Congenital muscular dystrophy: a clinical report on 17 patients

We concur with the idea that congenital muscular dystrophy (CMD) is a distinct clinical entity, and report 17 patients (2 negroes and 15 whites; 12M and 5 F; median age 6 years, range 1 to 24 years) with genetic, clinical, laboratorial, electrophysiological and histochemical studies. All our cases have an inheritance compatible with an autosomal recessive pattern. A decrease in fetal movements was reported by 57% of the mothers, generalized hypotonia at birth was present in 82%, limb girdle and neck weakness, absent or decreased deep tendon reflexes, and limb contractures were present in all. Severe muscular wasting was found in 41%. Calf pseudo-hypertrophy was observed in one patient. A patient was severely mentally retarded and another was borderline. During a 30-month follow-up, the muscle weakness of the majority remained essentially unchanged but the degree of motor activity deteriorated and was proportional to the worsening of the limb contractures. Serum CK levels were normal or increased to a maximum of 8 times. The electromyogram was myopathic in 74%, neurogenic in 13% and normal in 13%. CT scans showed a symmetrical white matter hipodensity in the hemispheres in 8 cases. All but 5 patients were operated upon to release the limb contractures and all were submitted to physical therapy. The contractures recurred in 4 patients submitted to surgery and were probably related to the cessation of physical therapy.Descrevemos 17 pacientes (12m, 5f) com idades que variaram de 1 a 24 anos (mediana 6 anos) com distrofia muscular congênita (DMC), que foram estudados do ponto de vista genético, clínico, laboratorial, eletrofisiológico e anátomo-patológico. A apresentação segundo a herança foi da forma esporádica (76,5%) ou possivelmente autossômica recessiva (23,5%). A diminuição da movimentação fetal intra-uterina foi referida em 57% dos casos, hipotonia neonatal em 82% e retardo no desenvolvimento motor em 88,2%. Fraqueza muscular, diminuição dos reflexos profundos e contraturas articulares estavam presentes em todos os casos. A piora na função motora estava muito relacionada ao aumento ou aparecimento de novas retrações articulares. A CK nunca ultrapassou valores acima de 8 vezes o normal. O ENMG foi de padrão miopático em 73,3%, neuropático em 13,3% e normal em 13,3% dos casos. Aspectos tomográficos com hipodensidade da substância branca subcortical foram vistos em 8 casos. Ao tratamento impôs-se fisioterapia adequada e cirurgia corretiva das deformidades articulares. Novas contraturas desenvolveram-se mais tarde e estavam relacionadas freqüentemente a fisioterapia insuficiente.Escola Paulista de MedicinaUNIFESP, EPMSciEL

Biologische bestrijding trips in de bloemisterij: gedagsbeinvloedende stoffen en roofmijten voor de beheersing van trips in de bloemisterij onder glas

Californische trips(Frankliniella occidentalis) is een belangrijke plaag in de glastuinbouw. In 2000 is een overkoepelend tripsproject gestart, waarin onderzoek van PPO Glastuinbouw en praktijkproeven van DLV Gewasbescherming op elkaar werden afgestemd. Dit resulteerde in onderzoek door PPO aan effectiviteit van bodemroofmijten en gedragsbeinvloedende stoffen. Uit de experimenten gedaan in het bodemroofmijtenonderzoek op de gewassen Impatiens, Petunia, Kalanchoe, Potchrysant, Saintpaulia en Yucca blijkt de bijdrage van bodemroofmijten aan de bestrijding van californische trips minimaal te zijn. De resultaten uit het onderzoek met gedragsbeïnvloedende stoffen laten zien dat er zeker mogelijkheden zijn om met behulp van 'natuurlijke stoffen' (GNO's) de tripsbestrijding te verbeteren. Enkele experimentele gedragsbeïnvloedende stoffen werden getes

Screening van herbiciden voor de boomkwekerij (in deze openbare versie worden de niet toegelaten middelen onder code vermeld) : resultaten praktijkproeven 2007-2009

In de boomteelt en ook die van vaste planten blijft onkruidbeheersing één van de belangrijkste kostenposten. Er zijn de afgelopen jaren veel ontwikkelingen geweest op het gebied van mechanische onkruidbestrijding en afdekmaterialen, maar vaak is het bedrijfseconomisch niet interessant om onkruid mechanisch de baas te blijven. Daarnaast is het resultaat van mechanische onkruidbestrijding sterk weersafhankelijk en is het niet op elk bedrijf mogelijk om (grote) machines in te zetten. Redenen waarom tot nu toe onkruid vaak chemisch bestreden wordt. In de afgelopen jaren is het middelenpakket aan herbiciden er niet groter op geworden. Door strengere toelatingseisen, die voortvloeien uit de nieuwe EU-verordening voor het op de markt brengen van gewasbeschermingsmiddelen, komen er minder middelen op de markt. Het beschermingsniveau voor het oppervlaktewater en voor niet-doelwitorganismen wordt versterkt en daarmee wordt het zelfs voor het huidige middelenpakket moeilijk om middelen op de markt te houde

