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Eur J Endocrinol ; 184(2): 267-276, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33434161


Objective: The European Increlex® Growth Forum Database Registry monitors the effectiveness and safety of recombinant human insulin-like growth factor-1 (rhIGF1; mecasermin, Increlex®) therapy in patients with severe primary IGF1 deficiency (SPIGFD). We present data from patients with and without a reported genetic diagnosis of Laron syndrome (LS). Design: Ongoing, open-label, observational registry (NCT00903110). Methods: Children and adolescents receiving rhIGF1 therapy from 10 European countries were enrolled in 2008-2017 (n = 242). The treatment-naïve/prepubertal (NPP) cohort (n = 138) was divided into subgroups based on reported genetic diagnosis of LS (n = 21) or non-LS (n = 117). Multivariate analysis of the NPP-non-LS subgroup was conducted to identify factors predictive of growth response (first-year-height standard deviation score (SDS) gain ≥ 0.3). Assessments included change in height and weight over 5 years and adverse events (AEs). Results: Height SDS gain from baseline was greater in the NPP-LS than the NPP-non-LS subgroup after 1 years' treatment (P < 0.05). In the NPP-non-LS subgroup, 56% were responders; young age at baseline was a positive independent predictive factor (P < 0.001). NPP-non-LS-responders and the NPP-LS subgroup had a similar mean age (6.07 years vs 7.00 years) at baseline and height SDS gain in year 1 (0.64 vs 0.70), although NPP-non-LS-responders were taller (P < 0.001) at baseline. BMI SDS changes did not differ across subgroups. Treatment-emergent AEs were experienced by 65.3% of patients; hypoglycaemia was most common. Conclusions: In most NPP children with SPIGFD, with or without LS, rhIGF1 therapy promotes linear growth. The safety profile was consistent with previous studies.

Transtornos do Crescimento/tratamento farmacológico , Perda Auditiva Neurossensorial/tratamento farmacológico , Fator de Crescimento Insulin-Like I/deficiência , Fator de Crescimento Insulin-Like I/uso terapêutico , Síndrome de Laron/tratamento farmacológico , Proteínas Recombinantes/uso terapêutico , Adolescente , Estatura , Peso Corporal/efeitos dos fármacos , Criança , Feminino , Crescimento/efeitos dos fármacos , Humanos , Hipoglicemia/sangue , Hipoglicemia/induzido quimicamente , Síndrome de Laron/genética , Estudos Longitudinais , Masculino , Segurança do Paciente , Puberdade , Resultado do Tratamento , Adulto Jovem
Mar Drugs ; 18(12)2020 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-33333811


High-fat diet (HFD) usually induces oxidative stress and astaxanthin is regarded as an excellent anti-oxidant. An 8-week feeding trial was conducted to investigate the effects of dietary astaxanthin supplementation on growth performance, lipid metabolism, antioxidant ability, and immune response of juvenile largemouth bass (Micropterus salmoides) fed HFD. Four diets were formulated: the control diet (10.87% lipid, C), high-fat diet (18.08% lipid, HF), and HF diet supplemented with 75 and 150 mg kg-1 astaxanthin (HFA1 and HFA2, respectively). Dietary supplementation of astaxanthin improved the growth of fish fed HFD, also decreased hepatosomatic index and intraperitoneal fat ratio of fish fed HFD, while having no effect on body fat. Malondialdehyde content and superoxide dismutase activity were increased in fish fed HFD, astaxanthin supplementation in HFD decreased the oxidative stress of fish. The supplementation of astaxanthin in HFD also reduced the mRNA levels of Caspase 3, Caspase 9, BAD, and IL15. These results suggested that dietary astaxanthin supplementation in HFD improved the growth performance, antioxidant ability and immune response of largemouth bass.

Antioxidantes/metabolismo , Bass , Dieta Hiperlipídica , Suplementos Nutricionais , Crescimento/efeitos dos fármacos , Imunidade/efeitos dos fármacos , Adiposidade/efeitos dos fármacos , Animais , Distribuição da Gordura Corporal , Citocinas/metabolismo , Inflamação/metabolismo , Inflamação/prevenção & controle , Metabolismo dos Lipídeos/efeitos dos fármacos , Malondialdeído/metabolismo , Estresse Oxidativo/efeitos dos fármacos , Superóxido Dismutase/metabolismo , Xantofilas/química , Xantofilas/farmacologia
Cochrane Database Syst Rev ; 9: CD001912, 2020 09 30.
Artigo em Inglês | MEDLINE | ID: mdl-32997797


BACKGROUND: Staphylococcus aureus causes pulmonary infection in young children with cystic fibrosis. Prophylactic antibiotics are prescribed hoping to prevent such infection and lung damage. Antibiotics have adverse effects and long-term use might lead to infection with Pseudomonas aeruginosa. This is an update of a previously published review. OBJECTIVES: To assess continuous oral antibiotic prophylaxis to prevent the acquisition of Staphylococcus aureus versus no prophylaxis in people with cystic fibrosis, we tested the following hypotheses to investigate whether prophylaxis: 1. improves clinical status, lung function and survival; 2. leads to fewer isolates of Staphylococcus aureus; 3. causes adverse effects (e.g. diarrhoea, skin rash, candidiasis); 4. leads to fewer isolates of other common pathogens from respiratory secretions; 5. leads to the emergence of antibiotic resistance and colonisation of the respiratory tract with Pseudomonas aeruginosa. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, comprising references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. Companies manufacturing anti-staphylococcal antibiotics were contacted. Most recent search of the Group's Register: 27 February 2020. Online trials registries were also searched. Most recent search of online trials registries: 15 September 2020. SELECTION CRITERIA: Randomised trials of continuous oral prophylactic antibiotics (given for at least one year) compared to intermittent antibiotics given 'as required', in people with cystic fibrosis of any disease severity. DATA COLLECTION AND ANALYSIS: The authors assessed studies for eligibility and methodological quality and extracted data. The quality of the evidence was assessed using the GRADE criteria. The review's primary outcomes of interest were lung function by spirometry (forced expiratory volume in one second (FEV1)) and the number of people with one or more isolates of Staphylococcus aureus (sensitive strains). MAIN RESULTS: We included four studies, with a total of 401 randomised participants aged zero to seven years on enrolment; one study is ongoing. The two older included studies generally had a higher risk of bias across all domains, but in particular due to a lack of blinding and incomplete outcome data, than the two more recent studies. We only regarded the most recent study as being generally free of bias, although even here we were not certain of the effect of the per protocol analysis on the study results. Evidence quality was judged to be low for all outcomes assessed after being downgraded based on GRADE assessments. Downgrading decisions were due to limitations in study design (all outcomes), for imprecision and for inconsistency . Prophylactic anti-staphylococcal antibiotics probably make little or no difference to lung function measured as FEV1 % predicted after six years (mean difference (MD) -2.30, 95% confidence interval (CI) -13.59 to 8.99, one study, n = 119, low-quality evidence); but may reduce the number of children having one or more isolates of Staphylococcus aureus at two years (odds ratio (OR) 0.21, 95% CI 0.13 to 0.35, three studies, n = 315, low-quality evidence). At the same time point, there may be little or no effect on nutrition as reported using weight z score (MD 0.06, 95% CI -0.33 to 0.45, two studies, n = 140, low-quality evidence), additional courses of antibiotics (OR 0.18, 95% CI 0.01 to 3.60, one study, n = 119, low-quality evidence) or adverse effects (low-quality evidence). There was no difference in the number of isolates of Pseudomonas aeruginosa between groups at two years (OR 0.74, 95% CI 0.45 to 1.23, three studies, n = 312, low-quality evidence), though there was a trend towards a lower cumulative isolation rate of Pseudomonas aeruginosa in the prophylaxis group at two and three years and towards a higher rate from four to six years. As the studies reviewed lasted six years or less, conclusions cannot be drawn about the long-term effects of prophylaxis. AUTHORS' CONCLUSIONS: Anti-staphylococcal antibiotic prophylaxis may lead to fewer children having isolates of Staphylococcus aureus, when commenced early in infancy and continued up to six years of age. The clinical importance of this finding is uncertain. Further research may establish whether the trend towards more children with CF with Pseudomonas aeruginosa, after four to six years of prophylaxis, is a chance finding and whether choice of antibiotic or duration of treatment might influence this.

