IGF-I and IGF Binding Protein-3 Generation Tests and Response to Growth Hormone in Children with Silver-Russell Syndrome

Objectives. To evaluate, in children with Silver-Russell Syndrome, the response to the IGF-I and IGFBP-3 generation test and compare results to the growth response after 6 months of rhGH. Methods. Eight children (6 males), with a mean age of 5.71 ± 2.48 years and height SDS of −3.88 ± 1.28 received rhGH for 6 months. IGF-I and IGFBP-3 were analyzed before and after 4 doses of rhGH. Results. The mean growth velocity (GV) before treatment was 5.28 ± 1.9 cm/year. GV increased after rhGH in five children to a mean GV of 10.3 ± 3.64 cm/year. Six children had normal basal IGF-I levels and two low levels. After 4 doses of rhGH, the IGF-I levels were normal in seven. There was no correlation between the growth response and the IGF-I generation test. Conclusions. Children with SRS have normal IGF-I generation test. There is no correlation between the generation test and the growth velocity after 6 months of rhGH

Comparative study of human exposure to mercury in riverside communities in the Amazon region

Four populations in the Amazon area were selected for a comparative study of mercury-exposed and non-exposed populations: São Luiz do Tapajós, Barreiras, Panacauera, and Pindobal Grande. The highest mercury levels in human hair samples were found in São Luiz do Tapajós and Barreiras, greatly exceeding the limits established by the World Health Organization. Panacauera showed an intermediate level below 9 µg/g. This was the first comparative and simultaneous evaluation of mercury exposure in the Amazon area. Also, thanks to this type of monitoring, we were able to eliminate the uncertainties about the reference dose. On the basis of these data, we can conclude that the mercury levels detected in exposed populations of the Tapajós River basin may be dangerous not only because they are above the World Health Organization limits, but also because the simultaneous mercury detection in non-exposed populations with similar characteristics provided a valid control and revealed lower mercury levels. Our results support the importance of continuous monitoring in both exposed and non-exposed populations

Insulin resistance in adolescents with Down syndrome: a cross-sectional study

BACKGROUND: The prevalence of diabetes mellitus is higher in individuals with Down syndrome (DS) than in the general population; it may be due to the high prevalence of obesity presented by many of them. The aim of this study was to evaluate the insulin resistance (IR) using the HOMA (Homeostasis Model Assessment) method, in DS adolescents, describing it according to the sex, body mass index (BMI) and pubertal development. METHODS: 15 adolescents with DS (8 males and 7 females) were studied, aged 10 to 18 years, without history of disease or use of medication that could change the suggested laboratory evaluation. On physical examination, the pubertal signs, acanthosis nigricans (AN), weight and height were evaluated. Fasting plasma glucose and insulin were analysed by the colorimetric method and RIA-kit LINCO, respectively. IR was calculated using the HOMA method. The patients were grouped into obese, overweight and normal, according to their BMI percentiles. The EPIINFO 2004 software was used to calculate the BMI, its percentile and Z score. RESULTS: Five patients were adults (Tanner V or presence of menarche), 9 pubertal (Tanner II – IV) and 1 prepubertal (Tanner I). No one had AN. Two were obese, 4 overweight and 9 normal. Considering the total number of patients, HOMA was 1.7 ± 1.0, insulin 9.3 ± 4.8 μU/ml and glucose 74.4 ± 14.8 mg/dl. The HOMA values were 2.0 ± 1.0 in females and 1.5 ± 1.0 in males. Considering the nutritional classification, the values of HOMA and insulin were: HOMA: 3.3 ± 0.6, 2.0 ± 1.1 and 1.3 ± 0.6, and insulin: 18.15 ± 1.6 μU/ml, 10.3 ± 3.5 μU/ml and 6.8 ± 2.8 μU/ml, in the obese, overweight and normal groups respectively. Considering puberty, the values of HOMA and insulin were: HOMA: 2.5 ± 1.3, 1.4 ± 0.6 and 0.8 ± 0.0, and insulin: 13.0 ± 5.8 μU/ml, 7.8 ± 2.9 μU/ml and 4.0 ± 0.0 μU/ml, in the adult, pubertal and prepubertal groups respectively. CONCLUSION: The obese and overweight, female and adult patients showed the highest values of HOMA and insulin

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

Combining ability of maize grain yield under different levels of environmental stress Capacidade de combinação da produtividade de milho em ambientes com diferentes intensidades de estresses

The objectives of this work were to caracterize the tropical maize germplasm and to compare the combining abilities of maize grain yield under different levels of environmental stress. A diallel was performed among tropical maize cultivars with wide adaptability, whose hybrid combinations were evaluated in two sowing dates, in two years. The significance of the environmental effect emphasized the environmental contrasts. Based on grain yield, the environments were classified as favorable (8,331 kg ha-1), low stress (6,637 kg ha-1), high stress (5,495 kg ha-1), and intense stress (2,443 kg ha-1). None of the genetic effects were significant in favorable and intense stress environments, indicating that there was low germplasm variability under these conditions. In low and high stresses, the specific combining ability effects (SCA) were significant, showing that the nonadditive genetic effects were the most important, and that it is possible to select parent pairs with breeding potential. SCA and grain yield showed significant correlations only between the closer environment pairs like favorable/low stress and high/intense stress. The genetic control of grain yield differed under contrasting stress environments for which maize cultivars with wide adaptability are not adequate.<br>O objetivo deste trabalho foi caracterizar o germoplasma de milho e comparar a capacidade de combinação da produtividade do milho em ambientes com diferentes graus de estresse. Um dialelo foi realizado entre cultivares de milho tropical com ampla adaptabilidade, cujas combinações híbridas foram avaliadas em duas épocas de plantio, em dois anos. A significância do efeito ambiental mostrou que os ambientes foram contrastantes. Com base na produtividade, os ambientes foram classificados como: favorável (8.331 kg ha-1); com baixo estresse (6.637 kg ha-1); com alto estresse (5.495 kg ha-1); e com intenso estresse (2.443 kg ha-1). Nenhum dos efeitos genéticos foi significativo nos ambientes classificados como favorável e com intenso estresse, o que indica haver baixa variabilidade para as combinações genéticas nesses ambientes. Em baixo e alto estresse, os efeitos da capacidade de combinação específica foram significativos, o que mostra que os efeitos genéticos não aditivos foram os mais importantes, e que é possível selecionar pares de genitores com potencial para melhoramento. A capacidade geral de combinação e a produtividade de grãos apresentaram correlações significativas somente entre os ambientes mais próximos como favorável/baixo estresse e alto/intenso estresse. O controle genético da produtividade de grãos difere em ambientes contrastantes quanto ao estresse para os quais as cultivares de milho com ampla adaptabilidade não são adequadas