Antropometria e composição de massa corporal sugerem achado intrínseco da síndrome de Williams-Beuren

OBJECTIVE:Although considered a well-known condition, there is only one study describing the body composition among individuals with Williams-Beuren syndrome. The aim was to characterize the nutritional status in Brazilian individuals with this condition. METHODS: Cross-sectional study was designed to evaluate clinical and nutritional data of 17 Brazilian patients. Z-scores for height, weight, body mass index, triceps and subscapular skinfold thickness, arm circumference, arm muscle area, arm fat area were calculated. Wilcoxon's test was used to investigate differences between the z-scores of the anthropometrical measures and zero. RESULTS: Four children were considered stunted and two severely malnourished. The z-score mean value for height was -1.14 ± 1.00 (p-value = 0.004), for weight, -0.67 ± 1.19 (p-value = 0.0443), for arm circumference, -0.94 ± 1.14 (p-value = 0.0222), for triceps skinfold thickness, -0.59 ± 0.63 (p-value = 0.0042) and for arm fat area -0.67 ± 0.67 (p-value = 0.0061). CONCLUSION: Short stature seen in this series confirms a previous study describing this feature in a German population, which would suggest it as an intrinsic feature in Williams-Beuren syndrome. In addition, skinfold thickness measures have not been previously performed in this syndrome and detected abnormalities in fat stores in this sample. Considering this method a fast and low-cost way to evaluate body composition, similar studies could be performed in other populations in order to better characterize this issue. Morbidity related with this genetics condition and information for clinical investigation and clinical follow-up are also discussed.OBJETIVO: Embora a síndrome de Williams-Beuren seja bem conhecida, há apenas um estudo descrevendo a composição corporal nesses pacientes. O objetivo foi caracterizar o estado nutricional de brasileiros com síndrome de Williams. MÉTODOS: Utilizou-se um estudo transversal com a avaliação de dados clínicos e nutricionais de 17 pacientes. Foram calculados os escores-z do peso, estatura, índice de massa corpórea, dobras cutâneas, circunferência do braço e áreas muscular e adiposa do braço. Para verificar diferenças, foi utilizado o teste de Wilcoxon, sendo considerado significativo p < 0,05. RESULTADOS: Os valores médios dos escores-z foram: -1,14 ± 1,00 (p = 0,004) para estatura, -0,67 ± 1,19 (p = 0,0443) para peso, -0,94 ± 1,14 (p = 0,0222) para a circunferência do braço, -0,59 ± 0,63 (p = 0,0042) para dobra cutânea do tríceps e -0,67 ± 0,67 (p = 0,0061) para a área gordurosa do braço. Foi observada desnutrição pregressa em quatro pacientes e crônica, em dois. CONCLUSÃO: A baixa estatura, semelhante à descrita na população alemã, parece ser intrínseca à síndrome. Esse foi o primeiro estudo que avaliou a composição corporal, utilizando as dobras cutâneas, e detectou anormalidades nas reservas de gordura. O método aqui utilizado é simples, rápido e de baixo custo, facilitando estudos similares em outras populações. Isso poderia melhor caracterizar esses aspectos na síndrome de Williams. Morbidade relacionada a essa doença e informações para investigação e seguimento clinico são discutidas.68168

Effect of synbiotic supplementation in children and adolescents with cystic fibrosis: a randomized controlled clinical trial

BACKGROUND/OBJECTIVES:Cystic fibrosis (CF) is characterized by excessive activation of immune processes. The aim of this study was to evaluate the effect of synbiotic supplementation on the inflammatory response in children/adolescents with CF. SUBJECTS/METHODS:A randomized, placebo-controlled, double-blind, clinical-trial was conducted with control group (CG, n = 17), placebo-CF-group (PCFG, n = 19), synbiotic CF-group (SCFG, n = 22), PCFG negative (n = 8) and positive (n = 11) bacteriology, and SCFG negative (n = 12) and positive (n = 10) bacteriology. Markers of lung function (FEV1), nutritional status [body mass index-for age (BMI/A), height-for-age (H/A), weight-for-age (W/A), upper-arm fat area (UFA), upper-arm muscle area (UMA), body fat (%BF)], and inflammation [interleukin (IL)-12, tumor necrosis factor-alpha (TNF-α), IL-10, IL-6, IL-1β, IL-8, myeloperoxidase (MPO), nitric oxide metabolites (NOx)] were evaluated before and after 90-day of supplementation with a synbiotic. RESULTS:No significance difference was found between the baseline and end evaluations of FEV1 and nutricional status markers. A significant interaction (time vs. group) was found for IL-12 (p = 0.010) and myeloperoxidase (p = 0.036) between PCFG and SCFG, however, the difference was not maintained after assessing the groups individually. NOx diminished significantly after supplementation in the SCFG (p = 0.030). In the SCFG with positive bacteriology, reductions were found in IL-6 (p = 0.033) and IL-8 (p = 0.009) after supplementation. CONCLUSIONS: Synbiotic supplementation shown promise at diminishing the pro-inflammatory markers IL-6, IL-8 in the SCFG with positive bacteriology and NOx in the SCFG in children/adolescents with CF

