A noninvasive index of atrial remodeling in patients with paroxysmal and persistent atrial fibrillation: a pilot study.

PURPOSE: This study aims to develop a noninvasive atrial remodeling index (RI) to separate patients presenting paroxysmal atrial fibrillation (ParAF) from those with sustained persistent atrial fibrillation (PerAF), that is, AF episodes interrupted 7 days or more after the onset. METHODS: Signal-averaged P-wave duration (SAPWd) and left atrial anteroposterior diameter (LADd) were measured in 33 ParAF patients, in 26 sustained PerAF patients, and in 18 control subjects. By using SAPWd and LADd, a dichotomous (0/1) RI was created. A logistic regression model on the probability of having a sustained PerAF vs a ParAF episode was estimated, including the RI, sex, age, and cardiac comorbidities as covariates. RESULTS: Signal-averaged P-wave duration was significantly longer in sustained PerAF (153 ± 15 milliseconds) than in ParAF patients (142 ± 13 milliseconds, P < .001) and in both ParAF and sustained PerAF groups vs control group (123 ± 7 milliseconds, P < .001). Left atrial anteroposterior diameter was larger both in sustained PerAF (43 ± 6 mm) vs ParAF patients (38 ± 5 mm, P = .002) and in sustained PerAF group vs control group (38 ± 2 mm, P = .004), but no differences were observed between ParAF patients and controls (P = .6). A 12-fold increase (odds ratio, 11.8; 95% confidence interval, 2.2-63.5) in the odds of having a sustained PerAF vs a ParAF episode was observed in patients with RI equal to 1. CONCLUSIONS: P-wave duration and left atrium diameter enabled to define a noninvasive atrial RI to separate patients with ParAF from those with sustained PerAF. This could be a useful tool to select a suitable strategy for AF treatment.

Evaluation of quality of the children's menu in mall's restaurants.

OBJECTIVE: To evaluate the quality of children's menus in restaurants located in shopping malls. METHODS: To select the sample, restaurants from 30% of shopping malls in each region of the city of São Paulo were included and, after considering only one restaurant per chain, the total was limited to 151 restaurants, 30.2% of which (n=35) presented a children's menu. Data were collected through a form on Google Forms. RESULTS: Of the restaurants with children's menu, 60% (n=21) were conventional restaurants and 40% (n=14) takeaway/fast-food. The large number of chains present in most visited malls showed a democratization of the way of eating, with popular and accessible menus, regardless of social status. Most of the analyzed foods were cooked (41.5%). Most preparations did not use grease in their preparation and there was a notable lack of fruit and vegetables (FV) (4%). Sweet desserts were offered in 11.4% of the places and 20% included gifts with meals. CONCLUSIONS: The scarce offer of children's menus, few options and low FV offer indicate the need for a new look at the development of children's menus and a greater integration between the possibilities of restaurants and the expectations of parents and children, in the challenge of integrating the relationship between the supply of new foods that promote healthier habits and their consumption.

Evaluation of quality of the children's menu in mall's restaurants / Avaliação da qualidade do menu infantil em restaurantes de shopping centers

Abstract Objective: To evaluate the quality of children's menus in restaurants located in shopping malls. Methods: To select the sample, restaurants from 30% of shopping malls in each region of the city of São Paulo were included and, after considering only one restaurant per chain, the total was limited to 151 restaurants, 30.2% of which (n=35) presented a children's menu. Data were collected through a form on Google Forms. Results: Of the restaurants with children's menu, 60% (n=21) were conventional restaurants and 40% (n=14) takeaway/fast-food. The large number of chains present in most visited malls showed a democratization of the way of eating, with popular and accessible menus, regardless of social status. Most of the analyzed foods were cooked (41.5%). Most preparations did not use grease in their preparation and there was a notable lack of fruit and vegetables (FV) (4%). Sweet desserts were offered in 11.4% of the places and 20% included gifts with meals. Conclusions: The scarce offer of children's menus, few options and low FV offer indicate the need for a new look at the development of children's menus and a greater integration between the possibilities of restaurants and the expectations of parents and children, in the challenge of integrating the relationship between the supply of new foods that promote healthier habits and their consumption.