Antibioticoprofilaxia , Fibrose Cística/microbiologia , Infecções Respiratórias/prevenção & controle , Infecções Estafilocócicas/prevenção & controle , Staphylococcus aureus , Viés , Criança , Pré-Escolar , Farmacorresistência Bacteriana , Volume Expiratório Forçado , Crescimento , Humanos , Lactente , Recém-Nascido , Pseudomonas aeruginosa/isolamento & purificação , Ensaios Clínicos Controlados Aleatórios como Assunto , Staphylococcus aureus/isolamento & purificação
Rev. cuba. med. mil ; 49(3): e573, jul.-set. 2020. []
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1144482


Introducción: En los últimos años la producción científica en América Latina y el Caribe, como en el resto del mundo, ha estado influenciada por el desarrollo de la red de redes y el movimiento de acceso abierto. También ha incidido en su desarrollo la escasa inversión en investigación, desarrollo e innovación (I+D+i), la poca cultura para la divulgación científica de muchos investigadores y la supremacía de la lengua inglesa en las revistas científicas más importantes. Objetivo: Analizar el estado de la producción científica en América Latina y el Caribe de 1996 a 2019. Desarrollo: Se analizan los principales constructos teóricos para realizar estudios bibliométricos. También se analiza el ranking latinoamericano de producción científica de las revistas indexadas en Scopus en el período 1996-2019, a partir de la herramienta SCImago Journal & Country Rank; particularmente en sus siete principales países. Conclusiones: Los resultados muestran que la producción científica en América Latina y el Caribe, ha tenido un importante crecimiento en los últimos 20 años, sin embargo, en el aumento de la socialización de los resultados científicos de investigación, existe concentración en solo algunos países y poco reconocimiento a escala global(AU)

Introduction: In recent years, scientific production in Latin America and the Caribbean, as in the rest of the world, has been influenced by the development of the network of networks and the Open Access Movement. The low investment in Research, Development and innovation (R + D + i) has also affected its development; the low culture for the scientific dissemination of many researchers; and the supremacy of the English language in the most important scientific journals. Objective: To analyze the state of scientific production in Latin America and the Caribbean from 1996-2019. Development: The main theoretical constructs for bibliometric studies are analyzed. The Latin American Ranking of scientific production of journals indexed in Scopus in the period 1996-2019 is also analyzed, based on the SCImago Journal & Country Rank tool; particularly in its seven main countries. Conclusions: The results show that scientific production in Latin America and the Caribbean has had an important growth in the last 20 years; however, in increasing the socialization of scientific research results, there is concentration in only some countries and little recognition on a global scale(AU)

Humanos , Publicações Periódicas como Assunto , Acesso à Informação , Pesquisa Científica e Desenvolvimento Tecnológico , Crescimento
Biosci. j. (Online) ; 36(5): 1507-1517, 01-09-2020. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1147329


Shading screens are widely used to control excessive solar radiation, thereby changing plant growth. Thus, through physiological indices the objective was to evaluate the growth of three cultivars of minitomatoes under colored meshes. The experiment was conducted from April to July in the DTCS / UNEB experimental field in experiment DBC with subdivided plots, and four replicates, comprised of three shaded environments and the control treatment, three cultivars, and seven plant sampling seasons. For growth analysis, we used the destructive method, total dry matter was determined after drying it in an oven at 65°C. The total leaf area (LAI) was obtained using the software QUANT. Results showed that there was no interaction between environments and the other factors. Regarding leaf area index, cvs. Shani and Sweet Million stood out in all environments, whereas cv. Red Sugar had a similar behavior in all environments. Shani obtained the best indices in open sun environment and in the environment covered with red shading screen, and Sweet Million obtained the best index under gray shading screen. Red Sugar had the lowest LAI, and consequently, the lowest SLA and LAR. On the other hand, it showed the highest RPF, since it obtained the highest dry matter in leaves compared to the other cultivars. Cvs. Shani and Sweet Million showed the best: TAL, TCR, TCA and TCC.

As telas de sombreamento são utilizadas para controlar a radiação solar excessiva, podendo alterar o crescimento das plantas. Desse modo, objetivou-se por meio de índices fisiológicos avaliar o crescimento de três cultivares de mini tomates sob malhas coloridas. O experimento foi conduzido no período de abril a julho no campo experimental do DTCS/UNEB em delineamento experimental DBC, com parcelas subsubdivididas, com quatro repetições, compostos de três ambientes sombreados além do controle, três cultivares e sete épocas de amostragens. Para análise de crescimento, utilizou-se o método destrutivo e as massas secas totais obtidas após secagem em estufa a 65°C. A área foliar total foi obtida usando-se o software QUANT. Os resultados mostraram que não houve interação entre os ambientes e os demais fatores. Quanto as cvs., para índice de área foliar (LAI), destacaram-se as cvs. Shani e Sweet Million dentro de cada ambiente, tendo a cultivar Red Sugar comportamento similar nos ambientes. A cv. Shani obteve os melhores índices no ambiente a céu aberto e com tela vermelha e a cv. Sweet Million o melhor índice na tela cinza. A cv. Red Sugar apresentou o menor LAI, e consequentemente à menor AFE e RAF, em contrapartida apresentou a maior RPF, uma vez que obteve a maior massa seca nas folhas quando comparada as outras cultivares. As cvs. Shani e Sweet Million apresentaram as melhores: TAL, TCR, TCA e TCC.

