RESUMEN
BACKGROUND: There appears little consensus concerning protein requirements in phenylketonuria (PKU). METHODS: A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. RESULTS: The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). CONCLUSIONS: The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population.
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Aminoácidos/administración & dosificación , Caseínas/administración & dosificación , Proteínas en la Dieta/administración & dosificación , Suplementos Dietéticos , Fragmentos de Péptidos/administración & dosificación , Fenilcetonurias/dietoterapia , Adulto , Niño , Preescolar , Europa (Continente) , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Fenilalanina , Encuestas y Cuestionarios , Turquía , Organización Mundial de la SaludRESUMEN
BACKGROUND: There is no published data comparing dietary management of urea cycle disorders (UCD) in different countries. METHODS: Cross-sectional data from 41 European Inherited Metabolic Disorder (IMD) centres (17 UK, 6 France, 5 Germany, 4 Belgium, 4 Portugal, 2 Netherlands, 1 Denmark, 1 Italy, 1 Sweden) was collected by questionnaire describing management of patients with UCD on prescribed protein restricted diets. RESULTS: Data for 464 patients: N-acetylglutamate synthase (NAGS) deficiency, n=10; carbamoyl phosphate synthetase (CPS1) deficiency, n=29; ornithine transcarbamoylase (OTC) deficiency, n=214; citrullinaemia, n=108; argininosuccinic aciduria (ASA), n=80; arginase deficiency, n=23 was reported. The majority of patients (70%; n=327) were aged 0-16y and 30% (n=137) >16y. Prescribed median protein intake/kg body weight decreased with age with little variation between disorders. The UK tended to give more total protein than other European countries particularly in infancy. Supplements of essential amino acids (EAA) were prescribed for 38% [n=174] of the patients overall, but were given more commonly in arginase deficiency (74%), CPS (48%) and citrullinaemia (46%). Patients in Germany (64%), Portugal (67%) and Sweden (100%) were the most frequent users of EAA. Only 18% [n=84] of patients were prescribed tube feeds, most commonly for CPS (41%); and 21% [n=97] were prescribed oral energy supplements. CONCLUSIONS: Dietary treatment for UCD varies significantly between different conditions, and between and within European IMD centres. Further studies examining the outcome of treatment compared with the type of dietary therapy and nutritional support received are required.
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Aminoácidos Esenciales/metabolismo , Dieta con Restricción de Proteínas , Trastornos Innatos del Ciclo de la Urea/dietoterapia , Trastornos Innatos del Ciclo de la Urea/patología , Adolescente , Adulto , N-Acetiltransferasa de Aminoácidos/deficiencia , Arginasa/metabolismo , Aciduria Argininosuccínica/dietoterapia , Ligasas de Carbono-Nitrógeno con Glutamina como Donante de Amida-N/deficiencia , Niño , Preescolar , Citrulinemia/dietoterapia , Europa (Continente) , Humanos , Lactante , Recién Nacido , Ornitina Carbamoiltransferasa/metabolismo , Encuestas y Cuestionarios , Resultado del Tratamiento , Trastornos Innatos del Ciclo de la Urea/enzimologíaRESUMEN
The Alpine area is particularly sensitive to climatic and environmental changes that might impact socio-ecosystems and modify the regime of natural hazards. Among them, wildfire is of major importance as it threatens both ecosystems and human lives and infrastructures. Wildfires result from complex interactions between available vegetation fuels, climate and weather, and humans who decide of the land use and are the main source of fire ignitions. The changes in fire weather during the past decades are rather unknown in the French Alps especially due to their complex topography. Moreover, local institutions and managers wonder if the ongoing climate changes might increase fire risk and affect the environmental quality and the different ecosystem services provided by the mountain forests. In this context, we used the national forest fires database together with daily meteorological observations from 1959 to 2015 to investigate the changes in wildfire danger in the French Alps. We analysed the spatial and temporal variations in terms of intensity, frequency, seasonality and window of opportunity of two fire weather indices: the fine fuel moisture code (FFMC) and the fire weather index (FWI) that measure the daily water content of vegetation and the potential intensity of fires, respectively. Our results showed a major contrast between Southern Alps with a high fire weather danger on average and a significant increase in the past decades, and Northern Alps with low to moderate danger on average that increased only at low elevations. This study contributes to the understanding of the consequences of ongoings climate changes on wildfires in the French Alps. It produced high resolution results that account for the topographic and climatic variability of the area. Finally, the maps of the different fire weather components have practical implications for fire management and modelling and for preventing indirect effects of fires on ecosystems and human assets.
