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Objective: To compare the phase angle (PhA) through bioelectrical impedance (BIA) of children with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by an Intestinal Rehabilitation Program, with a control group. Methods: Children under 10 years of age with IF using prolonged PN for >60 days (study group) were included. The control group consisted of healthy children without chronic pathologies, matched by sex and age. Anthropometric parameters evaluated were: weight, height, weight/age z-score (W/A), height/age z-score (H/A), BMI, BMI/A z-score, arm circumference, triceps skinfold, subscapular skinfold, mid-arm muscle circumference. BIA parameters were resistance (R), reactance (Xc), and phase angle (PhA). Results: Twenty-eight children were included in the study group, median (IQR) age was 11 (8–27) months, 53.6 % were male. In the control group, 28 children were included, median (IQR) age was 12.5 (8–24.7) months, 50 % were male. Children from the study group had W/A z-scores and H/A z-scores significantly lower than controls. There was no significant difference between PhA in the study group and controls, [median (IQR) 4.3° (3.8;4.6) vs 4.0° (3.8;5.4) respectively, p = 0.980]. Prematurity was significantly higher in the study group than in the controls, but there was no significant correlation between gestational age at birth and PhA of the children from the study group. Conclusion: Children with IF using prolonged PN showed lower W/A and H/A compared to the control group, but without significant difference between the PhA of children with IF compared to controls.

Soil properties that are considered difficult to measure are frequently determined through pedotransfer functions (PTFs). Calibration and validation datasets, containing measurements of the target soil property as well as widely available basic soil attributes, are needed to construct and validate PTFs. However, the target soil property data are prone to measurement errors, which are typically ignored when deriving new PTFs. In this contribution, we considered a PTF that predicts the soil cation exchange capacity (CEC) and aimed to study the effect of additive and multiplicative measurement error in the CEC calibration data on multiple linear regression (MLR) and random forest (RF) PTF predictions and associated prediction uncertainty. We used data from the National Cooperative Soil Survey Soil Characterization (NCSS-SC) Database, which unfortunately did not contain repeated measurements. Therefore, data from the Wageningen Evaluating Programmes for Analytical Laboratories (WEPAL) were used to quantify measurement error variance in CEC data. We assumed that the obtained measurement error variance was representative for the NCSS-SC Database CEC data, and used it to generate a probability distribution of the CEC measurement error. We used Monte Carlo simulations to add measurement errors to the original CEC values and fitted MLR- and RF-PTFs to ‘error-free’ and ‘error-contaminated’ datasets to study the effect of measurement error. For the MLR-PTFs, measurement error did not lead to large differences in the mean estimated model coefficients when compared to the estimates based on the ‘true’ CEC. Similarly, the variable importance scores of the RF-PTFs were comparable. However, a relationship between dataset size and the standard deviations of the model coefficient estimates and variable importance scores was present. Furthermore, the model performance reduced slightly (MEC decreased between 0.09 and 2.53%) compared to the MLR-PTFs fitted on the ‘true’ CEC. A larger effect of measurement error on model performance was found for the RF-PTFs, where the MEC decreased between 1.52 and 31.59%. The study showed that measurement error in the calibration data can impact the calibration and statistical validation of PTFs, especially when using small datasets or when measurement error variance is large.

The main aim of the study is to evaluate the effects of the pharmaceutical diphenhydramine (DPH) on embryo-larvae Danio rerio across distinct levels of organization – individual and subcellular – and correlate those effects with the DPH mode of action (MoA) assessed by in silico analysis. An embryos heartbeat rate reduction was observed at 10 mg/L DPH, but 0.001 to 10 mg/L did not significantly affect the zebrafish survival, hatching and morphology. Larvae swimming distance decreased (hypoactivity) at 1 and 10 mg/L DPH. Moreover, the straightforward movements decrease and the increase in the zigzag movements or movements with direction changes, shown an erratic swimming behavior. Energy budgets decreased for lipid (0.01 mg/L DPH) and carbohydrate (10 mg/L DPH) contents. Cholinesterase (neural function) and glutathione S-transferase (Phase II biotransformation/antioxidant processes) increased their activities at 10 mg/L DPH, where a decrease in the total glutathione content (antioxidant system) was observed. DNA damage was found at 0.01 and 10 mg/L DPH. However, a DNA repair occurred after subsequent 72 h in clean media. The in silico study revealed a relevant conservation between human and zebrafish DPH target molecules. These data provide a valuable ecotoxicological information about the DPH effects and MoA to non-target organisms.

Objective: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. Methods: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments. Results: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%). Conclusions: In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death. Resumo: Objetivo: Este estudo com acompanhamento de longo prazo visou avaliar o quadro clínico, os achados laboratoriais, o perfil histológico, os tratamentos e os resultados de crianças e adolescentes com hepatite autoimune. Métodos: Foram analisados os prontuários médicos de 828 crianças e adolescentes com HAI. Foi usado um questionário para coletar os dados anônimos sobre o quadro clínico, os achados bioquímicos e histológicos e os tratamentos. Resultados: De todos os pacientes, 89,6% tinham hepatite autoimune-1 e 10,4% hepatite autoimune-2. O sexo feminino foi predominante nos dois grupos. A idade média no início dos sintomas foi 111,5 (6; 210) e 53,5 (8; 165) meses nos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente. Foi observado início clínico agudo em 56,1% e 58,8% e sintomas insidiosos em 43,9% e 41,2% dos pacientes com hepatite autoimune -1 e hepatite autoimune-2, respectivamente. A probabilidade de insuficiência hepática foi 1,6 vezes maior para hepatite autoimune-2; 3,6% e 10,6% dos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente, apresentaram insuficiência hepática fulminante; o risco foi 3,1 vezes maior para hepatite autoimune-2. Os níveis de gamaglobulina e imunoglobulina G foram significativamente maiores nos pacientes com hepatite autoimune-1, ao passo que os níveis de imunoglobulina A e C3 foram menores em pacientes com hepatite autoimune-2; 22,4% dos pacientes apresentaram cirrose e a remissão bioquímica foi atingida em 76,2%. A taxa de sobrevida atuarial foi de 93,0%. Um total de 4,6% pacientes foram submetidos a transplante de fígado e 6,9% morreram (hepatite autoimune-1: 7,5%; hepatite autoimune-2: 2,4%). Conclusões: Nesta grande série clínica de crianças e adolescentes brasileiros, a hepatite autoimune-1 foi mais frequente e os pacientes com hepatite autoimune-2 mostraram maiores taxas de remissão da doença com respostas mais rápidas aos tratamentos. Os pacientes com hepatite autoimune-1 apresentaram maior risco de óbito. Keywords: Autoimmune hepatitis, Clinical, Laboratory, Treatments, Outcomes, Brazil, Palavras-chave: Hepatite autoimune, Clínico, Laboratorial, Tratamentos, Resultados, Brasil