Lycopersicon esculentum , Ambiente Controlado , Fenômenos Fisiológicos , Radiação Solar , Crescimento
Caracas; Observatorio Nacional de Ciencia, Tecnología e Innovación; ago. 2020. 144-167 p. (Observador del Conocimiento. Revista Especializada en Gestión Social del Conocimiento, 5, 2).
Monografia em Espanhol | LILACS, LIVECS | ID: biblio-1118165


Instalada la pandemia de la Covid-19 la gran expectativa colectiva gira en torno al retorno a la normalidad. En este ensayo se pone en entredicho el concepto de 'normalidad' en el contexto de los factores determinantes de la emergencia de los virus con altas tasas de morbilidad y mortalidad, evidenciando que el estado de 'normalidad pre-pandemia', al cual se aspira retornar, presenta indicadores socio-ambientales mucho más preocupantes y trágicos que aquellos que la mediática global nos suministra, de manera permanente y en tiempo real, sobre la Covid-19. Se presentan evidencias para argumentar que el principio sine qua non del crecimiento económico como motor determinante e insustituible del desarrollo, y más aún del modelo capitalista de desarrollo, es metabólicamente insostenible al demandar cantidades permanentemente incrementales de energía y bienes de la naturaleza en un planeta incapaz de sostener semejante pretensión. Las pandemias no son las únicas consecuencias fatales de la destrucción de la naturaleza y tampoco las más graves. Son síntomas de un modelo en crisis, que el desarrollo sustentable pretende aliviar sin avocarse al tratamiento de la patología que los origina. Por este motivo, se sostiene que es necesario construir una 'nueva normalidad', diferente a la 'normalidad pre-pandemia' por ser esta inviable, lo que implica evaluar los fundamentos del modelo. Se convoca a una impostergable reflexión, diversa e inclusiva, desde el seno de la comunidad científica, con el desprendimiento suficiente para admitir otras visiones y conocimientos que tienen mucho que aportar desde otras formas de coexistir con la naturaleza(AU)

Once Covid-19 pandemic established among us, the great collective expectation is about the return to normal pre-pandemic conditions. This assay questions the concept of 'normality' related to the context where high morbidity-mortality virus emerge out, showing that pre-pandemic normal conditions, have worst and overwhelming socio-environmental indicators than Covid-19 reports, daily supplied by global mass media in real time. We offer evidences that suggest that the sine qua non principle of economic growth, as indisputable driver of development, and even more, of the capitalist model, is metabolically unsustainable, due to the astonishing demands of energy and goods from nature, beyond its own capacity. Pandemics are not the only fatal consequences of ecological destruction, and by no means among the worst. They are just a symptom of the crisis of the development model, which the sustainable development pretends to relieve, ignoring the pathology that originated it. Due to this fact, we sustain that a new normal conditions is required to be created, truly different to pre-pandemic conditions, and that demands assessing the model's principles. We invite the scientific society for an urgent, inclusive and diverse reflection, willing to recognize other perspectives and knowledge, that could offer alternatives ways to coexist with nature(AU)

Desenvolvimento Econômico , Infecções por Coronavirus , Pandemias , Desenvolvimento Sustentável , Crescimento , Venezuela , Capitalismo , Determinantes Sociais da Saúde
N Engl J Med ; 383(4): 321-333, 2020 07 23.
Artigo em Inglês | MEDLINE | ID: mdl-32706533


BACKGROUND: Environmental enteric dysfunction (EED) is an enigmatic disorder of the small intestine that is postulated to play a role in childhood undernutrition, a pressing global health problem. Defining the incidence of this disorder, its pathophysiological features, and its contribution to impaired linear and ponderal growth has been hampered by the difficulty in directly sampling the small intestinal mucosa and microbial community (microbiota). METHODS: In this study, among 110 young children (mean age, 18 months) with linear growth stunting who were living in an urban slum in Dhaka, Bangladesh, and had not benefited from a nutritional intervention, we performed endoscopy in 80 children who had biopsy-confirmed EED and available plasma and duodenal samples. We quantified the levels of 4077 plasma proteins and 2619 proteins in duodenal biopsy samples obtained from these children. The levels of bacterial strains in microbiota recovered from duodenal aspirate from each child were determined with the use of culture-independent methods. In addition, we obtained 21 plasma samples and 27 fecal samples from age-matched healthy children living in the same area. Young germ-free mice that had been fed a Bangladeshi diet were colonized with bacterial strains cultured from the duodenal aspirates. RESULTS: Of the bacterial strains that were obtained from the children, the absolute levels of a shared group of 14 taxa (which are not typically classified as enteropathogens) were negatively correlated with linear growth (length-for-age z score, r = -0.49; P = 0.003) and positively correlated with duodenal proteins involved in immunoinflammatory responses. The representation of these 14 duodenal taxa in fecal microbiota was significantly different from that in samples obtained from healthy children (P<0.001 by permutational multivariate analysis of variance). Enteropathy of the small intestine developed in gnotobiotic mice that had been colonized with cultured duodenal strains obtained from children with EED. CONCLUSIONS: These results provide support for a causal relationship between growth stunting and components of the small intestinal microbiota and enteropathy and offer a rationale for developing therapies that target these microbial contributions to EED. (Funded by the Bill and Melinda Gates Foundation and others; number, NCT02812615.).

Duodeno/microbiologia , Microbioma Gastrointestinal , Transtornos do Crescimento/microbiologia , Transtornos da Nutrição do Lactente/complicações , Animais , Bactérias/isolamento & purificação , Bangladesh , Duodenoscopia , Duodeno/patologia , Doença Ambiental/complicações , Fezes/microbiologia , Feminino , Vida Livre de Germes , Crescimento , Transtornos do Crescimento/etiologia , Humanos , Lactente , Doenças Inflamatórias Intestinais/complicações , Fator de Crescimento Insulin-Like I/análise , Enteropatias/complicações , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Análise Multivariada , Proteínas Associadas a Pancreatite/análise , Proteoma/análise
Int. j. odontostomatol. (Print) ; 14(2): 141-143, June 2020. graf
Artigo em Inglês | LILACS | ID: biblio-1090665


The aim of the study was to determine the possibility of using panoramic X-ray as an auxiliary tool for the identification of talent in young athletes using the physical body growth indicator. Thirty three radiographs were used of athletes aged 13 to 15 years. Analysis was performed using the Eklof and Ringertz method. Dental age (40 %) came the closest to chronological age. Dental age determined in this study proved to be the most effective radiographic method for the determination of bone maturity for use in the identification of talent through imaging exams. This method was also the simplest and can be performed by the dentist of a sports club without exposing athletes to unnecessary radiation.