RESUMEN
BACKGROUND: Acute pyelonephritis can induce parenchymal scarring. The aim of this study was to evaluate the usefulness of procalcitonin (PCT) to predict renal involvement in febrile children with urinary tract infection (UTI). METHODS: In a prospective study serum PCT was measured and compared with others commonly used inflammatory markers in children admitted to the emergency unit with acute pyelonephritis. Renal parenchymal involvement was assessed by a (99 m)Tc-labeled dimercaptosuccinic acid (DMSA) renal scar performed in the first 3 days after the admission. RESULTS: Among 42 enrolled patients, 19 (45%) had acute renal involvement (Group A) ; 23 (55%) (Group B) had normal DMSA scan (n = 16), or old scarring (n = 4) or various anomalies related to uropathy (n = 3). In group A, the mean PCT level was significantly higher than in the group B (5.4 ng/ml, vs 0.4 ng /ml, p < 10(-5)). In these 2 groups, mean C reactive protein (CRP) levels were 99.1 mg/l and 44.6 mg/l respectively (p < 0.001). For a level of serum PCT > or = 0.5 ng/ml, the sensitivity and specificity to predict the renal involvement were 100% and 87% respectively; for a level> or= 20 mg/l CRP had a sensitivity of 94% but a specificity of 30%. CONCLUSION: Serum PCT levels were significantly increased in febrile children with UTI when acute renal parenchymal involvement was present. PCT seems a better marker than CRP for the prediction of patients at risk of renal lesions.
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Calcitonina/sangre , Precursores de Proteínas/sangre , Pielonefritis/sangre , Pielonefritis/diagnóstico , Algoritmos , Biomarcadores/sangre , Proteína C-Reactiva/metabolismo , Péptido Relacionado con Gen de Calcitonina , Niño , Preescolar , Femenino , Fiebre/etiología , Francia , Humanos , Lactante , Recién Nacido , Masculino , Reacción en Cadena de la Polimerasa/métodos , Estudios Prospectivos , Pielonefritis/complicaciones , Pielonefritis/diagnóstico por imagen , Pielonefritis/patología , Cintigrafía , Radiofármacos , Sensibilidad y Especificidad , Ácido Dimercaptosuccínico de Tecnecio Tc 99mRESUMEN
OBJECTIVES: We had for aim to determine the characteristics of carbapenemase-producing enterobacteria (CPE) carriers and to assess the economic impact of isolation measures leading to loss of activity (closed beds, prolonged hospital stays) and additional personnel hours. PATIENTS AND METHODS: We conducted a retrospective study for 2years (2012/2013), in a French general hospital, focusing on CPE carriers with clinical case description. The costs were estimated by comparing the activity of concerned units (excluding the ICU) during periods with CPE carriers or contacts, during the same periods of the year (n-1), plus additional hours and rectal swabs. RESULTS: Sixteen EPC carriers were identified: 10 men and 6 women, 65±10years of age. Seven patients acquired EPC in hospital during 2 outbreaks in 2012. Four patients presented with an infection (peritonitis, catheter infection, and 2 cases of obstructive pyelonephritis) with a favorable outcome. The median length of stay was 21days [4,150]. Six patients died, 1 death was indirectly due to CPE because of inappropriate empiric antibiotic therapy. A decrease in activity was observed compared to the previous year with an estimated 547,303 loss. The 1779 additional hours cost 63,870, and 716 screening samples cost 30,931. The total additional cost was estimated at 642,104 for the institution. CONCLUSIONS: Specialized teams for CPE carriers and isolation of contact patients, required to avoid/control epidemics, have an important additional cost. An appreciation of their support is needed, as well as participation of rehabilitation units.
