gêneros, com fissura de lábio e/ou palato, sem outros comprometimentos, nascidos a termo, e que chegaram para primeira consulta em um centro especializado em fissura entre zero e 12 meses (mediana=33 dias). Realizou-se análise estatística pelo teste de coeficiente de contingência (p<0,05). RESULTADOS: Da amostra total, 61% eram do gênero masculino e 39% do feminino, 51% apresentavam fissura de lábio e palato, 35% de palato e 14% de lábio. Quanto ao nascimento, 36% nasceram em maternidades particulares e 64% em públicas, 60% em Belo Horizonte, 15% em outras cidades da região metropolitana e 25% no interior do estado. O uso de sonda ocorreu em 23% dos casos. Não houve associação entre tipo de fissura ou de maternidade e o uso de sonda, mas este foi mais frequente na região metropolitana (p=0,007). CONCLUSÃO: A prevalência do uso de sonda em bebês com fissura foi considerada alta, visto que nasceram a termo e não apresentavam comprometimentos associados que indicassem o uso da mesma. O uso de sonda é mais frequente em bebês nascidos em maternidades da região metropolitana de Belo Horizonte, quando comparados a outras cidades do estado de Minas Gerais.

PURPOSE: To investigate the prevalence of the use of nasogastric tube in babies with cleft lip and/or palate, and to correlate its use with type of cleft, maternity hospital (private or public) and city of origin, and age at the first visit to a cleft center. METHODS: The sample consisted of data collected from 137 babies of both genders with cleft lip and/or palate, without any other associated anomalies, born full-term, who attended their first consultation at a specialized cleft center with ages between zero and 12 months (median=33 days). Statistical analyses used the coefficient of contingency test (p<0,05). RESULTS: From the subjects, 61% were male and 39% were female; 51% presented cleft lip and palate, 35% cleft palate, and 14% cleft lip. Regarding place of birth, 36% were born in private and 64% in public maternity hospitals; 60% were born in the city of Belo Horizonte (Minas Gerais, Brazil), 15% in other cities of the metropolitan area, and 25% in other cities at the state of Minas Gerais (Brazil). The use of nasogastric tube was reported in 23% of the cases. There was no association between the type of cleft or maternity and the use of the tube, but the later was more frequent within the metropolitan area (p=0,007). CONCLUSION: The prevalence of the use of nasogastric tube in babies with cleft lip and/or palate was considered high since they were born full-term and without any other associated anomalies that would indicate the need of the tube. The use of nasogastric tube was more frequent in babies born at the metropolitan area of Belo Horizonte, when compared to other cities in the state of Minas Gerais (Brazil).

A pandemia da obesidade juvenil e do sedentarismo está relacionada com o maior risco de doenças cardiovasculares no futuro. Ambas com fisiopatologia inflamatória, essas condições têm atraído significativa atenção científica. O início dos eventos que agridem o endotélio vascular pode ocorrer na infância, porém, sinais clínicos da aterosclerose geralmente só aparecem na fase adulta. O tecido adiposo é hoje reconhecido por seu papel ativo, induzindo a produção de citocinas pró e anti-inflamatórias, como o fator de necrose tumoral-alfa, as interleucinas 1, 6 e 10, a proteína C-reativa e outras. Há várias evidências de que os níveis sanguíneos dessas citocinas se encontram mais altos em indivíduos com excesso de peso, inclusive crianças e adolescentes. A prevenção precoce, desde a infância, apresenta-se como a melhor maneira de evitar os danos da obesidade na fase adulta, enquanto o potencial da prática regular de exercícios físicos tem se mostrado surpreendente. Sua ação anti-inflamatória se manifesta através de menores concentrações séricas de interleucina 6, de proteína C-reativa e leptina, além de aumentos na adiponectina. Observa-se escassez de estudos randomizados e controlados avaliando as relações entre obesidade, inflamação e exercícios para a população jovem. Com resultados às vezes controversos, a maioria das conclusões é procedentede estudos com adultos. O objetivo desta revisão é avaliar o papel anti-inflamatório, e assim cardioprotetor, da atividade física regular na obesidade infantojuvenil.

Nowadays, juvenile obesity and physical inactivity are pandemic conditions which relate to a greater future risk of cardiovascular diseases. From an inflammatory point of view, they have attracted massive scientific attention. The beginning of the events related to atherosclerosis may occur in childhood, generating endothelial and metabolic dysfunction; however, the symptoms usually only appear later on, in adulthood. The fat tissue is recognized as being metabolically active, stimulating the production of inflammatory cytokines, such as the tumoral necrosis factor, interleukines 1, 6 and 10, C-reactive protein, among others. There is plenty of evidence that the serum levels of these citokines are higher in overweight individuals, including children and adolescents. Early prevention as young as possible is the best way to avoid future consequences of obesity. In this context, the potential benefits of regular physical exercise have been surprising. Researchers have shown evidence of anti-inflammatory responses, including lower levels of interleukine 6, C-reactive protein, leptin, besides higher levels of adiponectins after engagement in regular physical activity. However, we still have few controlled randomized studies addressing the relations between obesity, inflammation and exercise for the pediatric population. There are controversial findings in this field, and many of them come from adult studies. Thus, the purpose of the present review is to evaluate the metabolic role of physical exercise in juvenile obesity, aiming at heart protection.