This article presents new data on individual altruistic choices and beliefs in a two-player sharing game, collected for the study reported in te Velde (2018) [2]. The decision maker in this game had to choose how much of a fixed budget to share with another anonymous Amazon Mechanical Turk user participating in the same study. With a known probability, the decision maker׳s choice was overridden by one of two known default options, so that the recipient was unable to attribute those two outcomes to the decision maker with certainty. In addition to choice data, each participant׳s incentivized guess of average behavior is included in the dataset, along with responses to a set of survey questions asking for opinions or beliefs about the morality of different options or the preferences or experienced utility of the recipient. Lastly, the dataset contains basic demographic information for each participant, along with detailed timing information about recruitment and each phase of the study.

Objectives: To transculturally adapt and validate the Karitane Parenting Confidence Scale to the Brazilian Portuguese language and culture and verify the combination of the results with the maternal sociodemographic characteristics. Methodology: This is a validation and transcultural adaptation nestled in a longitudinal and observational study in Porto Alegre, RS, Brazil, assessing mother-infant pairs from different gestational and perinatal environments. The original authors authorized the translation into Brazilian Portuguese, unified version creation, back-translation, analysis by specialists, final version implementation, and acceptance. Cronbach's alpha analysis was performed. The Kruskal–Wallis test with post-hoc Dunn's test was used to compare the study groups. Socioeconomic and demographic characteristics, obtained through a questionnaire in the first 24-48 h of the newborns’ life, were associated with maternal results by the Brazilian version of the scale, using Spearman's correlation and Mann–Whitney's test. Results: The sample consisted of 251 postpartum women, with the confidence maternal questionnaire being applied at 15 days postpartum. The median score of the mothers’ confidence was 40.00 (37.00–43.00). The protocol obtained a Cronbach's alpha of 0.717. There were significant weak positive correlations between maternal confidence and age (p = 0.013, r = 0.157) and between maternal confidence and schooling (p = 0.048, r = 0.125). Additionally, a significant association was observed between maternal confidence and parity (p = 0.030). Conclusion: The transcultural adaptation and validation of the confidence maternal questionnaire into Brazilian Portuguese language and culture showed good reliability for this sample. The results of its use demonstrated that maternal confidence was associated with schooling, age and parity. Resumo: Objetivos: Adaptar transculturalmente e validar a ferramenta Karitane Parenting Confidence Scale para a língua portuguesa e cultura brasileira além de verificar a associação de seus resultados com as características sociodemográficas maternas. Metodologia: Trata-se da validação e adaptação transcultural aninhada a estudo observacional longitudinal realizado em Porto Alegre (RS), contendo puérperas com diferentes condições gestacionais e perinatais. Os processos ocorreram mediante autorização dos autores originais da escala Karitane Parenting Confidence Scale para a tradução para o português brasileiro, montagem de versão unificada, retradução, análise por experts, aplicação da versão final e validação. Realizou-se a análise Alpha de Cronbach. Para a comparabilidade entre os grupos do estudo utilizou-se o teste Kruskal-Wallis com post hoc de Dunn. As características socioeconômicas e demográficas das puérperas, obtidas através de questionário estruturado nas 24-48 h pós-parto, foram relacionadas com a confiança materna obtida através da aplicação da escala, utilizando-se a correlação de Spearman e o teste Mann-Whitney. Resultados: A amostra foi composta por 251 puérperas, com a aplicação do questionário sobre confiança materna aos 15 dias pós-parto. A mediana da pontuação de confiança materna foi 40,00 [37,00-43,00]. O protocolo obteve valor de Alpha de Cronbach de 0,717. Houve correlações fracas significativas positivas entre confiança e idade materna (p = 0,013; r = 0,157) e entre confiança e escolaridade materna (p = 0,048; r = 0,125). Além disso, houve associação significativa entre a confiança materna e a paridade (p = 0,030). Conclusão: A adaptação transcultural e validação da ferramenta sobre a confiança materna para o português brasileiro mostrou boa confiabilidade. Os resultados de sua aplicação demonstraram que a confiança materna esteve associada à escolaridade, à idade e à paridade. Keywords: Validation studies, Confidence, Postpartum period, Mother–child relations, Palavras-chave: Estudos de validação, Confiança, Período pós-parto, Relações mãe-criança

Circadian clocks regulate various aspects of photoreceptor physiology, but their contribution to photoreceptor development and function is unclear. Cone photoreceptors are critical for color vision. Here, we define the molecular function of circadian activity within cone photoreceptors and reveal a role for the clock genes Bmal1 and Per2 in regulating cone spectral identity. ChIP analysis revealed that BMAL1 binds to the promoter region of the thyroid hormone (TH)-activating enzyme type 2 iodothyronine deiodinase (Dio2) and thus regulates the expression of Dio2. TH treatment resulted in a partial rescue of the phenotype caused by the loss of Bmal1, thus revealing a functional relationship between Bmal1 and Dio2 in establishing cone photoreceptor identity. Furthermore, Bmal1 and Dio2 are required to maintain cone photoreceptor functional integrity. Overall, our results suggest a mechanism by which circadian proteins can locally regulate the availability of TH and influence tissue development and function.