El objetivo del estudio consistió en determinar la posibilidad de usar radiografía panorámica como herramienta auxiliar para la identificación de talentos en los atletas usando el indicador de crecimiento del cuerpo físico. Fueron utilizadas 33 radiografías de atletas, de entre 13 a 15 años. Se realizó un análisis de la aplicación utilizando el método de Eklof y Ringertz. Se encontró que la edad dentaria (40 %) se aproximó a la edad cronológica. La edad dental utilizada en este estudio demostró ser el método radiográfico más efectivo para la determinación de la madurez ósea para su uso en la identificación del talento a través de exámenes de imágenes. Este método también fue el más simple y puede ser realizado por el dentista de un club deportivo sin exponer a los atletas a radiaciones innecesarias.

Humanos , Masculino , Adolescente , Aptidão , Futebol , Desenvolvimento Ósseo , Radiografia Panorâmica/métodos , Desempenho Atlético , Projetos Piloto , Crescimento
Cochrane Database Syst Rev ; 5: CD012284, 2020 05 28.
Artigo em Inglês | MEDLINE | ID: mdl-32463488


BACKGROUND: Thalassaemia is a recessively-inherited blood disorder that leads to anaemia of varying severity. In those affected by the more severe forms, regular blood transfusions are required which may lead to iron overload. Accumulated iron from blood transfusions may be deposited in vital organs including the heart, liver and endocrine organs such as the pituitary glands which can affect growth hormone production. Growth hormone deficiency is one of the factors that can lead to short stature, a common complication in people with thalassaemia. Growth hormone replacement therapy has been used in children with thalassaemia who have short stature and growth hormone deficiency. This review on the role of growth hormone was originally published in September 2017 and updated in April 2020. OBJECTIVES: To assess the benefits and safety of growth hormone therapy in people with thalassaemia. SEARCH METHODS: We searched the Cochrane Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. Date of latest search: 14 November 2019. We also searched the reference lists of relevant articles, reviews and clinical trial registries. Date of latest search: 06 January 2020. SELECTION CRITERIA: Randomised and quasi-randomised controlled trials comparing the use of growth hormone therapy to placebo or standard care in people with thalassaemia of any type or severity. DATA COLLECTION AND ANALYSIS: Two authors independently selected trials for inclusion. Data extraction and assessment of risk of bias were also conducted independently by two authors. The certainty of the evidence was assessed using GRADE criteria. MAIN RESULTS: We included one parallel trial conducted in Turkey. The trial recruited 20 children with homozygous beta thalassaemia who had short stature; 10 children received growth hormone therapy administered subcutaneously on a daily basis at a dose of 0.7 IU/kg per week and 10 children received standard care. The overall risk of bias in this trial was low except for the selection criteria and attrition bias which were unclear. The certainty of the evidence for all major outcomes was moderate, the main concern was imprecision of the estimates due to the small sample size leading to wide confidence intervals. Final height (cm) (the review's pre-specified primary outcome) and change in height were not assessed in the included trial. The trial reported no clear difference between groups in height standard deviation (SD) score after one year, mean difference (MD) -0.09 (95% confidence interval (CI) -0.33 to 0.15 (moderate-certainty evidence). However, modest improvements appeared to be observed in the following key outcomes in children receiving growth hormone therapy compared to control (moderate-certainty evidence): change between baseline and final visit in height SD score, MD 0.26 (95% CI 0.13 to 0.39); height velocity, MD 2.28 cm/year (95% CI 1.76 to 2.80); height velocity SD score, MD 3.31 (95% CI 2.43 to 4.19); and change in height velocity SD score between baseline and final visit, MD 3.41 (95% CI 2.45 to 4.37). No adverse effects of treatment were reported in either group; however, while there was no clear difference between groups in the oral glucose tolerance test at one year, fasting blood glucose was significantly higher in the growth hormone therapy group compared to control, although both results were still within the normal range, MD 6.67 mg/dL (95% CI 2.66 to 10.68). There were no data beyond the one-year trial period. AUTHORS' CONCLUSIONS: A small single trial contributed evidence of moderate certainty that the use of growth hormone for a year may improve height velocity of children with thalassaemia although height SD score in the treatment group was similar to the control group. There are no randomised controlled trials in adults or trials that address the use of growth hormone therapy over a longer period and assess its effect on final height and quality of life. The optimal dosage of growth hormone and the ideal time to start this therapy remain uncertain. Large well-designed randomised controlled trials over a longer period with sufficient duration of follow up are needed.

Transtornos do Crescimento/tratamento farmacológico , Crescimento/efeitos dos fármacos , Hormônio do Crescimento Humano/uso terapêutico , Talassemia beta/complicações , Adolescente , Criança , Intervalos de Confiança , Feminino , Crescimento/fisiologia , Transtornos do Crescimento/etiologia , Homozigoto , Humanos , Masculino , Talassemia beta/genética
Georgian Med News ; (299): 47-55, 2020 Feb.
Artigo em Russo | MEDLINE | ID: mdl-32242844


The physical development of children is one of the main criteria for the health status of the child population, reflecting the influence of endogenous and exogenous factors. Dynamic study of schoolchildren's physical development allows one to determine the characteristics of the formations of their morphofunctional parameters and then influence the health of the whole population. The study of the orientation of time shifts in physical development has an important predictive preventive component and is the basis for updating regional standards every 5-10 years. Objective - to identify the main trends in the physical development of schoolchildren in Kazakhstan according to anthropometric measurements among schoolchildren of Almaty over the past 60 years (1956, 1972(2), 1983, 1989, 2005, 2017). Object of study: 13136 schoolchildren of 7-16 years old, various general education institutions (schools) of Almaty, who studied in 1956, in 1972, in 1983, in 1989, in 2005, and in 2017, which were used to carry out transverse and longitudinal studies of physical development using a standardized anthropometric method using standard tools. A comparative analysis of the basic indicators of physical development (length and body weight), conducted between 1956 and 2017, shows a pronounced tendency to increase them across all ages. The largest increase in basic body size in both boys and girls was in the period from 1956 to 1972 (p <0.05). Later, until 2005, stabilization and even slowing down of the observed processes of increasing somatometric indicators was noted. The economic crisis that swept the country in the 1990 led to a significant decrease in the mass-growth indicators in children of both sexes in 2005. The results of a 2017 study indicate a "new round of acceleration" of modern children of Kazakhstan of both sexes. A retrospective study of the physical development of schoolchildren, conducted in Kazakhstan over the past 60 years, showed a pronounced tendency to increase the basic mass and growth indicators, especially in males, and the acceleration of the period of puberty. In modern schoolchildren, there was a change in the timing of the annual "crosses" of growth curves at an earlier age period. For children of Kazakhstan in the new millennium, a decrease in the degree of correlative connections between length and body weight is characteristic, which indicates their disharmonious development.