Resumo Objetivo: Avaliar a qualidade dos menus infantis de restaurantes localizados em shopping centers. Métodos: Para a seleção da amostra foram incluídos os restaurantes de 30% dos shopping centers de cada região da cidade de São Paulo e, após considerar-se somente um restaurante por rede, o total ficou limitado a 151 restaurantes, dos quais 30,2% (n=35) apresentaram cardápio infantil. Os dados foram coletados por meio de um formulário no Google Forms. Resultados: Dos restaurantes com menu infantil, 60% (n=21) eram convencionais e 40% (n=14) eram de takeaway/fast-food. O grande número de redes presente na maioria dos shopping centers visitados mostrou a democratização da forma de comer, com cardápios populares e acessíveis, independentemente de classe social. A maior parte dos alimentos analisados era cozida (41,5%). A maioria das preparações não utilizava gordura em seu preparo e foi notável a falta da oferta de frutas, legumes e verduras (FLV) (4%). Sobremesas doces eram oferecidas em 11,4% dos locais e 20% incluíam brindes com as refeições. Conclusões: A escassa oferta de menus infantis, as poucas opções e a baixa oferta de FLV indicam a necessidade de um novo olhar no desenvolvimento de cardápios infantis e de um maior entrosamento entre as possibilidades dos restaurantes e as expectativas de pais e crianças, no desafio de integrar a relação entre a oferta de novos alimentos, que promovam hábitos mais saudáveis, e o seu consumo.

Lung ultrasound for monitoring cardiogenic pulmonary edema.

Several studies address the accuracy of lung ultrasound (LUS) in the diagnosis of cardiogenic pulmonary edema (CPE) evaluating the interstitial syndrome, which is characterized by multiple and diffuse vertical artifacts (B-lines), and correlates with extravascular lung water. We studied the potential role of LUS in monitoring CPE response to therapy, by evaluating the clearance of interstitial syndrome within the first 24 h after Emergency Department (ED) admission. LUS was performed at arrival (T0), after 3 (T3) and 24 (T24) hours. Eleven regions were evaluated in the antero-lateral chest; the B-lines burden was estimated in each region (0 = no B-lines, 1 = multiple B-lines, 2 = confluent B-lines/white lung) and a mean score (B-Score, range 0-2) was calculated. Patients received conventional CPE treatment. Blood chemistry, vital signs, blood gas analysis, diuresis at T0, T3, T24 were also recorded. A complete echocardiography was obtained during hospitalization. Forty-one patients were enrolled. Respiratory and hemodynamic parameters improved in all patients between T0 and T3 and between T3 and T24. Mean B-score significantly decreased at T3 (from 1.59 ± 0.40 to 0.73 ± 0.44, P < 0.001) and between T3 and T 24 (from 0.73 ± 0.44 to 0.38 ± 0.33, P < 0.001). B-score was higher in the lower pulmonary regions at any time. At final evaluation (T24) 75 % of apical and only 38 % of basal regions were cleared. LUS allows one to assess the clearance of interstitial syndrome and its distribution in the early hours of treatment of CPE, thus representing a possible tool to guide therapy titration.

Chemokine receptor expression and function in childhood acute lymphoblastic leukemia of B-lineage.

Scanty information is available on chemokine receptor expression and function in childhood B-lineage acute lymphoblastic leukemia (ALL). Thirteen pro-B, 17 early pre-B, 12 pre-B, and 9 B-ALL/Burkitt lymphoma (BL) pediatric cases were tested for CXCR1 to CXCR5 and CCR1 to CCR7 expression. CXCR2, CXCR3, and CXCR4 were expressed in the majority of cases, while the other receptors were variably expressed or absent. CXCR4 mediated chemotaxis of all leukemic cell subtypes. Freshly isolated CCR7(+) early pre-B-ALL cells migrated to CCL19, whereas CCR7(+) pro-B- and pre-B-ALL cells were attracted by CCL19 only following culture with soluble recombinant CD40 ligand.

Two consecutive immunophenotypic switches in a child with MLL-rearranged acute lymphoblastic leukemia.

An 18-month-old girl was diagnosed with pre-pre-B ALL/t(4;11) leukemia, which during the treatment and after matched bone marrow transplantation (BMT), underwent two consecutive switches from lymphoid to myeloid lineage and vice versa. The high expression of HOXA9 and FLT3 genes remaining genotypically stable in a leukemia throughout phenotypic switches, suggests that this leukemia may have originated as a common B/myeloid progenitors.