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Proteínas Bacterianas/análisis , Portador Sano , Infecciones por Enterobacteriaceae/epidemiología , Enterobacteriaceae/enzimología , Costos de Hospital/estadística & datos numéricos , Hospitales Generales/estadística & datos numéricos , Resistencia betalactámica , beta-Lactamasas/análisis , Anciano , Carbapenémicos/farmacología , Portador Sano/economía , Portador Sano/epidemiología , Infección Hospitalaria/economía , Infección Hospitalaria/epidemiología , Infección Hospitalaria/microbiología , Brotes de Enfermedades/economía , Enterobacteriaceae/genética , Enterobacteriaceae/aislamiento & purificación , Infecciones por Enterobacteriaceae/economía , Infecciones por Enterobacteriaceae/microbiología , Femenino , Francia/epidemiología , Unidades Hospitalarias/economía , Hospitales Generales/economía , Humanos , Control de Infecciones/economía , Unidades de Cuidados Intensivos/economía , Infecciones por Klebsiella/economía , Infecciones por Klebsiella/epidemiología , Infecciones por Klebsiella/microbiología , Klebsiella pneumoniae/enzimología , Tiempo de Internación/estadística & datos numéricos , Masculino , Persona de Mediana Edad , Aislamiento de Pacientes/economía , Personal de Hospital/economía , Estudios RetrospectivosRESUMEN
OBJECTIVES: The aim of this study was to evaluate our practices regarding the use of supplementation for breast-fed neonates. METHODS: A descriptive and prospective study conducted between 22/05/2010 and 23/03/2010 comprising breast-fed, healthy, singleton infants at the Maternity University Hospital of Tours. Indications for supplementation were collected prospectively by paramedics. RESULTS: The study included 281 breast-fed neonates, of whom 99 (35 %) received supplementation. Supplemented neonates were more often children of primiparae (61.6 % versus 44%; P=0.005), or born to mothers without experience of breast-feeding (69.7% versus 48.9%; P=0.001), born by cesarean section (21.2% versus 10.4%; P=0.01), or were small for gestational age (10.1 % versus 6.6%; P=0.003). The main indications were: to prevent additional weight loss, hunger of the newborn, hypoglycemia, and difficulty to breast-feed. Twenty-nine percent of the neonates were given supplements without medical indication. CONCLUSION: One third of breast-fed infants receive supplementation, not always medically justified. A better understanding of medical indications would avoid supplementation being given to breast-fed infants.
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Lactancia Materna , Fórmulas Infantiles/estadística & datos numéricos , Adulto , Cesárea , Femenino , Humanos , Hambre , Hipoglucemia/prevención & control , Recién Nacido , Recién Nacido Pequeño para la Edad Gestacional , Masculino , Paridad , Embarazo , Estudios Prospectivos , Pérdida de PesoRESUMEN
A satellite DNA family (APSU) was isolated and characterized in the ant Aphaenogaster subterranea. This satellite DNA is organized in tandem repeats of 162 bp and is relatively AT rich (51.9%). Sequence analysis showed a high level of homogeneity between monomers. Loss of satellite DNA has been detected in queens in relation to workers, because the amount of satellite DNA in queens is about 25% of the amount found in workers. Restriction analysis of the total DNA with methylation-sensitive enzymes suggests that this DNA is not methylated. Analysis of the electrophoretic mobility of satellite DNA on non-denaturing polyacrylamide showed that this satellite DNA is only very lightly curved. Their possible transcription was analyzed using reverse transcription and polymerase chain reaction (RT-PCR). The satellite DNA is transcribed on the two DNA strands at the same level in worker and queen pupae, as well as in worker adults.