Crescimento , Maturidade Sexual , Adolescente , Antropometria , Estatura , Índice de Massa Corporal , Peso Corporal , Criança , Feminino , Humanos , Cazaquistão , Masculino , Fenômenos Fisiológicos da Nutrição , Estudos Retrospectivos , Fatores Sexuais
Cochrane Database Syst Rev ; 4: CD010205, 2020 04 08.
Artigo em Inglês | MEDLINE | ID: mdl-32266964


BACKGROUND: Zinc is a vital micronutrient for humans and is essential for protein synthesis, cell growth, and differentiation. Severe zinc deficiency can lead to slower physical, cognitive and sexual growth, cause skin disorders, decrease immunity, increase incidence of acute illnesses in infants and children and contribute to childhood stunting. By estimation, 17.3% of the world population is at risk of inadequate zinc intake. Such nutritional impairment increases the risk of diarrhoea and pneumonia by 20%, as well as leads to a global loss of more than 16 million disability-adjusted life years in children less than five years of age. Not only does zinc deficiency affect lives, it adds to the considerable financial burden on depleted resources in countries that are most affected. By preventing or curing this deficiency, we can improve childhood mortality, morbidity and growth. OBJECTIVES: To assess the effectiveness of zinc supplementation for the promotion of growth, reduction in mortality, and the prevention of infections in infants less than six months of age. SEARCH METHODS: We used the standard search strategy of the Cochrane Neonatal Group to search the Cochrane Central Register of Controlled Trials (CENTRAL 2018, Issue 4), MEDLINE via PubMed (1966 to 18 May 2018), Embase (1980 to 18 May 2018), and CINAHL (1982 to 18 May 2018). We also searched clinical trials databases, conference proceedings, and the reference lists of retrieved articles for randomised controlled trials and quasi-randomised trials. An updated search from 1 January 2018 to 29 January 2020 was run in the following databases: CENTRAL via CRS Web, MEDLINE via Ovid, and CINAHL via EBSCOhost. SELECTION CRITERIA: All randomised controlled (individual and cluster randomised) and quasi-randomised trials of zinc supplementation in healthy, term infants, less than six months of age comparing infant mortality, incidence of diarrhoea or respiratory illnesses, growth and/or serum zinc levels were eligible. DATA COLLECTION AND ANALYSIS: Two review authors screened search results (title and abstracts) and relevant full texts. Studies fulfilling prespecified inclusion criteria were included with any disagreements resolved by consensus. Extraction and analysis were then conducted. We used the GRADE approach to assess the quality of evidence as indicated by certainty in effect estimates. MAIN RESULTS: Eight studies (with 85,629 infants) were included and five studies were meta-analysed, out of which four studies compared zinc with placebo, and one compared zinc plus riboflavin versus riboflavin. Certain growth outcomes after six months of intervention (Weight for Age Z-scores (WAZ) (standardised mean difference) (SMD) 0.16, 95% CI 0.03 to 0.29; three studies, n = 955; fixed-effect; heterogeneity Chi² P = 0.96); I² = 0%); change in WAZ (SMD 0.16, 95% CI 0.07 to 0.25; one study, n = 386; fixed-effect); (Weight-for-Length Z-score (WLZ) (SMD 0.15, 95% CI 0.02 to 0.28; three studies, n = 955; fixed-effect; heterogeneity: Chi² P = 0.81); I² = 0%); (change in WLZ (SMD 0.17, 95% CI 0.06 to 0.28; one study, n = 386; fixed-effect)) were positively affected by zinc supplementation compared to placebo. A single study reported no difference in the incidence of diarrhoea and lower respiratory tract infection with zinc supplementation. Zinc had no effect on mortality in children younger than 12 months. When zinc plus riboflavin was compared to riboflavin only, significant improvement was observed in the incidence of wasting at 24 months (risk ratio (RR) 0.59, 95% CI 0.37 to 0.96; one study, n = 296; fixed-effect), but significant worsening of incidence of stunting was present at 21 months (RR 1.53, 95% CI 1.09 to 2.16; one study, n = 298; fixed-effect). AUTHORS' CONCLUSIONS: There was a significant positive impact of zinc supplementation on WAZ and WLZ after six months of intervention in infants compared to placebo. When a combined supplement of zinc and riboflavin was compared to riboflavin, there was a significant reduction in wasting at 24 months, but stunting at 21 months was negatively affected. Although included trials were of good-to-moderate quality, evidence that could be meta-analysed was based on a few studies which affected the overall quality of results. Regardless, there is a need for strong trials conducted in infants younger than six months before a strong recommendation can be made supporting zinc supplementation in this age group.

Crescimento , Controle de Infecções/métodos , Oligoelementos/administração & dosagem , Zinco/administração & dosagem , Zinco/deficiência , Peso Corporal , Humanos , Lactente , Mortalidade Infantil , Recém-Nascido , Ensaios Clínicos Controlados Aleatórios como Assunto , Riboflavina/administração & dosagem , Complexo Vitamínico B/administração & dosagem , Síndrome de Emaciação/prevenção & controle
Cochrane Database Syst Rev ; 4: CD013376, 2020 04 09.
Artigo em Inglês | MEDLINE | ID: mdl-32270495