In the present work, venoms from five species of the genus Agkistrodon were evaluated in terms of their enzymatic (Phospholipase A2 and caseinolytic) and biological (edema forming, hemorrhagic, procoagulant and lethal) effects. Horses were used to produce monovalent hyperimmune sera against each of three venoms (A. bilineatus, A. contortrix and A. piscivorus) and their neutralizing potency, expressed as Median Effective Dose (ED50), was determined against the venoms of all five species. In terms of PLA2 and caseinolytic activities, all venoms are extremely homogeneous. PLA2 activity is high, while caseinolytic activity is low when in contrast with that of the rattlesnake Crotalus simus. On the other hand, biological activities showed marked interspecific differences, particularly between the species from Mexico and those from the United States. Mexican species displayed higher edema-forming, hemorrhagic and lethal effects than US species, while none of the species studied presented procoagulant activity. All three monovalent hyperimmune sera showed good neutralizing potency against the analyzed venoms. Nonetheless, we observed relevant immunochemical differences among the venoms using ELISA and Western Blot assays. We conclude that the venoms of A. piscivorus (USA) and A. bilineatus would be ideal to use as immunogens for the production of a polyvalent antivenom with good neutralizing potency against the venoms of all the species of the genus. Keywords: Agkistrodon venoms, Antivenom neutralization, Edema, Hemorrhage, Immunochemistry

The practice of intensive herbicide use in the sugarcane industry has a high risk of compromising the quality of the water and the organisms that live there due to losses through runoff, leaching and other processes. In this work, the dynamics of four herbicides present in three different mixtures were evaluated through their incorporation and elimination in the muscle tissue of tilapia (Oreochromis niloticus). The highest mean values of bioconcentration factors were 1.730 for ametryn, 0.891 for tebuthiuron, 0.322 for hexazinone and 4.783 for diuron. Diuron presented the highest risk regarding the consumption of tilapia fillets by the population. However, considering that the fish would reach maximum levels of diuron when exposed to extremely high concentrations, an individual weighing 70 kg would need to ingest approximately 1.5 kg of this food product to surpass the acceptable daily intake of 0.007 mg kg−1 body weight. It was concluded that the risk of injury to the population consuming tilapia fillets from fish exposed to herbicides in water arising from sugarcane activities is very low.According to the risk estimation performed in this work, which is substantiated by the assumptions of the World Health Organization and the International Life Sciences Institute, there is a low risk of injury to the population consuming tilapia fillets from fish exposed to water containing herbicides in concentrations arising from sugarcane activities. However, as the risk was estimated from laboratory conditions, caution should be taken where herbicide applications are carried out with high frequency near water bodies, as the consumption of fish from these areas is quite common.

ABSTRACT OBJECTIVES: To describe prevalence of associated defects and clinical-genetic characteristics of patients with typical orofacial clefts seen at a reference genetic service. METHODS: Descriptive study conducted between September of 2009 and July of 2014. Two experienced dysmorphologists personally collected and coded clinical data using a validated, standard multicenter protocol. Syndromic cases were defined by the presence of four or more minor defects, one or more major defects, or recognition of a specific syndrome. Fisher's exact and Kruskal-Wallis tests were used for statistics. RESULTS: Among 141 subjects, associated defects were found in 133 (93%), and 84 (59.5%) were assigned as syndromic. Cleft palate was statistically associated with a greater number of minor defects (p < 0.0012) and syndromic assignment (p < 0.001). Syndromic group was associated with low birth weight (p < 0.04) and less access to surgical treatment (p < 0.002). There was no statistical difference between syndromic and non-syndromic groups regarding gender (p < 0.55), maternal age of 35 years and above (p < 0.50), alcohol (p < 0.50) and tobacco consumption (p < 0.11), consanguinity (p < 0.59), recurrence (p < 0.08), average number of pregnancies (p < 0.32), and offspring (p < 0.35). CONCLUSIONS: There is a lack of information on syndromic clefts. The classification system for phenotype assignment adopted in this study has facilitated recognition of high prevalence of associated defects and syndromic cases. This system may be a useful strategy to gather homogeneous samples, to elect appropriate technologies for etiologic and genotype-phenotype approaches, and to assist with multiprofessional care and genetic counseling.

The appropriate trafficking of glutamate receptors to synapses is crucial for basic synaptic function and synaptic plasticity. It is now accepted that NMDA receptors (NMDARs) internalize and are recycled at the plasma membrane but also exchange between synaptic and extrasynaptic pools; these NMDAR properties are also key to governing synaptic plasticity. Scribble1 is a large PDZ protein required for synaptogenesis and synaptic plasticity. Herein, we show that the level of Scribble1 is regulated in an activity-dependent manner and that Scribble1 controls the number of NMDARs at the plasma membrane. Notably, Scribble1 prevents GluN2A subunits from undergoing lysosomal trafficking and degradation by increasing their recycling to the plasma membrane following NMDAR activation. Finally, we show that a specific YxxR motif on Scribble1 controls these mechanisms through a direct interaction with AP2. Altogether, our findings define a molecular mechanism to control the levels of synaptic NMDARs via Scribble1 complex signaling.