Het ontwerpen van een nieuw duurzaam bewaar- en verwerkingssysteem voor bloembollen

De huidige bewaar-, verwerkings- en ontsmettingssystemen voor bloembollen functioneren verre van optimaal: de bewaring wordt gekenmerkt door een hoog energieverbruik, tijdens het verwerken en ontsmetten treedt verspreiding van ziekten op, en stress en verwonding van de bollen dragen bij aan uitval door ziekten en andere vormen van kwaliteitsverlies. Daarnaast staat het ontsmetten onder grote druk door de risico’s van blootstelling van medewerkers en emissie van middelen naar het milieu. Het sorteren van bollen voorkomt niet dat in de broeierij grote variatie in groeisnelheid en bloemkwaliteit optreedt en de onmogelijkheid om bollen geautomatiseerd rechtop te planten brengt hoge arbeidskosten met zich mee. Dit project had tot doel innovatieve technieken voor de verschillende onderdelen van het verwerken (inclusief ontsmetten) en bewaren te inventariseren en die te integreren tot een totaalsysteem waarin energiezuinig bewaard wordt, blootstelling aan en emissie van middelen tot het verleden behoren, en gezondere en kwalitatief betere bollen geproduceerd worden

Revisiting the term neuroprotection in chronic and degenerative diseases

Thanks to the development of several new researches, the lifetime presented a significant increase, even so, we still have many obstacles to overcome - among them, manage and get responses regarding neurodegenerative diseases. Where we are in the understanding of neuroprotection? Do we really have protective therapies for diseases considered degeneratives such as amyotrophic lateral sclerosis and its variants, Parkinson's disease, Alzheimer's disease and many others? Neuroprotection is defined by many researches as interactions and interventions that can slow down or even inhibit the progression of neuronal degeneration process. We make some considerations on this neuroprotective effect.Department of Neurology, Antonio Pedro University Hospital, Fluminense Federal University , NiteróiNeurology Service, Nova Iguaçu Hospital , PosseBrain Mapping Laboratory and Electroencephalogram, Federal University of Rio de JaneiroBrain Mapping and Functionality Laboratory, Federal University of PiauíSeverino Sombra University Center, School of Medicine , VassourasDepartment of Neurology, Federal University of São Paulo , BrazilDepartment of Neurology, Federal University of São Paulo , BrazilWeb of Scienc

The Glasgow consensus on the delineation between pesticide emission inventory and impact assessment for LCA

Purpose: Pesticides are applied to agricultural fields in order to optimise crop yield and their global use is substantial. Their consideration in Life cycle assessment (LCA) is currently affected by important inconsistencies between the emission inventory and impact assessment phases of LCA. A clear definition of the delineation between the product system model (life cycle inventory, technosphere) and the natural environment (life cycle impact assessment, ecosphere) is currently missing and could be established via consensus building. Methods: A workshop held on the 11 May 2013, in Glasgow, UK, back to back with the 23rd SETAC Europe meeting had the goal of establishing consensus and creating clear guidelines where the boundary between the emission inventory and the impact characterisation model should be set in all three spatial dimensions and time when considering application of substances to an open agricultural field or in greenhouses, and consequent emissions to the natural environment and their potential impacts. More than 30 specialists in agrifood LCI, LCIA, risk assessment, and ecotoxicology, representing industry, government, and academia from 15 countries and four continents met to discuss and reach consensus. The resulting guidelines target LCA practitioners, data (base) and characterisation method developers, and decision makers. Results and discussion: Although, the initial goal was to define recommendations concerning boundaries between technosphere and ecosphere, it became clear that these strongly depend on goal and scope of an LCA study. Instead, the focus was on defining a clear interface between LCI and LCIA, capable of supporting any goal and scope requirements while avoiding double counting or exclusion of important emission flows and their potential impacts. Consensus was reached accordingly on distinct sets of recommendations for LCI and LCIA respectively, recommending for example that buffer zones should be considered as part of the crop production system and the change in yield per ha be considered. While the spatial dimensions of the field were not fixed, the temporal boundary between dynamic LCI fate modelling and steady-state LCIA fate modelling needs to be defined. Conclusions and recommendations: For pesticides application, the inventory should report: pesticide identification, crop, mass applied of each active ingredient, application method or formulation type, presence of buffer zones (y/n), location/country, application time in days before harvest and crop growth stage during application, adherence with Good Agricultural Practice (GAP), and whether the field is considered part of the technosphere or the ecosphere. Additionally, emission fractions to defined environmental media on-field and off-field should be reported. For LCIA, the directly concerned impact categories were identified as well as a list of relevant fate and exposure processes. Next steps and future work were identified: 1) establishing default emission fractions to environmental media for integration into LCI databases, and 2) interaction among impact model developers to extend current methods with new elements/processes mentioned in the recommendations, including targeted technical workshops on “how to” model specific processes.JRC.H.8-Sustainability Assessmen