BACKGROUND: Aflatoxins are carcinogenic mycotoxins that contaminate many food crops. Maize and groundnuts are prone to aflatoxin contamination, and are the major sources of human exposure to aflatoxins, due to their high intake as staple foods, particularly in low- and middle-income countries (LMICs). Observational studies suggest an association between dietary exposure to aflatoxins during pregnancy and early childhood and linear growth in infants and young children. OBJECTIVES: To assess the effects on pre- and postnatal growth outcomes when agricultural and nutritional education interventions during the post-harvest period that aim to reduce aflatoxin exposure are compared to usual support or no intervention. We assessed this in infants, children, and pregnant and lactating women at the household or community level in LMICs. SEARCH METHODS: In July and August 2019, we searched: CENTRAL, MEDLINE, Embase, CINAHL, Web of Science Core Collection, Africa-Wide, LILACS, CAB Abstracts, Agricola, and two trials registers. We also checked the bibliographies of the included studies and contacted relevant mycotoxin organisations and researchers for additional studies. SELECTION CRITERIA: We included randomised controlled trials (RCTs) and cluster-RCTs of agricultural education and nutritional education interventions of any duration, at the household or community level, aimed at reducing aflatoxin intake by infants, children, and pregnant and lactating women, in LMICs during the post-harvest period, compared to no intervention or usual support. We excluded studies that followed participants for less than four weeks. We assessed prespecified prenatal (at birth) and postnatal growth outcomes (during infancy, childhood, and adolescence), with linear growth (as the primary outcome), infectious disease morbidity, and unintended consequences. DATA COLLECTION AND ANALYSIS: Two authors independently assessed study eligibility using prespecified criteria, extracted data, and assessed risk of bias of included RCTs. We evaluated the certainty of the evidence using GRADE, and presented the main results in a 'Summary of findings' table. MAIN RESULTS: We included three recent cluster-RCTs reporting the effects of agricultural education plus post-harvest technologies, compared to usual agricultural support or no intervention. The participants were pregnant women and their children, lactating women and their infants (< 6 months), women of childbearing age, and young children (< 59 months), from rural, subsistence maize-farming communities in Kenya, Zimbabwe, and Tanzania. Two trials randomised villages to the intervention and control groups, including a total of at least 979 mother-child pairs from 60 villages. The third trial randomised 420 households, including 189 mother-child pairs and 231 women of childbearing age. Duration of the intervention and follow-up ranged between five and nine months. Due to risk of attrition bias, the overall risk of bias was unclear in one trial, and high in the other two trials. None of the included studies addressed the effects of nutritional education on pre- and postnatal growth. One trial reported outcomes not prespecified in our review, and we were unable to obtain unpublished growth data from the second trial, even after contacting the authors. The third trial, in lactating women and their infants in Tanzania, reported on the infants' weight-for-age z-score (WAZ) after six months. This trial found that providing agricultural education aimed at changing farmers' post-harvest practices to reduce aflatoxin exposure, by using demonstrations (e.g. handsorting, de-hulling of maize, drying sheets, and insecticides), may improve WAZ in infants from these farmers' households, on average, by 0.57 (95% confidence interval (CI) 0.16 to 0.98; 1 study; 249 participants; very low-certainty evidence), compared to infants from households where the farmers received routine agricultural extension services. Another way of reporting the effect on WAZ is to compare the proportion of underweight infants (WAZ > 2 SD below the reference median value) per group. This trial found that the intervention may reduce the proportion of underweight infants in the intervention households by 6.7% (95% CI -12.6 to -1.4; 249 participants; very low-certainty evidence) compared to control households. No studies reported on unintended effects of agricultural and nutritional education. AUTHORS' CONCLUSIONS: Evidence on the effects on child growth in LMICs of agricultural or nutritional education interventions that reduce aflatoxin exposure was very limited; no included study reported on linear growth. Very low-certainty evidence suggested that agricultural education aimed at changing farmers' post-harvest practices to reduce aflatoxin exposure by using demonstrations, may result in an increase in WAZ, when compared to usual or no education.

Aflatoxinas/envenenamento , Agricultura/educação , Países em Desenvolvimento , Contaminação de Alimentos/prevenção & controle , Crescimento , Adulto , Agricultura/métodos , Aleitamento Materno , Pré-Escolar , Feminino , Humanos , Lactente , Quênia , Gravidez , Efeitos Tardios da Exposição Pré-Natal/prevenção & controle , Ensaios Clínicos Controlados Aleatórios como Assunto , Tanzânia , Magreza/prevenção & controle , Zimbábue
Arch. argent. pediatr ; 118(2): 117-: I-124, IV, abr. 2020. tab, ilus
Artigo em Inglês, Espanhol | LILACS, BINACIS | ID: biblio-1100161


Introducción. La evaluación del crecimiento durante la infancia y la adolescencia es un componente fundamental de la atención de salud en todos sus niveles, pues es parte del diagnóstico nutricional y permite la detección oportuna de patologías relacionadas. Ecuador, al no disponer de referencias nacionales de crecimiento, ha adoptado los estándares internacionales de la Organización Mundial de la Salud. El objetivo de este estudio fue construir referencias nacionales de peso, estatura e índice de masa corporal para niños, niñas y adolescentes.Métodos. Se investigaron escolares y adolescentes ecuatorianos entre 5 y 19 años de edad durante 1999 y 2012. Los centilos 3, 10, 25, 50, 75, 90 y 97 de peso, talla e índice de masa corporal fueron estimados por el método LMS para datos transversales, que utiliza la transformación Box-Cox para normalizar la distribución de los datos a cada edad.Resultados. Participaron 5934 sujetos sanos (2788 niños y 3146 niñas). Los niños pesaban más y eran más altos que las niñas. En todos los casos, los valores mostraron un aumento creciente conforme la edad avanzaba. A los 18 años, las diferencias entre sexos promediaron 8 kg y 12,5 cm.Conclusión. Las tablas y curvas producto de este estudio constituyen la primera referencia descriptiva del crecimiento de niños ecuatorianos de 5-19 años. Son un importante instrumento de evaluación nutricional. Su implementación en la atención primaria de salud permitirá complementar el diagnóstico nutricional que, tradicionalmente, se realiza sobre la base de los estándares internacionales de la Organización Mundial de la Salud.

Introduction. The assessment of growth during childhood and adolescence is a critical component of health care at all levels, but it is also part of nutritional status diagnosis and the timely detection of related conditions. Ecuador lacks national growth references, so it has decided to adopt the international standards proposed by the World Health Organization. The objective of this study was to develop national references for weight, height, and body mass index for children and adolescents.Methods. Ecuadorian schoolchildren and adolescents aged 5-19 years were studied between 1999 and 2012. The LMS method for cross-sectional data, which uses the Box-Cox transformation to normalize data distribution at each age, was applied to estimate the 3rd, 10th, 25th, 50th, 75th, 90th, and 97th centiles for weight, height, and body mass index.Results. A total of 5934 healthy subjects (2788 boys and 3146 girls) participated. Boys were heavier and taller than girls. In all cases, values increased with age. At 18 years old, the differences between sexes averaged 8 kg and 12.5 cm.Conclusion. The tables and curves obtained with this study are the first descriptive growth references for Ecuadorian children and adolescents aged 5-19 years. They are relevant for nutritional assessment. Their use at the primary level of care will aid in nutritional status diagnosis, which has traditionally been done based on the World Health Organization's international standards.