OBJETIVO: O objetivo deste estudo foi determinar a influência do aleitamento materno na capacidade intelectual de crianças pertencentes a uma coorte de um país em desenvolvimento, controlando para os principais fatores de confusão. MÉTODOS: Foi realizado um estudo de coorte prospectiva com todos os recém-nascidos nos hospitais de uma cidade de porte médio e acompanhou-se uma amostra aleatória destes aos 30, 90 e 180 dias de vida e aos 8 anos. No acompanhamento foram avaliados diversos aspectos da amamentação e, aos 8 anos, foi realizada avaliação da capacidade intelectual geral aplicando-se o Teste de Raven. As análises estatísticas utilizaram Teste T, ANOVA e regressão linear e logística, considerando como associações estatisticamente significativas aquelas cujo valor de p foi inferior a 0,05. RESULTADOS: Aos 8 anos 560 crianças participaram da avaliação com o Teste de Raven. A média da pontuação das crianças no teste foi de 22,56 pontos com desvio padrão de 5,93. A diferença de médias entre os grupos em aleitamento materno ou não, aos seis meses, foi de 1,33 (p = 0,008). Permaneceram associadas ao desfecho, após regressão linear e logística, a cor materna e da criança, classe socioeconômica, escolaridade e tabagismo maternos e o aleitamento materno aos 6 meses de idade (p = 0,007). CONCLUSÕES: As crianças que mamaram por seis meses ou mais tiveram melhor desempenho na avaliação intelectual geral, mesmo após ajuste para os principais fatores de confundimento.

OBJETIVO: Determinar se o número de áreas anatômicas envolvidas pode modificar os grupos de risco padrão no linfoma de Hodgkin pediátrico, identificando as crianças que poderiam se beneficiar de uma redução da intensidade do tratamento. MÉTODOS: Estudo retrospectivo com avaliação de idade, sexo, histologia, classificação de Ann-Arbor, presença de sintomas B, número de áreas anatômicas envolvidas, grupos de risco (favorável versus desfavorável) e exames laboratoriais. Todos os pacientes receberam quimioterapia com doxorrubicina. Os pacientes em remissão completa por 5 anos ou mais foram avaliados para a detecção de efeitos tardios. RESULTADOS: Sessenta e nove pacientes (2-18 anos) foram incluídos, sendo que 68% pertenciam ao grupo de risco desfavorável. A sobrevida global e a sobrevida livre de eventos foram de 94 e 87%, respectivamente. Os efeitos tardios foram detectados em 46 casos. Estágio avançado e > quatro áreas anatômicas envolvidas tiveram impacto negativo sobre a sobrevida livre de eventos, enquanto que o número de áreas anatômicas envolvidas apresentou significância estatística de acordo com a análise de Cox (razão de risco = 6,4; IC95% = 1,08-38,33; p = 0,04). Os grupos de risco foram ajustados por número de áreas anatômicas envolvidas (< quatro/> quatro áreas anatômicas envolvidas), com uma significativa realocação de pacientes (p = 0,008). Dos 30 pacientes com efeitos tardios, 21 estavam no grupo de risco desfavorável original, e 14 poderiam ter sido realocados para o grupo de risco favorável com base no número de áreas anatômicas envolvidas. CONCLUSÃO: Se uma reestratificação tivesse sido aplicada, um número considerável de crianças teria recebido tratamento de menor intensidade e, consequentemente, poderia ter tido menores chances de apresentar efeitos tardios. Um estudo prospectivo poderia definir se o ajuste de grupos de risco pelo número de áreas anatômicas envolvidas teria algum impacto sobre as taxas de sobrevida.OBJECTIVE: To determine if the number of involved anatomic areas can modify the standard risk groups in pediatric Hodgkin's lymphoma, identifying children who would benefit from a reduction in treatment intensity. METHODS: Retrospective study evaluating age, sex, histology, Ann-Arbor stage, presence of B symptoms, number of involved anatomic areas, risk grouping (favorable vs. unfavorable), and laboratory exams. All patients received doxorubicin-containing chemotherapy. Patients in complete remission for 5 years or longer were evaluated as for late effects. RESULTS: Sixty-nine patients (2-18 years) were included, 68% belonged to the unfavorable risk group. Overall survival and event-free survival were 94 and 87%, respectively. Late effects were screened in 46 cases. Advanced stage and > four involved anatomic areas had negative impact on event-free survival, while only the number of involved anatomic areas retained statistical significance when using Cox analysis (hazard ratio = 6.4, 95%CI = 1.08-38.33; p = 0.04). Risk groups were adjusted by number of involved anatomic areas (< four/> four involved anatomic areas), with a significant reallocation of patients (p = 0.008). Of the 30 patients with late effects, 21 were in the original unfavorable risk group and 14 (66.6%) could have been reallocated to the favorable risk group based on the number of involved anatomic areas. CONCLUSION: If re-stratification had been applied, a considerable number of children would have received less intensive treatment and, consequently, could have had lower chances of late effects. A prospective study could define if adjustment of risk group by number of involved anatomic areas would have any impact on survival rates.