Avaliação da composição de cardápios de escolas públicas do município de São Paulo utilizando o índice de qualidade da Coordenação de Segurança Alimentar e Nutricional e o método de análise qualitativa das preparações do cardápio / Evaluation of the composition of public-school menu in the municipality of São Paulo using the quality index of the Food and Nutrition Security Coordination and the method of qualitative analysis of menu preparations

Introdução: Na infância, a escola tem importante impacto na formação de hábitos alimentares. A alimentação qualitativa e quantitativamente adequada é essencial para garantir o crescimento e o desenvolvimento e, nesse cenário, o Programa Nacional de Alimentação Escolar tem por objetivo realizar ações de educação alimentar e nutricional e oferecer refeições que garantam as necessidades nutricionais durante o período letivo. Objetivo: Avaliar quanti-qualitativamente a composição dos cardápios do Programa de Alimentação Escolar da rede municipal de São Paulo. Métodos: A coleta de dados foi realizada nos meses de agosto a outubro de 2019, por meio dos cardápios disponibilizados na plataforma eletrônica "Prato Aberto", publicada sob responsabilidade da Secretaria Municipal de Educação de São Paulo nos meses de agosto a outubro de 2019, para as Escolas Municipais de Ensino Fundamental. Os cardápios foram avaliados por sorteio das escolas de acordo com sua localização. Resultados: A média de pontuação total demonstra que todos os cardápios avaliados foram classificados como adequados; entretanto, ao analisar detalhadamente os resultados mensais para cada escola, foi possível notar o impacto das diferentes formas de gestão, compras de alimentos e planejamento dos cardápios. Conclusão: Os cardápios foram avaliados positivamente frente às duas ferramentas e estavam adequados quanto às recomendações do PNAE, embora houvesse pontos a serem melhorados, sobretudo quanto à alta previsão de oferta de alimentos restritos. (AU)

Introduction: In childhood, school has an important impact on the formation of eating habits. Qualitatively and quantitatively adequate food is essential to ensure growth and development and, in this scenario, the National School Feeding Program aims to carry out food and nutrition education actions and offer meals that guarantee nutritional needs during the school term. Objective: To quantitatively and qualitatively evaluate the composition of the menus in the School Meals Program of the municipal network of São Paulo. Methods: Data collection was carried out from August to October 2019, using menus available on the electronic platform "Prato Aberto", published under the responsibility of the Municipal Education Department of São Paulo from August to October 2019, for Municipal Elementary Schools. The menus were evaluated by drawing lots of schools according to their location. Results: The average total score demonstrates that all evaluated menus were classified as adequate; however, when analyzing in detail the monthly results for each school, it was possible to notice the impact of different forms of management, food purchases and menu planning. Conclusion: The menus were positively evaluated against the two tools and were adequate in terms of the PNAE recommendations, although there were points to be improved, especially regarding the high forecast of restricted food supply. (AU)

Percepção de pais e responsáveis por crianças diagnosticadas com câncer: a gastronomia hospitalar em foco / Perception of parents and caregivers of children diagnosed with cancer: the hospital gastronomy in focus

INTRODUÇÃO: Crianças com câncer apresentam dificuldades de aceitação alimentar no ambiente hospitalar, que podem impactar na recuperação e amenização de efeitos coletarais decorrentes do tratamento. A gastronomia hospitalar aparece como um diferencial ao agregar dietoterapia e comfort food. OBJETIVOS: Avaliar a percepção de pais e responsáveis por crianças diagnosticadas com câncer quanto à alimentação oferecida no ambiente hospitalar no que se refere à gastronomia hospitalar. MÉTODOS: Estudo transversal realizado em 2018 com aplicação de questionário presencial e online para responsáveis por crianças que receberam tratamento oncológico e se alimentaram no ambiente hospitalar, sendo perguntados: tipo de câncer; sintomas observados; sabor, temperatura, quantidade servida, apresentação e aparência da refeição servida; aceitação alimentar durante e após internação; adequação da dieta às preferências alimentares e sintomatologia. RESULTADOS: Responderam ao questionário 17 familiares. A média de idade das crianças foi de 5,3 ± 2,7 anos, 52,6% em tratamento oncológico, sendo a leucemia o câncer mais prevalente (57,9%). Os principais sintomas foram vômitos, náuseas e anorexia (73%), alteração do paladar (63,2%) e xerostomia (52,6%). Não foi relatada adaptação da apresentação para o público infantil ou uso de ervas aromáticas. A alimentação oferecida no ambiente hospitalar influenciou na exclusão de certos alimentos por 36,9% dos participantes, como também a inclusão de alimentos não habituais e nunca provados por 36,9% e 47,1%, respectivamente. A recusa alimentar também foi observada em 52,9% das crianças. Apenas 20% dos hospitais incluíram preparações regionais no cardápio. CONCLUSÕES: A gastronomia hospitalar não foi observada na amostra, entretanto trata-se de um conceito útil para alinhar as expectativas de todos os envolvidos na terapêutica e o despertar do interesse pelo momento da refeição para favorecer a adesão ao tratamento nutricional e evitar impactos decorrentes da terapia oncológica