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Influencia das proteinas de soro de leite bovino no estado nutricional, composição corporal e sistema imune em coorte de crianças com Sindrome da Imunodeficiencia Adquirida (AIDS)

Orientadores: Valdemiro Carlos Sgarbieri, Maria Marluce dos Santos VilelaDissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Engenharia de AlimentosResumo: As crianças infectadas pelo HIV-1 despertam preocupações especiais em relação à nutrição e ao estado de saúde, pois os efeitos imunossupressores da infecção colocam-nas sobre grande risco de complicações infecciosas e deficit nutricional. O desenvolvimento de estratégias que visem a recuperação do estado nutricional e o estímulo da resposta imunológica é de grande interesse para indivíduos imunodeprimidos. A suplementação alimentar com as proteínas do soro de leite bovino é reconhecida como estimulante do sistema imune, devido ao aumento da síntese de glutationa. Esse antioxidante se encontra reduzido em indivíduos infectados pelo HIV-1 e correlaciona-se com a progressão da AIDS. Com o objetivo de conhecer a influência da suplementação alimentar oral com determinado Concentrado de Proteínas de Soro de Leite Bovino sobre o estado nutricional, a composição corporal e o sistema imune inato e adaptativo em coorte de crianças com AIDS, foi realizado um ensaio clínico. Esse ensaio foi prospectivo, duplo-cego, com 18 crianças, entre 1 e 6 anos de idade que receberam como suplemento nutricional oral o Concentrado de Proteínas de Soro de Leite ou placebo (maltodextrina) ao longo de 4 meses. O estado nutricional foi determinado pelo ganho de peso, crescimento e ingestão alimentar; a composição corpórea, através do perímetro braquial, pregas cutâneas, composição corpórea e, o sistema imune, pelos níveis de glutationa dos eritrócitos, liberação dos intermediários reativos do oxigênio, linfócitos T CD4+ e CD8+ e ocorrência de infecções associadas. Os dados obtidos foram submetidos à análise estatística. Não foram observadas evidências de influência da suplementação alimentar com o Concentrado de Proteínas de Soro de Leite Bovino sobre o estado nutricional ou sobre a composição corporal da coorte estudada. Quanto ao sistema imunológico, obteve-se modulação da síntese de glutationa eritrocitária e redução da ocorrência dos episódios infecciosos. Não foi observada, entretanto influência sobre os marcadores de estresse oxidativo. Conclui-se que o Concentrado de Proteínas do Soro de Leite Bovino modularam o sistema imune através do estímulo à síntese de glutationa nos eritrócitos e diminuição dos episódios infecciosos.Abstract: HIV-1 infected children rise special concem about their nutritional and health status, due to immunosuppressive effects of the infection. The development of strategies for the recovery of the nutritional status and enhancement of the immune system is of great interest for immunodepressed patients. Nutritional supplementation with whey proteins is recognized as stimulating the immune system toward an increase of glutathione synthesis. The levels of this antioxidant are reduced in some HIV-1 infected individuaIs and are correlated with disease progression. With the objective of investigating the influence of a whey protein concentrate on the nutritional status body composition, innate and adaptative immune system in a cohort of children with AIDS, a prospective double-blind clinical trial with 18 children, between 1 and 6 years of age was conduced. During the experiment, children received whey protein or placebo (maltodextrin) for 4 months. The nutritional status was assessed by weight gain, growth and food intake. Midarm circumference, skinfold thickness and bioelectrical impedance were used to determine body composition. Influence on immune system was evaluated by erythrocyte glutathione levels, reactive oxygen species release, T lymphocytes counts (CD4+and CD8+) and occurrence of associated infections. Data were submitted to statistical analysis. Whey proteins did not influence nutritional status or body composition, but a modulation on the glutathione synthesis, a trend for improvement on CD4+/CD8+ ratios and reduction of infectious episodes were observed. The data suggested that whey proteins modulate the immune system by the increase of the erythrocyte glutathione synthesis and reduction of the opportunistic episodes.MestradoMestre em Alimentos e Nutriçã