OBJETIVO: Comparar o ganho pôndero-estatural e a frequência de complicações clínicas em recém-nascidos pré-termo com peso inferior a 1.500 g, alimentados exclusivamente com leite humano cru da própria mãe com e sem aditivo até atingirem o peso de 1.800 g. MÉTODOS: Ensaio clínico prospectivo randomizado duplo-cego em 40 recém-nascidos pré-termo com peso de nascimento < 1.500 g e ≤ 34 semanas, internados em unidade de terapia intensiva neonatal no período de agosto de 2005 a abril de 2007. Foram randomizados em 2 grupos: controle (leite humano puro) e intervenção (leite humano com aditivo). A fortificação foi feita no leite humano cru ordenhado no momento da oferta, quando a dieta atingiu 100 mL/kg/dia (até os neonatos atingirem peso de 1.800 g). Foram comparados ganho de peso diário, crescimento e perímetro cefálico semanalmente, variáveis nutricionais e complicações clínicas. RESULTADOS: A fortificação resultou em melhor crescimento, com ganho de 1,09 e 0,87 cm/semana (p = 0,003) e perímetro cefálico observado de 0,73 e 1,02 cm/semana (p = 0,0001), respectivamente grupo intervenção e controle. O ganho de peso foi de 24,4 e 21,2 g/dia (p = 0,075). Quanto às complicações clínicas observadas, não houve diferença significante. CONCLUSÕES: O uso de aditivo no leite humano cru da própria mãe proporcionou melhor crescimento, com aumento significativo do comprimento e do perímetro cefálico.OBJECTIVE: To compare the weight and height gain and the frequency of clinical complications in preterm newborns weighing less than 1,500 g, exclusively fed human milk or fortified human milk until reaching 1,800 g. METHODS: Prospective double-blind randomized controlled trial involving 40 preterm infants weighing < 1,500 g at birth and ≤ 34 weeks of gestational age, admitted to a neonatal intensive care unit from August 2005 to April 2007. Preterm infants were randomized into two groups: control (human milk) and intervention (fortified human milk). Fortifiers were added to manually expressed human milk when feeding volume reached 100 mL/kg/day until newborns reached 1,800 g. Daily weight gain, weekly length and head circumference gain, nutritional variables and clinical complications were compared. RESULTS: Human milk fortification resulted in better growth, with length gain of 1.09 and 0.87 cm/week (p = 0.003) and head circumference gain of 0.73 and 1.02 cm/week (p = 0.0001), respectively, for intervention and control groups. The weight gain was 24.4 and 21.1 g/day (p = 0.075). There were no significant clinical complications. CONCLUSIONS: Human milk fortification resulted in better growth, significant increase in length and head circumference.

A osteoporose, caracterizada por aumento da fragilidade óssea e suscetibilidade a fraturas, é inversamente proporcional ao pico de massa óssea adquirido na infância. Por outro lado, a doença óssea é uma importante causa de morbidade em pacientes portadores de beta-talassemia maior (TM). Apesar de intensamente descrita em pacientes talassêmicos adultos, não existem estudos sobre as alterações de densidade óssea em crianças talassêmicas brasileiras. Foram avaliados 11 pacientes (idade mediana de 10,0, variando de 5 a 12 anos), portadores de TM, e 24 crianças (idade mediana de 9,5, variando de 6 a 12 anos) saudáveis, utilizando medida de emissão dupla de raios-X para avaliar a densidade mineral óssea (DMO). A análise de marcadores bioquímicos tais como concentração de ferritina sérica, cálcio ionizado, fosfatase alcalina, fósforo, albumina, tempo de protrombina e fator V foi realizada. A estatura foi significativamente diferente entre os dois grupos estudados, pOsteoporosis is characterized by low bone mass and disruption of bone architecture, resulting in greater bone fragility with increased risk of fractures. Bone disease is an important cause of morbidity in beta thalassemia major patients. Osteoporosis has been described extensively in adult thalassemia. However, there are no studies describing Brazilian thalassemic children. We evaluated eleven patients with beta thalassemia major (median age of 10.0 years, range from 5 to 12 years) and twenty-four healthy children (median age of 9.5 years, range from 6 to 12 years), using dual X-ray absorptiometry to assess bone mineral density (BMD). Analysis of biochemical markers such as serum ferritin concentration, ionized calcium, alkaline phosphatase, phosphorus, albumin, prothrombin time and factor V was performed. The height was very different between the groups, p

OBJETIVO: Analisar o perfil bioquímico da água de coco de coqueiros-anões plantados em região não litorânea, no período de maturação (sexto ao nono mês). MÉTODOS: Selecionaram-se por sorteio oito de 15 coqueiros plantados em região não litorânea, e os cocos foram enviados ao laboratório para extração e análise da água de coco. Avaliou-se glicose, eletrólitos, proteínas totais, osmolaridade, e identificaram-se açúcares da água de coco, em um total de 45 cocos, do sexto ao nono mês de maturação. RESULTADOS: A análise da água de coco do sexto ao nono mês não demonstrou diferença da mediana da concentração do sódio (3 mEq/L; 2 e 3), glicose (0,6 g/L; 0,3 e 17,3) e proteínas totais (9 g/L; 6 e 12); houve redução na concentração de potássio (64 mEq/L; 46 e 99), cálcio (6,5 mmol/L; 5 e 8,5), magnésio (8 mmol/L; 3,9 e 9,8), cloro (38,5 mEq/L; 30 e 48,7) e osmolaridade (419 mOsmol/L; 354 e 472). Com relação aos açúcares identificados por cromatografia em papel, observou-se aumento da concentração de frutose (68 mg/µL; 44 e 320) e de glicose (299 mg/µL; 262 e 332) e redução na concentração de sacarose (340 mg/µL; 264 e 390) do sexto ao nono mês. CONCLUSÕES: O perfil bioquímico da água de coco variou durante a maturação do fruto, observando-se redução da concentração de potássio, cálcio, magnésio, cloro e osmolaridade. Segundo a cromatografia em papel descendente, observou-se aumento da concentração de frutose e de glicose, bem como redução da concentração de sacarose.OBJECTIVE: To analyze the biochemical profile of coconut water from dwarf coconut palms planted in non-coastal regions, during the maturation period (sixth to ninth month). METHODS: Eight of 15 coconut palms planted in a non-coastal region were selected by lots and their coconuts sent to a laboratory for extraction and analysis of the coconut water. Coconut water from a total of 45 coconuts, from the sixth to ninth months' maturity, were analyzed to measure glucose, electrolytes, total proteins and osmolarity and to identify the sugars contained. RESULTS: The analysis of coconut water from the sixth to ninth month did not find any differences in the median concentrations of sodium (3 mEq/L; 2 and 3), glucose (0.6 g/L; 0.3 and 17.3) or total proteins (9 g/L; 6 and 12), but detected a reduction in the concentration of potassium (64 mEq/L; 46 and 99), calcium (6.5 mmol/L; 5 and 8.5), magnesium (8 mmol/L; 3.9 and 9.8), chloride (38.5 mEq/L; 30 and 48.7) and osmolarity (419 mOsmol/L; 354 and 472). With relation to the sugars, identified by chromatography on paper, an increase was observed from the sixth to the ninth month in the concentration of fructose (68 mg/µL; 44 and 320) and glucose (299 mg/µL; 262 and 332) and in conjunction with a concentration of sucrose (340 mg/µL; 264 and 390). CONCLUSIONS: The biochemical profile of coconut water varied as the coconuts matured, observing reductions in the concentration of potassium, calcium, magnesium, chloride and osmolarity. Descending paper chromatography revealed an increase in the concentration of fructose and glucose and also a reduction in the concentration of sucrose.