Humanos , Masculino , Feminino , Criança , Adolescente , Estatura , Peso Corporal , Índice de Massa Corporal , Crescimento , Epidemiologia Descritiva , Estudos Transversais , Equador , Gráficos de Crescimento
J Nutr ; 150(6): 1405-1412, 2020 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-32211798


BACKGROUND: Milk intake stimulates linear growth and improves cognition in children from low-income countries. These effects may be mediated through insulin-like growth factor-1 (IGF-1). OBJECTIVE: The objective was to assess the effect of milk supplement on circulating IGF-1 and to assess IGF-1 as a correlate of growth and cognition in children. METHODS: Secondary data on blood spot IGF-1 from a randomized, double-blind, controlled trial in 6-9-y-old children from rural Ghana were analyzed. Intervention groups received porridge with non-energy-balanced supplements: 8.8 g milk protein/d, 100 kcal/d (Milk8); 4.4 g milk and 4.4 g rice protein/d, 100 kcal/d (Milk/rice); 4.4 g milk protein/d, 48 kcal/d (Milk4); or a control (no protein, 10 kcal/d). IGF-1, length, body composition, and Cambridge Neuropsychological Test Automated Battery (CANTAB) were measured at 3.5 or 8.5 mo. Linear regressions were used to assess the effect of milk interventions on IGF-1 and IGF-1 as a correlate of growth and cognition. RESULTS: The increase in IGF-1 was 15.3 (95% CI: 3.3, 27.3) ng/mL higher in children receiving Milk8 compared with the control. The IGF-1 increases in the isonitrogenous, isoenergetic Milk/rice or the Milk4 groups were not different from the control (P ≥ 0.49). The increase in IGF-1 was associated with improvements in 4 out of 5 CANTAB domains. The strongest associations included reductions in "mean correct latency" from Pattern Recognition Memory and "pre-extradimensional (pre-ED) shift errors" from Intra/Extradimensional Set Shift (P ≤ 0.005). In addition, change in IGF-1 was positively associated with changes in height, weight, and fat-free mass (P ≤ 0.001). CONCLUSIONS: Intake of skimmed milk powder corresponding to one, but not half a glass of milk on school days stimulates IGF-1 in 6-9-y-old Ghanian children. IGF-1 seems to mediate the effect of milk intake on growth and cognition. The association between IGF-1 and cognition in relation to milk intake is novel and opens possibilities for dietary interventions to improve cognition.

Cognição , Crescimento , Fator de Crescimento Insulin-Like I/metabolismo , Leite , Aminoácidos/análise , Animais , Composição Corporal , Criança , Suplementos Nutricionais , Método Duplo-Cego , Teste em Amostras de Sangue Seco , Feminino , Gana , Humanos , Masculino , Proteínas do Leite/química , Proteínas do Leite/metabolismo , População Rural
Toxicol Appl Pharmacol ; 394: 114957, 2020 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-32173372


The adverse effects of triphenyltin (TPT) on aquatic systems have attracted much attention because TPT is widely used and prevalent in aquatic environments. Here, zebrafish embryos/larvae were exposed to TPT (0, 0.039, 0.39, and 3.9 nM; 0, 15, 150 and 1500 ng/L) for 7 or 14 days to determine its toxic effects on the hypothalamic-pituitary-thyroid (HPT) axis. The results showed that whole-body total T4 and T3 levels were significantly decreased, which was accompanied by the significant upregulation of the expression of the dio1, dio2 and ugt1ab genes after exposure to TPT for 7 and 14 days. Genes related to thyroid hormone synthesis (crh, tshß, nis, tpo and tg) were upregulated at both 7 and 14 days after TPT exposure. This might have been due to the positive feedback regulation of the HPT axis, which is caused by a decrease in thyroid hormone in the whole body in zebrafish. In addition, the survival rates and body lengths were reduced after treatment with TPT for 7 and 14 days. This indicated that TPT caused adverse effect on the development of zebrafish embryos/larvae. In summary, the results suggested that TPT caused thyroid disruption and developmental toxicity in zebrafish larvae.

Desenvolvimento Embrionário/efeitos dos fármacos , Larva/efeitos dos fármacos , Compostos Orgânicos de Estanho/toxicidade , Doenças da Glândula Tireoide/induzido quimicamente , Peixe-Zebra , Animais , Disruptores Endócrinos/toxicidade , Regulação da Expressão Gênica no Desenvolvimento/efeitos dos fármacos , Crescimento/efeitos dos fármacos , Sistema Hipotálamo-Hipofisário/efeitos dos fármacos , Metamorfose Biológica/efeitos dos fármacos , Sistema Hipófise-Suprarrenal/efeitos dos fármacos , Análise de Sobrevida , Glândula Tireoide/efeitos dos fármacos , Tiroxina/metabolismo , Tri-Iodotironina/metabolismo , Poluentes Químicos da Água/toxicidade
Biosci. j. (Online) ; 36(2): 527-538, 01-03-2020. tab
Artigo em Inglês | LILACS | ID: biblio-1146414


In order to evaluate the performance of Nile tilapia (Oreochromis niloticus), daily feeding amounts were varied in an experiment following a completely randomized design for 154 days and consisting of four treatments with three replications each, totaling 12 tanks with 3 m3 (3 x 1 x 1 m) of area each at densities of 5 fish m-3 (initial) and 3 fish m-3 until the end of the experiment. Throughout the experimental period, the daily amount of feed was varied and delimited for each feeding time (8h00, 11h00, 14h00 and 17h00) as: 25; 25; 25 and 25% in daily treatment 1 - T1; 20; 30; 30 and 20% in treatment 2 - T2; 30; 20; 20 and 30% in daily treatment 3 - T3; and 20; 30; 20 and 30% in treatment 4 - T4. Feed was offered six days a week, with extruded isocaloric (1,412 KJ) and isoproteic (32% crude protein) diets from 2 to 4 mm. Every two weeks, a sample containing 30% of stocked individuals was collected in each replication to monitor fish growth. Nile tilapia (O. niloticus) fed in daily amounts of 20; 30; 20 and 30% showed a better growth performance in the final of experiment, mainly on biomass, final average weight and feed conversion.

Para avaliar o desempenho da tilápia do Nilo (Oreochromis niloticus), as quantidades diárias de alimentação foram variadas em um experimento após um delineamento inteiramente casualizado de 154 dias e consistindo em quatro tratamentos com três repetições cada, totalizando 12 tanques com 3 m3 (3 x 1 x 1 m) de área cada uma em densidades de 5 peixes m-3 (inicial) e 3 peixes m-3 até o final do experimento. Ao longo do período experimental, a quantidade diária de alimentos foi variada e delimitada para cada tempo de alimentação (8h00, 11h00, 14h00 e 17h00) como: 25; 25; 25 e 25% no tratamento diário 1 - T1; 20; 30; 30 e 20% no tratamento 2 - T2; 30; 20; 20 e 30% no tratamento diário 3 - T3; e 20; 30; 20 e 30% no tratamento 4 - T4. O alimento foi oferecido seis dias por semana, com dietas isocalóricas extrudadas (1.412 KJ) e isoprotéicas (32% de proteínas brutas) de 2 a 4 mm. A cada duas semanas, uma amostra contendo 30% de indivíduos estocados foi coletada em cada replicação para monitorar o crescimento do peixe. Tilápias do Nilo (O. niloticus) alimentadas em quantidades diárias de 20; 30; 20 e 30% mostraram um melhor desempenho de crescimento no final do experimento, principalmente na biomassa, peso médio final e conversão alimentar.