INTRODUCTION: Children with cancer have difficulties accepting food at the hospital environment, which may impact the recovery and softening of colateral effects as a result from the treatment. The gastronomy appears as a differential when adding dietotherapy and comfort food. OBJECTIVES: To evaluate the perception of parents and guardians of children diagnosed with cancer regarding the diet offered in the hospital environment related to hospital gastronomy. METHODS: Cross-sectional study carried out in 2018 with application of a face-to-face and online questionnaire for those responsible for the children who received cancer treatment and fed in the hospital environment. The questions were: type of cancer; symptoms observed; flavor, temperature, quantity presentation and appearance of the meal served; food acceptance during and after hospitalization; adequacy of diet to dietary preferences and symptomatology. RESULTS: Seventeen family members participated. The average age of the children was 5.3 ± 2.7 years, 52.6% in cancer treatment, with leukemia being the most prevalent cancer (57.9%). The main symptoms were vomiting, nausea and anorexia (73%), taste change (63,2%) and xerostomia (52.6%). It has not been reported food presentation adaptation of aromatic herbs. The power supply offered in the hospital environment (The hospital food offered) influenced the exclusion of certain foods by 36.9% of participants, as well as the inclusion of unusual foods and never tasted for 36.9% and 47.1%, respectively. Food refusal was also observed in 52.9% of children. Only 20% of hospitals included regional preparations in menu. CONCLUSIONS: Hospital gastronomy was not observed in the sample, however, it is a useful concept to align the expectations of all those involved in the therapy and the interest in the moment of the meal to adherence to nutritional treatment and to avoid impacts arising from oncological therapy

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Imatinib mesylate (STI571) interference with growth of neuroectodermal tumour cell lines does not critically involve c-Kit inhibition.

A therapeutic role of STI571 (imatinib mesylate) has been anticipated in patients with c-Kit positive neuroectodermal tumors. We examined the efficacy of STI571 to inhibit expansion of c-Kit positive neuroectodermal tumor cell lines in vitro and in a mouse model inoculated with ES (Ewing sarcoma) derived tumor cells, and investigated the molecular mechanism of STI571 action. Eleven tumor lines of ES, PNET (primitive neuroectodermal tumors) and NB (neuroblastoma) were assayed in the presence of 1, 5, 10, 15, 20 or 30 micro M STI571 for 24, 48, 72 h and 7 days. The mechanism of STI571 action was investigated using a microphysiometer cytosensor that determines cellular metabolic rates in the presence of STI571. c-Kit and global protein phosphorylation was assayed by immunoprecipitation and a direct enzyme-linked immunoadsorbent assay after 72 h of 10 micro M STI571. Apoptosis was investigated by propidium iodide (PI), Annexin V staining and by enzymatic activity of caspase-3. Moreover, apoptotic gene expression was investigated using microarray technology. In nude mice, tumor volume and histology were analyzed in STI571 treated and untreated mice, and apoptotic gene expression analysis was performed on tumor masses. A decrease in cell proliferation and increase of cell apoptosis was caused by STI571 in a concentration- and time-dependent manner. Cytosensor microphysiometer and immunoprecipitation experiments demonstrated a time- and concentration-dependent decrease of cellular metabolic activity and global protein dephosphorylation after STI571 exposure. The inhibition by STI571 appeared at least to some extent independent of c-Kit inhibition since cells remained sensitive to SCF stimulation. Tumor volume was significantly reduced in STI571-treated mice compared to tumors from control inoculated non-treated mice. The apoptosis pathway in response to STI571 appeared not to be dependent on caspase activation, while gene expression profiles suggested accumulation of reactive oxygen species (ROS) resulting in cell death after exposure to STI571. The results point to the potential relevance of STI157 for neuroectodermal tumor therapy in view of its inhibitory effect on tumor cell growth, in spite of the observation that the inhibition of the c-Kit signaling pathway is not critically involved.

Synthesis, cytotoxicity, and apoptosis induction in human tumor cells by geiparvarin analogues.

A series of geiparvarin analogues modified on the unsaturated alkenyloxy bridge, where a H-atom replaced the 3'-Me group, were synthesized and evaluated against a panel of human tumor cell lines in vitro. These compounds demonstrated a stronger increase in growth inhibitory activity when compared to the parent compound geiparvarin (8). In particular, the activity increased even further in the series of demethylated compounds when a Me substituent in the coumarin moiety is introduced. On the contrary, the same modifications exerted on the parent compound led to an activity reduction. Interestingly, the new derivatives proved to be fully inhibitory to drug-resistant cell lines, thus suggesting that they are not subject to the pump-mediating efflux of antitumor drugs. On the basis of their cytotoxic profiles, the most-active compounds were selected for further biological evaluation. The extracellular acidification rate by the new geiparvarin analogues was measured with the Cytosensor microphysiometer. The new derivatives significantly increased the acidification rate during the 24-48 h of incubation in a concentration-dependent manner. Cell-cycle analysis, evaluated by flow cytometry, revealed a strong apoptotic induction by these compounds confirmed by DNA laddering and observation by electron microscopy. Interestingly, the apoptotic pathway did not appear to be mediated by the activation of caspase-3.