Chronic lymphocytic leukemia is the most prevalent type of leukemia in the West. It is characterized by an extremely variable clinical course. The aim of the study was to detect the most frequent chromosomal abnormalities in patients with CLL using FISH, and assess them regarding age, gender, clinical stage and CD38 and ZAP-70 expressions. We found 51.7% of the patients with chromosome abnormalities. The most frequent one was del 13q14 in 34.5% of cases. It was associated to other alterations in 17.2%. 17p13 deletions were found in 17.2% and trisomy 12 in 13.8% (in isolation in 6.9% and associated to del 13q14, in 6.9% of the cases). An 11q22 deletion was found in one case associated to a 13q14 deletion. To better evaluate the relationship between chromosome aberrations and other prognostic factors in CLL, two cytogenetics groups were considered: favorable (13q deletion in isolation and no alteration) and unfavorable outcomes (trisomy 12, 17p13 deletion, 11q22 deletion and two simultaneous alterations).The unfavorable alterations were more frequently seen among young individuals (A leucemia linfocítica crônica (LLC) é o tipo de leucemia mais prevalente no Ocidente e é caracterizada por curso clínico extremamente variável. O objetivo deste estudo foi detectar as anomalias cromossômicas mais freqüentes em pacientes com LLC, empregando a técnica FISH, e correlacioná-las com idade, sexo, estádio clínico, expressão de CD 38 e ZAP-70. Foram encontradas alterações cromossômicas em 51,7% dos pacientes. A mais freqüente foi a del 13q14, observada em 34,5% dos casos e que esteve associada a outras anomalias em 17,2%. Deleção 17p13 foi encontrada em 17,2% e trissomia 12 em 13,8% (isolada em 6,9% e associada à del 13q14 em 6,9%). Deleção 11q22 foi observada em um caso em concomitância à del 13q14. Para melhor avaliar a relação entre alteração cromossômica e outros fatores prognósticos em LLC, dois grupos citogenéticos foram considerados: favorável (deleção 13q isolada e ausência de alterações) e desfavorável (trissomia 12, deleção 17p13, deleção 11q22 e duas anomalias simultâneas). As alterações desfavoráveis foram mais freqüentemente observadas em indivíduos jovens (

A leucemia linfocítica crônica (LLC) é uma doença linfoproliferativa crônica (DLPC) que apresenta características epidemiológicas peculiares; acomete indivíduos com idade mais avançada (não ocorre em crianças e é rara abaixo dos 30 anos) e a sua incidência varia conforme a origem étnica dos pacientes. É a leucemia mais freqüente nos países ocidentais enquanto muito rara nos orientais. A sua etiologia não está ainda esclarecida, não se conhecendo fatores ambientais que mostrem forte associação com o surgimento da doença. Não tem relação com radiação ionizante (sobreviventes da bomba atômica não apresentaram aumento na incidência da LLC), assim como não foi demonstrada associação com agentes tóxicos ou virais específicos. A LLC familiar é assim denominada quando, pelo menos, dois membros de uma família apresentam LLC e o diagnóstico nestes pacientes costuma ocorrer em idade mais precoce. A ocorrência é maior em parentes de primeiro grau (irmãos, filhos), podendo, porém, afetar parentes mais distantes. Os familiares de pacientes com LLC apresentam maior freqüência de outras DLPC (risco relativo 30x maior) e de proliferação monoclonal de linfócitos B (13%-18%) e parece que o HLADR1.11 está implicado na LLC familiar, pelo menos em algumas populações.Chronic lymphocytic leukemia (CLL) is a chronic lympho-proliferative desorder (CLPD) with peculiar epidemiologic characteristics. It is a disease of the elderly, which is very rare in under 30-year-old individuals and absent among children. Its incidence largely varies according to the ethnical origin: CLL is the most common leukemia in Western countries while it is rarely seen in Eastern countries. The etiology of CLL is still unknown. Environmental factors such as exposure to ionizing radiation (atomic bomb survivors did not show an increased incidence of CLL) or toxic or viral agents are not associated to the occurrence of CLL. Familiar CLL is characterized when at least two members of the same family develop CLL The diagnosis of familiar CLL seems to be made 10 to 20 years earlier than sporadic cases. CLL occurs more frequently among siblings and offspring although it may appear in other members such as cousins. The frequency of other CLPD (relative risk is 30 times greater) and monoclonal CD5+B cells proliferations (13.5%-18%) are higher among "healthy" members of familial CLL and HLADR1/11 is implicated in at least some populations with familial CLL.