Aquicultura , Ciclídeos , Dieta , Tanques , Pesqueiros , Peixes , Crescimento
Biosci. j. (Online) ; 36(2): 519-526, 01-03-2020. tab
Artigo em Inglês | LILACS | ID: biblio-1146412


The aim of research was to evaluate the performance and characteristics of the carcasses of Nellore young bulls in grazing supplemented with or without addition of lipids. Twenty­eight young bulls, 301 ± 5.8 kg in body weight, which four constituted the reference group and the twenty-four remnants were used in the experiment and randomly distributed into four groups according to treatment: only Panicum maximum cv. Mombasa grass; Mombasa grass with concentrated supplementation based on soybean meal; Mombasa grass with concentrate containing lipids from soybean oil; and Mombasa grass with concentrate containing lipids derived from soybean grains. The total dry matter intake of the animals fed only Mombasa grass did not differ from the average intake of the three treatments supplemented with concentrate. There were no differences (P>0.05) between Mombasa grass and diets with concentrate, supplementation with or without lipid and lipids sources in the carcass traits, gastrointestinal tract, organs and loin eye area. Nellore bulls receiving concentrate increased subcutaneous fat thickness compared with bulls that received only Mombasa grass. Nellore young bulls fed Mombasa grass only, supplemented with or without addition of lipids were similar regarding growth performance and carcass traits

Objetivou-se com esta pesquisa avaliar o desempenho e as características das carcaças de novilhos Nelore em pastejo suplementados com ou sem adição de lipídios. Vinte e oito novilhos, 301 ± 5,8 kg de peso vivo, sendo que quatro constituíram o grupo de referência e os vinte e quatro remanescentes foram utilizados no experimento e distribuídos aleatoriamente em quatro grupos de acordo com o tratamento: somente Panicum maximum cv. capim-mombaça; capim-mombaça com suplementação concentrada à base de farelo de soja; capim-mombaça com concentrado contendo lipídios do óleo de soja; e capim-mombaça com concentradocontendo lipídios derivados de grãos de soja. O consumo total de matéria seca dos animais alimentados apenas com capim-mombaça não diferiu da ingestão média dos três tratamentos suplementados com concentrado. Não houve diferenças (P>0,05) entre capim-mombaça e dietas com concentrado, suplementação com ou sem lipídeos e fontes de lipídeos nas características de carcaça, trato gastrintestinal, órgãos e área de olho de lombo. Os novilhos Nelore que receberam concentrado aumentaram a espessura de gordura em comparação aos novilhos que receberam apenas capim-mombaça. Novilhos Nelore criados em capim-mombaça, alimentados apenas com capim-mombaça ou suplementados com ou sem adição de lipídeos, foram semelhantes quanto ao desempenho de crescimento e características de carcaça.

Bovinos , Dieta , Lipídeos , Soja , Óleo de Soja , Brachiaria , Poaceae , Crescimento , Panicum
J Pediatr ; 219: 98-105.e4, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32059815


OBJECTIVE: To compare growth in children with intestinal failure-associated liver disease (IFALD) who received a fish oil intravenous lipid emulsion (FOLE) to those who received a soybean oil intravenous lipid emulsion (SOLE). STUDY DESIGN: This multisite, retrospective study pair-matched FOLE (n = 82) to SOLE recipients (n = 41) using baseline serum direct bilirubin levels and postmenstrual age. Study subjects received open-label FOLE (1 g/kg/day) until IFALD resolved or parenteral nutrition was stopped. Historical control subjects received SOLE (up to 3 g/kg/day). Growth measures (changes in body weight, height/length, and head circumference), prealbumin, triglycerides, and glucose were compared between groups over time using the Wilcoxon rank-sum test. RESULTS: Although changes in all of the growth measures were similar for both groups (P > .05), FOLE recipients demonstrated an overall improved growth trajectory. After 28 weeks, FOLE recipients had a mean body weight within a z score range of -1 to 1 indicating age-appropriate growth. FOLE recipients consistently had higher prealbumin, lower triglyceride, and more normal glucose concentrations over time compared with SOLE recipients. CONCLUSIONS: Children with IFALD who received FOLE had similar growth and fewer metabolic abnormalities compared with those who received SOLE. TRIAL REGISTRATION: NCT00910104 and NCT00738101.

Óleos de Peixe/administração & dosagem , Crescimento/efeitos dos fármacos , Enteropatias/terapia , Hepatopatias/terapia , Nutrição Parenteral/métodos , Estudos de Casos e Controles , Pré-Escolar , Ingestão de Energia , Emulsões Gordurosas Intravenosas , Ácidos Graxos , Feminino , Humanos , Masculino , Estudos Retrospectivos
N Engl J Med ; 382(9): 835-844, 2020 02 27.
Artigo em Inglês | MEDLINE | ID: mdl-32101665


Mutations in VHL, which encodes von Hippel-Lindau tumor suppressor (VHL), are associated with divergent diseases. We describe a patient with marked erythrocytosis and prominent mitochondrial alterations associated with a severe germline VHL deficiency due to homozygosity for a novel synonymous mutation (c.222C→A, p.V74V). The condition is characterized by early systemic onset and differs from Chuvash polycythemia (c.598C→T) in that it is associated with a strongly reduced growth rate, persistent hypoglycemia, and limited exercise capacity. We report changes in gene expression that reprogram carbohydrate and lipid metabolism, impair muscle mitochondrial respiratory function, and uncouple oxygen consumption from ATP production. Moreover, we identified unusual intermitochondrial connecting ducts. Our findings add unexpected information on the importance of the VHL-hypoxia-inducible factor (HIF) axis to human phenotypes. (Funded by Associazione Italiana Ricerca sul Cancro and others.).

Mutação em Linhagem Germinativa , Transtornos do Crescimento/genética , Hipoglicemia/genética , Fator 1 Induzível por Hipóxia/deficiência , Mitocôndrias/metabolismo , Proteína Supressora de Tumor Von Hippel-Lindau/genética , Expressão Gênica , Crescimento/genética , Humanos , Masculino , Metaboloma/genética , Metaboloma/fisiologia , Síndrome , Adulto Jovem