[Reliability and validity of palmar and conjunctival pallor for anemia detection purposes]. / Confiabilidade e validade da palidez palmar e de conjuntivas como triagem de anemia.

OBJECTIVE: To evaluate the reliability and the validity of the use of simple clinical signs as a method of anemia detection. METHODS: The study was carried out in a S o Paulo, Brazil, day-care center, and included 135 children from ages 3 months-6 years. Hemoglobin level results and palmar and conjunctival pallor assessment were used. Children with Hb under 11g/dl were considered as anemic; subjective criteria were used for the assessment of palmar and conjunctival pallor. Kappa statistics were used in order to verify agreement, and, in order to evaluate the technique's validity, sensitivity and specificity levels were calculated. RESULTS: The results show low levels of agreement. There was a greater level of sensitivity to conjunctival pallor than to palmar pallor. Specificity results may be considered as good. CONCLUSIONS: It is still early to recommend the routine use of this technique. However, it could promote substantial savings if perfected.

Avaliação de rótulos de alimentos destinados ao público infantil de acordo com as regulamentações da legislação brasileira / Evaluate of food labels for young public in according to regulations of brazilian legislation

Objetivo ­ Verificar a conformidade da rotulagem de 18 categorias de alimentos destinados ao público infantil comercializados na cidade de São Paulo. Métodos ­ Foram analisados dados qualitativos e quantitativos dos rótulos de acordo com as regulamentações da legislação brasileira vigente. Resultados ­ Dos 60 rótulos analisados, 90% apresentaram pelo menos um tipo de não conformidade frente à legislação e apenas 10% estavam em total acordo. Todas as categorias avaliadas apresentaram um tipo de irregularidade. Os itens que apresentaram maiores percentuais de não conformidades foram frases não previstas em regulamentos técnicos e figuras, símbolos e ilustrações e/ou desenhos, representando 85% e 63,3%, respectivamente. Não foram encontradas irregularidades para tamanho de letra, rótulo (existência e registro), denominação de acordo com o PIC (Padrão de Identidade e Qualidade), uso de aditivos, modo de preparo, dados do fabricante, origem, alimentos de primeira infância, expressão "contém fenilalanina" ou "não contém fenilalanina", painel principal, data de validade, corantes artificiais. Conclusão ­ O estudo indica que há a necessidade de um maior controle para que os rótulos tenham informações fidedignas, para que se tenha o cumprimento das exigências legais e para não induzir os consumidores ao erro.

Objective ­ To verify the compliance of the labeling of 18 categories of foods intended to children sold in São Paulo. Methods ­ Data were analized qualitative and quantitative in accordance with the regulations of the current brazilian legislation. Results ­ Analyzed 60 labels, 90% had at least one type of non-compliance against the law and only 10% were in full agreement. All categories evaluated showed a type of irregularity. The items with highest percentage of non-compliance were not provided in technical regulations phrases and figures, symbols and illustrations and/or drawings, representing 85% and 63.3%, respectively. There were no irregularities for font size, label (existence and record), designation according to the PIC (Identity and Quality Standard), use of additives, method of preparation, manufacturer's data, origin, early childhood food, expression "contains phenylalanine" or "doesn't contain phenylalanine" main panel, expiration date, artificial coloring. Conclusion ­ The study indicates that there is a need for greater control for the labels have reliable information, in order to provide compliance with legal requirements and not to induce consumers to error.

Loss-of-function JAK3 mutations in TMD and AMKL of Down syndrome.

Acquired mutations activating Janus kinase 3 (jak3) have been reported in Down syndrome (DS) and non-DS patients with acute megakaryoblastic leukaemia (AMKL). This highlighted jak3-activation as an important event in the pathogenesis of AMKL, and predicted inhibitors of jak3 as conceptual therapeutics for AMKL. Of 16 DS-transient myeloproliferative disorder (TMD)/AMKL patients tested, seven showed JAK3 mutations. Three mutations deleted the kinase (JH1) domain, abolishing the main function of jak3. Another patient displayed a mutation identical to a previously reported inherited loss-of-function causing severe combined immunodeficiency. Our data suggest that both gain-, and loss-of function mutations of jak3 can be acquired in DS-TMD/AMKL.