HISTÓRIA E OBJETIVOS: A indicação de transfusão sangüinea em pacientes criticamente enfermos é complexa e pode ser influenciada por muitos fatores. Embora seja prática freqüente, poucos estudos são disponibilizados sobre o uso de sangue e hemocomponentes em pacientes sob cuidados intensivos. O objetivo deste trabalho é analisar a taxa e a incidência-densidade de transfusão de concentrado de hemácias (CH) na Unidade de Terapia Intensiva (UTI) do Hospital Universitário Prof. Polydoro Ernani de São Thiago (HU/UFSC), bem como descrever os critérios clínicos utilizados e os valores de hemoglobina pré-transfusionais. MÉTODOS: Foram selecionados os pacientes admitidos na UTI do HU/UFSC entre janeiro de 2003 e dezembro de 2004. RESULTADOS: Dos 654 pacientes analisados, 108 (16,51%) receberam transfusão de CH, com incidência-densidade de 3,80 por 100 pacientes-dia. O CH foi o hemocomponente mais utilizado, com um total de 257 eventos transfusionais e 549 unidades. Baixo nível de hemoglobina foi o critério mais utilizado (58%). Valores de hemoglobina pré-transfusional inferiores a 7,0 g/dL foram verificados em 51,75% das transfusões. INTERPRETAÇÃO E CONCLUSÕES: Nos últimos 15 anos, diversos autores analisaram taxas de transfusões de CH em UTIs, encontrando percentuais entre 19% e 85%. Atualmente, em pacientes criticamente enfermos, recomendam-se transfusões quando os níveis de hemoglobina são inferiores a 7,0 g/dL e evitam-se quando são superiores a 10,0 g/dL. Na UTI do HU/UFSC, o hemocomponente mais utilizado é o CH, com percentuais inferiores aos de UTIs de outras instituições. Baixa concentração de hemoglobina com valor pré-transfusional inferior a 7,0 g/dL é o principal critério de indicação.HISTORY AND OBJECTIVES: Many factors influence the indication of blood transfusion for critical ill patients. Nevertheless there are few studies concerning the transfusion of blood components in patients under intensive care. This study is aimed at analyzing the rate and the transfusion incidence-density of red blood cells (RBC) in an Intensive Care Unit (ICU) of the University Hospital of Federal University of Santa Catarina and to describe the clinical criteria and the haemoglobin values applied to indicate transfusions. METHOD: For this study, patients admitted into the ICU between January 2003 and December 2004 were included. RESULTS: A total of 654 patients were studied. From those 108 (16.51%) received RBC transfusions with an incidence-density of 3.8 per 100 patients day. RBC was the most commonly used blood component (257 transfusions were performed using 549 RBC units). The criterion most frequently used for RBC transfusion was a low haemoglobin level (58%). Pre-transfusion haemoglobin levels lower than 7.0 g/dL were observed in 51.75% patients. ANALYSIS AND CONCLUSIONS: Over the last 15 years many authors found that RBC transfusion percentages in the ICU were between 19 and 85%. Currently, RBC transfusion is indicated for haemoglobin levels of less than 7.0 g/dL and transfusions should be avoided when the level is higher than 10.0 g/dL. In the ICU of the University Hospital, RBC is the most commonly used blood component with a lower percentage when compared to ICUs of other institutions. Low haemoglobin levels with pre-transfusion values less than 7.0 g/dL is the main criterion to indicate this conduct.

OBJETIVO: Analisar alguns fatores relacionados a injúrias que resultaram em hospitalizações de crianças e adolescentes. MÉTODOS: Pesquisa prospectiva, descritiva e observacional realizada no Hospital Márcio Cunha, em Ipatinga, Minas Gerais, no período de 1º de dezembro de 1999 a 30 de novembro de 2000. A amostra incluiu 696 crianças e adolescentes, na faixa etária de 0 a 19 anos, hospitalizados por injúrias. Para classificar os tipos e os locais de ocorrência das injúrias utilizou-se a Classificação Estatística Internacional de Doenças e Problemas Relacionados à Saúde, 10ª revisão. RESULTADOS: As injúrias representaram 9,9% das hospitalizações de crianças e adolescentes; 69,7% dos pacientes eram do gênero masculino. Quanto à opinião dos informantes sobre as causas das injúrias, 57,2% citaram falhas humanas, 18% acreditavam que se deviam ao destino, e 12,7% não sabiam opinar. Somente 23,1% dos informantes haviam recebido, anteriormente, orientação sobre a prevenção de injúrias, e as principais fontes foram mídia, escola e empresa. Do total de eventos, 31,9% ocorreram nas residências. Acidentes de transporte causaram 34,5% das internações; quedas, 33,2%; exposição a forças mecânicas inanimadas, 9%; contato com animais venenosos, 5,2%; agressões, 5%; queimaduras, 4,9%; exposição a forças mecânicas animadas, 3,3%; intoxicações, 2,3%; e outros tipos, 2,6%. CONCLUSÕES: Houve predomínio do gênero masculino. Foi baixo o percentual de informantes que haviam recebido, anteriormente, orientação sobre prevenção de injúrias. A faixa etária mais acometida foi a de 15 a 19 anos. Os acidentes de transporte e as quedas foram os mais freqüentes.OBJECTIVE: To analyze some factors related to injuries and children and adolescents hospitalization. METHODS: This is a prospective, descriptive and observational research that took place at Márcio Cunha Hospital, Ipatinga, Minas Gerais, in the period of December 1st, 1999 to November 30th, 2000. The sample included 696 children and adolescents hospitalized for injuries, ages ranging from 0 to 19 years old. The International Statistical Classification of Diseases and Related Health Problems (10th version) was used as a parameter to classify the types of injuries and sites where they happened. RESULTS: The injuries represented 9.9% of children and adolescents' hospitalizations, of which, 69.7% were male patients. As for the informants' opinion about causes of injuries, 57.2% attributed then to human failure, 18% believed they were due to fate and 12.7% did not know how to express their opinions. Only 23.1% of the informants had received previous orientation about prevention and the main sources were media, school and work place. A total of 31.9% of the events took place at home. Transportation injuries caused 34.5% of hospitalizations; falls, 33.2%; exposure to inanimate mechanical forces, 9%; contact with poisonous animals, 5.2%; aggression, 5%; burns, 4.9%; exposure to animated mechanical forces, 3.3%; intoxication, 2.3%; and other types, 2.6%. CONCLUSIONS: Males were more affected than females. Only a low percentage of the informants had received previous orientation about injury prevention. The most affected age group was between 15 and 19 years. Transportation accidents and falls were the most frequent causes of injuries.