OBJETIVOS: determinar os motivos de encaminhamento e diagnósticos de crianças com anemia para serviço especializado. MÉTODOS: coorte histórico e concorrente de crianças com anemia encaminhadas ao Serviço de Hematologia do Hospital das Clínicas da Universidade Federal de Minas Gerais, Belo Horizonte, Minas Gerais, Brasil, entre maio de 1999 e dezembro de 2001. RESULTADOS: foram avaliadas 153 crianças menores de 16 anos, sendo 83% procedentes de Centros de Saúde. Aproximadamente um terço das crianças não recebeu qualquer terapêutica no serviço de origem. Cento e onze (71,3%) tiveram diagnóstico de anemia ferropriva. Treze crianças (8,6%) tiveram diagnóstico de talassemia minor; outras treze crianças apresentaram outros diagnósticos de anemias e 11,5% das crianças não apresentavam anemia. Os valores médios de hemoglobina na abordagem pelo hematologista foram significativamente maiores que os dos serviços de origem (9,7±1,9 vs. 8,9±2,0); p=0,001. Somente 16,2% crianças não responderam ao primeiro tratamento com sais de ferro oral, sendo o sulfato ferroso o principal medicamento prescrito (79,3%). CONCLUSÕES: A deficiência de ferro foi a principal etiologia das crianças encaminhadas por motivo de anemia para serviço especializado. Encaminhamentos desnecessários de indivíduos com anemia ferropriva para serviços especializados representam prejuízo aos pacientes e ônus excedente para o sistema de saúde.

OBJECTIVES: to determine the reasons for diagnosis of anemia among children and referral to specialized services. METHODS: an historical and concurrent cohort study was carried out among anemic children referred to the Hematology Service of the Federal University of Minas Gerais's Clinical Hospital, Belo Horizonte, in the State of Minas Gerais, Brazil, between May 1999 and December 2001. RESULTS: 153 children aged under 16 were evalua-ted, 83% of whom had been referred by Health Centers. Approximately one third of the children did not receive any treatment at the referring service. One hundred and eleven (71.3%) were diagnosed as having iron-deficiency anemia. Thirteen children (8.6%) received a diagnosis of thalassemia minor; and a further thirteen other anemia-related diagnoses. 11.5% had no kind of anemia. The mean hemoglobin levels were significantly higher when tested by a hematologist than at the referring services (9.7±1.9 vs. 8.9±2.0); p=0.001. Only 16.2% of children did not respond to initial treatment with oral iron salts, with iron (II) sulfate being the medication usually prescribed (79.3%). CONCLUSIONS: iron deficiency was the main cause of children being referred to a specialized service for reason of anemia. Unnecessary referral of individuals with iron-deficiency anemia is potentially harmful to patients and puts an excessive burden on the health system.

OBJETIVO: Avaliar o estado nutricional e a prevalência de anemia em crianças de sete a 74 meses, que freqüentam creches. MÉTODOS: Estudo transversal em 25 creches da regional leste de Belo Horizonte, Minas Gerais. Realizaram-se avaliação antropométrica e dosagem de hemoglobina por hemoglobinômetro portátil. Considerou-se anemia se hemoglobina <11,0g/dL para crianças menores que 60 meses e <11,5g/dL, para maiores de 5 anos. Na avaliação do estado nutricional, utilizaram-se os índices peso/idade, estatura/idade e peso/estatura, sendo categorizados em três intervalos: abaixo de -2 escores Z (desnutrição/baixa estatura), de -2 a -1 escore Z (risco para desnutrição/baixa estatura) e maior ou igual a -1 escore Z. A análise estatística incluiu: qui-quadrado, qui-quadrado de tendência linear e análise de variância (ANOVA) seguida pelo teste de Tukey, sendo significante p<0,05. RESULTADOS: Foram avaliadas 402 crianças, com mediana de idade de 46,3 meses. A prevalência de anemia foi 28,8%, observando-se o dobro da prevalência nas crianças com idade inferior a 24 meses (70,4%), comparada às de faixa etária maior. A prevalência de desnutrição correspondeu a 5,0 e 5,5% da população para os índices peso/estatura e peso/idade, respectivamente. A prevalência de baixa estatura foi 4,2%. Em crianças menores de 5 anos, a anemia associou-se à idade e ao deficit estatural. CONCLUSÕES: A anemia em crianças matriculadas nas creches da regional leste de Belo Horizonte constitui importante problema de saúde pública, acometendo, principalmente, os menores de 24 meses. Os deficits nutricionais estiveram acima dos valores esperados.

OBJECTIVE: Assess the prevalence of anemia and the nutritional status of children with 7-74 months old, enrolled in day care centers. METHODS: This cross-sectional study enrolled children of 25 day care centers of the eastern district of Belo Horizonte city, Minas Gerais, Brazil. Anthropometric parameters were evaluated and hemoglobin count was done by a portable hemoglobinometer. The cutoff level for anemia was 11.0g/dL for children less than 60 months, and 11.5g/dL for those older than 5 years old. Nutritional status was assessed by anthropometric indexes: weight-for-age, height-for-age and weight-for-height and classified in 3 groups: below -2 Z score (malnutrition/height deficit), -2 to -1 Z score (nutritional risk for malnutrition/height deficit) and above or equal to -1 Z score. Statistical analysis included: chi-square, chi-square with linear trend, analysis of variance (ANOVA) followed by Tukey test, being significant p<0.05. RESULTS: 402 children, with a median age of 46.3 months, were assessed. The overall anemia prevalence was 28.8%. The rate of anemia in children <24 months (70.4%) was twice that observed in older children. The prevalence of malnutrition was 5,0 and 5.5%, as defined by weight-for-height and weight-for-age indexes, respectively. The prevalence of height deficit was 4.2%. Malnutrition was associated to age and height deficit in children <5 years old. CONCLUSIONS: Anemia in children enrolled in day care centers in Belo Horizonte is a serious public health problem, especially in those younger than 24 months. Nutritional deficits were beyond the expected values.