[Risk factors for anemia among 6- to 12-month-old children in Brazil]. / Fatores de risco para anemia em crianças de 6 a 12 meses no Brasil.

OBJECTIVE: To estimate the prevalence of anemia and to determine associated risk factors among infants receiving routine health care in public clinics in Brazil. METHOD: This cross-sectional study included 2,715 infants between 6 and 12 months old in 12 cities, in all five of the geographic regions of Brazil. Information regarding the child and its feeding habits was obtained from the mother or other caregiver, using a questionnaire. Nutritional status was determined based on height and weight measurements. The hemoglobin concentration was measured using the HemoCue portable hemoglobinometer. Anemia was defined as hemoglobin < 11 g/dL. The infants' eating habits were assessed based on what they were eating around the time of the questionnaire interviews. The association between anemia and the different variables was evaluated through bivariate analysis, followed by multiple logistic regression using a hierarchical selection model. RESULTS: The prevalence of anemia for the entire group was 65.4%. Multiple regression analysis identified the following risk factors for anemia: living in the Southeastern Region of Brazil (odds ratio (OR) = 1.57, 95% confidence interval (95% CI) = 1.25- 1.99), maternal age < 20 years (OR = 1.58, 95% CI = 1.21-2.07), birthweight < 2,500 g (OR = 2.09, 95% CI = 1.48-2.95), not being breast-fed (OR = 1.28, 95% CI = 1.02-1.61), receiving both breast milk and other foods (OR = 1.40, 95% CI = 1.10-1.78), and male gender (OR = 1.24, 95% CI = 1.06-1.46). CONCLUSIONS: The high proportion of anemic children indicates the need to emphasize, in prenatal and infant health programs, intervention measures for anemia control. Our results could guide these measures, focusing on the groups at greatest risk, such as low birthweight babies and the children of adolescent mothers.

Somatic PTPN11 mutations in childhood acute myeloid leukaemia.

Somatic mutations in PTPN11, the gene encoding the transducer SHP-2, have emerged as a novel class of lesions that upregulate RAS signalling and contribute to leukaemogenesis. In a recent study of 69 children and adolescents with de novo acute myeloid leukaemia (AML), we documented a non-random distribution of PTPN11 mutations among French-American-British (FAB) subtypes. Lesions were restricted to FAB-M5 cases, where they were relatively common (four of 12 cases). Here, we report on the results of a molecular screening performed on 181 additional unselected patients, enrolled in participating institutions of the Associazione Italiana Ematologia Oncologia Pediatrica-AML Study Group, to provide a more accurate picture of the prevalence, spectrum and distribution of PTPN11 mutations in childhood AML and to investigate their clinical relevance. We concluded that PTPN11 defects do not represent a frequent event in this heterogeneous group of malignancies (4.4%), although they recur in a considerable percentage of patients with FAB-M5 (18%). PTPN11 lesions rarely occur in other subtypes. Within the FAB-M5 group no clear association of PTPN11 mutations with any clinical variable was evident. Nearly two third of the patients with this subtype were found to harbour an activating mutation in PTPN11, NRAS, KRAS2 or FLT3.

Teores de sódio e lipídios em refeições almoço consumidas por trabalhadores de uma empresa do município de Suzano, SP / Sodium and lipid contents of lunch meals consumed by workers of a company in Suzano, SP, Brazil

OBJETIVO: Avaliar os valores de sódio e lipídios em refeições almoço consumidas por usuários de uma unidade de alimentação e nutrição. MÉTODOS: Análise laboratorial das porções médias consumidas, utilizando-se o método de fotometria de chama para determinação de sódio e o método de extração de lipídios totais por meio de solventes orgânicos. Os resultados foram comparados com as recomendações da Organização Mundial da Saúde e com as novas recomendações do Programa de Alimentação do Trabalhador. RESULTADOS: Observaram-se valores de sódio e lipídios acima das recomendações do Programa de Alimentação do Trabalhador. Neste estudo, o valor médio de sódio foi de 2435mg (DP=518mg) e o valor energético médio de lipídios foi de 329kcal (DP=40kcal) em uma porção média de refeição - 745g (DP=60g). CONCLUSÃO: São necessárias mudanças para que haja uma adequação das quantidades de sal e de lipídios utilizadas nas refeições, uma redução da utilização de alimentos que contenham grande quantidade de sódio e/ou gordura, e que se programem ações educativas junto aos usuários da unidade de alimentação e nutrição, para a melhoria da qualidade de vida de quem representa a força produtiva da empresa.