Healthy lifestyle moderates the relationship between cardiovascular disease with blood pressure, body composition, carotid intima-media thickness, and glycated hemoglobin among adults

We investigate whether the adherence to healthy lifestyle recommendations (regular physical activity, healthy diet, reduced alcohol consumption, nonsmoking) moderates the relationship between cardiovascular disease (CVD) and its risk factors (obesity, diabetes, dyslipidemia, and hypertension) with clinical variables among adults. This cross-sectional population-based study comprised 862 adults (39.3 ± 11.4 years, 46.4% men) from Florianópolis, Brazil. Clinical variables were systolic (SBP) and diastolic blood pressure, waist circumference, body mass index (BMI), percentage of body fat, muscular strength, carotid intima-media thickness (IMT), high sensitive C-reactive protein, and lipid and glucose metabolism markers. Multiple linear regression adjusted for confounding factors was used. Reduced IMT and HbA1c were observed in males with CVD or its risk factors who adopted healthy lifestyle recommendations (p < 0.001), and lower SBP levels were observed in females without CVD or its risk factors (p = 0.034). Females with CVD or its risk factors who followed healthy lifestyle recommendations showed higher BMI (p = 0.035). Adherence to number of healthy lifestyle recommendations moderated cardiovascular health in adults with CVD and without CVD. Novelty The adherence to healthy lifestyle recommendations moderated the relationship between IMT and HbA1c with CVD or its risk factors among males. The lower values of SBP among females without CVD or its risk factors were moderated by the adherence to healthy lifestyle recommendations.The accepted manuscript in pdf format is listed with the files at the bottom of this page. The presentation of the authors' names and (or) special characters in the title of the manuscript may differ slightly between what is listed on this page and what is listed in the pdf file of the accepted manuscript; that in the pdf file of the accepted manuscript is what was submitted by the author

Partial chemical and functional characterization of milk whey products obtained by different processes Caracterização parcial química e funcional de produtos de soro de leite obtidos por diferentes processos

Whey protein samples (S-1 to S-5) were tested in vivo and in vitro for nutritional properties and selected bioactivities. Weanling male Wistar rats fed modified AIN-93G (12 g protein.100 g-1) diets for 21 days were used the in vivo studies. The nutritional parameters did not differ among the protein diets tested. Erythrocyte glutathione content was considered high and was higher for S-3, but liver glutathione was the same for all dietary groups. For S-3, cytokine secretion (IL-10 and TNF-&#945;) by human peripheral blood mononuclear cells (in RPMI-1640 medium) was higher in the absence of antigen than in the presence of BCG antigen. Interleukin-4 secretion was repressed in all treatments. The IC50, whey protein concentration required to inhibit 50% of the melanoma cell proliferation, was 2.68 mg.mL-1 of culture medium for the S-3 sample and 3.66 mg.mL-1 for the S-2 sample. Based on these results, it was concluded that S-3 (whey protein concentrate enriched with TGF-&#946; and lactoferrin) produced better nutritional and immunological responses than the other products tested.Amostras de proteínas de soro de leite bovino (S-1 a S-5) foram testadas, in vivo e in vitro, para algumas propriedades nutricionais e bioatividades. Para os testes in vivo foram usados ratos da linhagem Wistar alimentados por 21 dias com dieta AIN-93G modificada quanto ao teor de proteína (12 g proteínas.100 g-1 de dieta). Os parâmetros nutricionais medidos não diferiram entre as proteínas do soro de leite administradas através das dietas. O teor de glutationa nos eritrócitos foi mais elevado para o grupo alimentado com a amostra S-3. No grupo alimentado com S-3, a secreção de citocinas (IL-10 e TNF-&#945;) por células humanas mononucleares da circulação periférica, cultivadas no meio RPMI-1640, foi mais elevada na ausência do antígeno BCG do que em sua presença. A secreção de IL-4 foi inibida em todos os tratamentos. O IC50, concentração de proteína de soro necessária para inibir 50% da proliferação de células de melanoma de camundongo foi 2,68 mg.mL-1 de meio de cultura para a amostra S-3 e 3,66 mg.mL-1 para a amostra S-2. Com base nesses resultados concluiu-se que a amostra S-3 (concentrado de proteína de soro enriquecida com TGF-&#946; e lactoferrina) produziu melhores respostas nutricional e imunológica quando comparada aos demais produtos testados