OBJETIVO: Avaliar a ingestão alimentar e o estado nutricional de crianças em dieta isenta de leite de vaca e derivados. MÉTODOS: Foram avaliadas 26 crianças, na primeira consulta realizada em ambulatório de gastroenterologia pediátrica, que vinham recebendo dieta isenta de leite de vaca e derivados (média de idade = 19,1 meses) e 30 crianças com dieta normal, isto é, sem nenhum tipo de restrição alimentar (média de idade = 16,8 meses). Empregou-se o método do dia alimentar habitual para a obtenção dos dados sobre consumo alimentar. A ingestão alimentar foi comparada entre os grupos e em relação às Dietary Reference Intakes (DRIs). O estado nutricional foi avaliado com base nos escores z de peso/idade, estatura/idade e peso/estatura. RESULTADOS: O grupo em dieta isenta de leite de vaca apresentou menor ingestão de energia (p = 0,005), proteínas (p < 0,001), lipídios (p < 0,001), cálcio (p < 0,001) e fósforo (p < 0,001) quando comparado ao grupo controle. Houve maior número de crianças no grupo em dieta isenta de leite de vaca com ingestão de energia, cálcio e fósforo inferior às DRIs em comparação ao grupo controle. As médias dos escores z dos grupos com dieta isenta de leite de vaca e controle, foram, respectivamente: estatura/idade -0,81±1,06 versus +0,42 ±1,25 (p < 0,001), peso/idade -1,03±1,21 versus +0,02 ±0,91 (p < 0,001) e peso/estatura -0,63 ±1,08 versus +0,30 ±1,11 (p = 0,004). CONCLUSÕES: Durante a terapêutica de exclusão do leite de vaca e seus derivados, deve ser realizado, periodicamente, monitoramento da ingestão alimentar qualitativa e quantitativamente, a fim de se prevenir prováveis inadequações no atendimento às necessidades nutricionais, bem como prejuízo ao crescimento e desenvolvimento dessas crianças.OBJECTIVE: To assess the food intake and the nutritional status of children on a cow's milk and cow's milk by-products free diet. METHODS: Twenty-six children receiving a cow's milk and cow's milk by-products free diet were assessed during their first visit to the Pediatric Gastroenterology Clinic (mean age = 19.1 months). Thirty children with no food restriction (mean age = 16.8 months) were also assessed. The usual daily food intake method was used to make the dietary assessment. The food intake was compared between the groups and in relation to the Dietary Reference Intakes (DRIs). The z-scores for weight/age, height/age and weight/height were used to evaluate the nutritional status. RESULTS: The cow's milk free diet group presented lower energy (p = 0.005), protein (p < 0.001), lipid (p < 0.001), calcium (p < 0.001) and phosphorous (p < 0.001) intake when compared to the control group. The number of children who had energy, calcium and phosphorous intake below the DRIs was higher in the cow's milk free diet group than in the control group. The z-score means for the cow's milk free diet and control groups were, respectively: height/age -0.81±1.06 vs +0.42±1.25 (p < 0.001), weight/age -1.03±1.21 vs +0.02+0.91 (p < 0.001), and weight/height -0.63+1.08 vs +0.30+1.11 (p = 0.004). CONCLUSIONS: During the cow's milk and cow's milk by-products exclusion therapy, qualitative and quantitative food intake monitoring must be carried out periodically, so as to prevent inadequacies in meeting nutritional requirements and impairment of growth and development.

OBJETIVO: Revisar as características das injúrias físicas não intencionais e seu impacto sobre a criança e o adolescente. FONTES DOS DADOS: Foram selecionados artigos publicados principalmente entre 2000 e 2005 nas bases de dados MEDLINE, EBSCO, Proquest, SciELO, BVS e Google Scholar. Utilizaram-se as seguintes palavras-chave: injúrias, injúrias não intencionais, casa segura, queimaduras, quedas, afogamentos, escorpionismo, ofidismo, intoxicação, criança, adolescente, mortalidade, controle de injúrias e internações. Alguns artigos foram avaliados a partir de referência de publicações pesquisadas. SÍNTESE DOS DADOS: São contextualizadas as injúrias não intencionais no mundo e no Brasil, bem como identificadas as condutas atualmente adotadas para sua prevenção e controle. São avaliados os impactos sobre a mortalidade, sobre o conjunto de danos físicos e o impacto econômico das injúrias. É enfatizado o enfoque sobre o ambiente doméstico, com a abordagem evolutiva da criança, as disparidades sociais e a contextualização do ambiente doméstico sobre o mundo infantil e as vulnerabilidades. São identificados os principais tipos de eventos que causam dano físico à criança e ao adolescente, especialmente no âmbito domiciliar. CONCLUSÃO: A prevenção de injúrias no ambiente doméstico é uma realidade possível, determinando desafios aos profissionais da área de saúde para a redução do impacto das lesões não intencionais sobre a morbimortalidade de crianças e jovens do Brasil e do mundo.OBJECTIVE: To review the characteristics of unintentional injuries and their impact on children and adolescents. SOURCES OF DATA: Articles published between 2000 and 2005 in the MEDLINE, EBSCO, Proquest, SciELO, BVS and Google Scholar databases were selected. The authors used the keywords unintentional injuries, injuries, safe home, burns, falls, drowning, scorpions, snakes, poisoning, child, adolescent, mortality, injury control, and hospitalization. Some articles were evaluated based on the selected publications. SUMMARY OF THE FINDINGS: Unintentional injuries in the world and in Brazil are analyzed, and so are the behaviors currently adopted for injury prevention and control. The impact on mortality, on physical damage, and the economic burden of injuries are evaluated. Special emphasis is placed on home environment, approaching the effects of child development, social disparities and contextualization of home environment on children's world and vulnerabilities. The main types of events that cause physical damage to the child and adolescent in the home environment are described. CONCLUSION: The prevention of injuries in the home environment is possible. In this case, health professionals have the challenge to reduce the consequences of unintentional injuries on the morbidity and mortality of children and young people in Brazil and in the whole world.