OBJECTIVE: The objective of this study was to determine the sodium and lipid contents of lunch meals consumed in a food and nutrition unit. METHODS: Flame photometry and total lipid extraction with organic solvents were used to determine the amount of sodium and lipids, respectively, per average-size meal. The results were compared with the amounts recommended by the World Health Organization and Brazilian Worker's Food Program. RESULTS: Sodium and lipid contents were above those recommended by the Brazilian Worker's Food Program. The mean sodium content was 2435mg (SD=518mg) and mean energy provided by lipids was 329kcal (SD=40kcal) per average-size meal (745g, SD=60g). CONCLUSION: Changes are needed to reduce the amounts of sodium and lipids present in these meals, such as using fewer high-fat and/or high-sodium foods. Furthermore, nutrition education should be made available for users of these food and nutrition units in order to improve the quality of life of those who are the driving force of a company.

Genetic evidence for lineage-related and differentiation stage-related contribution of somatic PTPN11 mutations to leukemogenesis in childhood acute leukemia.

SHP-2 is a protein tyrosine phosphatase functioning as signal transducer downstream to growth factor and cytokine receptors. SHP-2 is required during development, and germline mutations in PTPN11, the gene encoding SHP-2, cause Noonan syndrome. SHP-2 plays a crucial role in hematopoietic cell development. We recently demonstrated that somatic PTPN11 mutations are the most frequent lesion in juvenile myelomonocytic leukemia and are observed in a smaller percentage of children with other myeloid malignancies. Here, we report that PTPN11 lesions occur in childhood acute lymphoblastic leukemia (ALL). Mutations were observed in 23 of 317 B-cell precursor ALL cases, but not among 44 children with T-lineage ALL. In the former, lesions prevalently occurred in TEL-AML1(-) cases with CD19(+)/CD10(+)/cyIgM(-) immunophenotype. PTPN11, NRAS, and KRAS2 mutations were largely mutually exclusive and accounted for one third of common ALL cases. We also show that, among 69 children with acute myeloid leukemia, PTPN11 mutations occurred in 4 of 12 cases with acute monocytic leukemia (FAB-M5). Leukemia-associated PTPN11 mutations were missense and were predicted to result in SHP-2 gain-of-function. Our findings provide evidence for a wider role of PTPN11 lesions in leukemogenesis, but also suggest a lineage-related and differentiation stage-related contribution of these lesions to clonal expansion.

Acquired mutations in GATA1 in neonates with Down's syndrome with transient myeloid disorder.

Transient myeloid disorder is a unique self-regressing neoplasia specific to Down's syndrome. The transcription factor GATA1 is needed for normal growth and maturation of erythroid cells and megakaryocytes. Mutations in GATA1 have been reported in acute megakaryoblastic leukaemia in Down's syndrome. We aimed to investigate changes in GATA1 in patients with Down's syndrome and either transient myeloid disorder (n=10) or acute megakaryoblastic leukaemia (n=6). We recorded mutations eliminating exon 2 from GATA1 in all patients with transient myeloid disorder (age 0-24 days) and in all with acute megakaryoblastic leukaemia (age 14-38 months). The range of mutations did not differ between patients with each disorder. Patients with transient myeloid disorder with mutations in GATA1 can regress spontaneously to complete remission, and mutations do not necessarily predict later acute megakaryoblastic leukaemia.

Microarray transcript profiling distinguishes the transient from the acute type of megakaryoblastic leukaemia (M7) in Down's syndrome, revealing PRAME as a specific discriminating marker.

Transient myeloproliferative disorder (TMD) is a unique, spontaneously regressing neoplasia specific to Down's syndrome (DS), affecting up to 10% of DS neonates. In 20-30% of cases, it reoccurs as progressive acute megakaryoblastic leukaemia (AMKL) at 2-4 years of age. The TMD and AMKL blasts are morphologically and immuno-phenotypically identical, and have the same acquired mutations in GATA1. We performed transcript profiling of nine TMD patients comparing them with seven AMKL patients using Affymetrix HG-U133A microarrays. Similar overall transcript profiles were observed between the two conditions, which were only separable by supervised clustering. Taqman analysis on 10 TMD and 10 AMKL RNA samples verified the expression of selected differing genes, with statistical significance (P < 0.05) by Student's t-test. The Taqman differences were also reproduced on TMD and AMKL blasts sorted by a fluorescence-activated cell sorter. Among the significant differences, CDKN2C, the effector of GATA1-mediated cell cycle arrest, was increased in AMKL but not TMD, despite the similar level of GATA1. In contrast, MYCN (neuroblastoma-derived oncogene) was expressed in TMD at a significantly greater level than in AMKL. MYCN has not previously been described in leukaemogenesis. Finally, the tumour antigen PRAME was identified as a specific marker for AMKL blasts, with no expression in TMD. This study provides markers discriminating TMD from AMKL-M7 in DS. These markers have the potential as predictive, diagnostic and therapeutic targets. In addition, the study provides further clues into the pathomechanisms discerning self-regressive from the progressive phenotype.

Avaliação do consumo de sal em uma unidade de alimentação e nutrição / Salt intake evaluation in a food service

Hypertension is currently considered one of the most common public health problems and it is associated to cardiovascular risks. Due to the great number of people who lunch at work, the increasing number of hypertension cases and the lack of reports about salt intake, the present study intended to evaluatesalt consumption in a Food and Nutrition Centre. The study was developed in the restaurant located in a pharmaceutical industry in Suzano ­ SP. The salt contents of foods, leftovers and sachets distributed to the workers were assessed. The average salt intake was calculated as the ratio of the amount of salt offered and the number of employees having lunch at the company's restaurant each day. The average daily salt consumption was (5.37±0.38) gper person. The study found a high prevalence of inactivity and 47.6% of overweight or obese workers. In addition, 53.9% of the normotense workers were eutrophic and 44.9% showed some degree of overweight. Among the hypertense workers, 16.4% were eutrophic and 83.6% showed some degree of overweight. In the population studied, high salt intake, overweight and physical inactivity can increase the incidence of hypertension and cardiovascular diseases

La hipertensión es uno de los problemas de salud pública más comunes en el mundo y está asociada a serios riesgos de morbimortalidad cardiovascular. Considerando el elevado número de trabajadores que almuerzan en el local de trabajo, el aumento de la incidencia de hipertensión y la escasez de estudios sobre sal,nos propusimos en este trabajo a estimar el consumo de sal de adición en una Unidad de Alimentación y Nutrición. El estudio fue realizado en el restaurante de una indústria farmacéutica del municipio de Suzano- SP. Fue estimada la sal utilizada en las preparaciones, la que era distribuida y la sal de las sobras y de los restos de comida. El consumo medio de sal por persona fue calculado dividiendo la sal ofrecida por el número de personas que participaban del almuerzo cada día. Los resultados mostraron un consumo medio de salde 5,37 (± 0,38) gramas, por persona, por almuerzo. Fue detectada también una grande inactividad física y que 47,6% de los trabajadores presentaban sobrepeso u obesidad. Entre los trabajadores con tensión normal,53,9% estaban con el peso adecuado para la altura y 44,9% mostraban algún grado de sobrepeso. Entre los funcionarios hipertensos apenas 16,4% eran eutróficos y 83,6% presentaban algún grado de sobrepeso. En la población estudiada, el elevado consumo de sal, el exceso de peso y la inactividad física pueden estar contribuyendo para la incidência de hipertensión observada y consecuentemente para el aparecimiento de enfermidades cardiovasculares

A hipertensão é, atualmente, um dos mais comuns problemas de saúde pública mundial e está associada a sérios riscos de morbimortalidade cardiovascular. Frente ao grande número de trabalhadores que fazem sua principal refeição na empresa onde trabalham, ao aumento dos casos de hipertensão e à escassez de artigos que tratam do consumo de sal, este estudo tem o objetivo de avaliar o consumo de sal de adição em uma Unidade de Alimentação e Nutrição. O estudo foi desenvolvido em um restaurante industrial de uma empresa farmacêutica do Município de Suzano ­ SP. Foram avaliados o sal utilizado nas preparações, o sal das sobras e dos restos e o sal distribuído. O consumo médio de sal por pessoa foi calculado pela razão entre o sal oferecido e o número de funcionários que almoçaram a cada dia. O consumo médio de sal por pessoa no almoço foi de 5,37 (± 0,38) gramas. Foi encontrada uma alta prevalência de inatividade e 47,6% dos funcionários apresentaram sobrepeso ou obesidade. Observou-se também que 53,9% dos funcionários normotensos apresentavam peso adequado para a altura e 44,9% apresentavam algum grau de sobrepeso. Dentre os funcionários hipertensos, verificou-se que apenas 16,4% eram eutróficos e 83,6% apresentavam algum grau de sobrepeso. Na população estudada, o elevado consumo de sal, o excesso de peso e a inatividade física podem contribuir para a incidência da hipertensão e, conseqüentemente, de doenças cardiovasculares