Brain MRI findings in children and adolescents with Fabry disease.

OBJECTIVE: To evaluate the presence of white matter and hemorrhagic lesions in brain MRI of children and adolescents with Fabry disease (FD). METHODS: Brain MRI studies in 44 consecutive children and teenagers (20 boys, mean age 14.6 years, range 7-21 years) were evaluated using classic sequences as well as, GRE-weighted images, for white matter lesions (WML) and chronic microbleed detection. All patients lacked history of stroke or TIA. Brain MRI findings in 46 consecutive children and adolescents without FD, referred for the evaluation of headaches (36 females, mean age 14.1 years, range 7-21 years) were evaluated as a control group. Additionally, we assessed the clinical manifestations of FD. RESULTS: Seven children (15.9%) with FD had brain MRI evidence of asymptomatic WML (5 girls, mean age 14.8 years, range: 13-20 years) compared with 3 children (6.5%) in the control group (p = 0.01). Brain abnormalities in patients with FD revealed WML, deep gray matter and infratentorial involvement. Three patients presented two lesions each. None of the children showed microbleeds. Regarding clinical manifestations, 90.9% of the patients had signs or symptoms of FD. CONCLUSION: We identified asymptomatic white matter brain lesions in 15.9% of children with FD without clinical history of stroke. FD is a treatable disorder that should be routinely included in the differential diagnosis of both symptomatic and asymptomatic brain lesions in children and adolescents. The detection of brain lesions may foster earlier treatment.

Neurografía de alta resolución en resonancia magnética 3 Tesla del plexo braquial / High resolution neurography of the brachial plexus by 3 Tesla magnetic resonance imaging

El estudio de las estructuras que conforman el plexo braquial se ha visto particularmente beneficiado con las imágenes de alta resolución que brindan los equipos de resonancia 3 T. El plexo braquial puede presentar mononeuropatías o polineuropatías. Entre las primeras se distinguen los traumatismos, el atrapamiento, como el síndrome de la abertura torácica por costillas cervicales, apófisis transversas prominentes o tumores. En el grupo de las polineuropatías se encuentran los procesos inflamatorios, entre los que destacan la polineuropatía desmielinizante inflamatoria crónica, la plexitis autoinmunitaria (síndrome de Parsonage Turner), enfermedades granulomatosas y la neuropatía por radiación. Entre los procesos vasculares se mencionan la polineuropatía diabética y las vasculitis. En esta revisión se repasa la anatomía del plexo braquial y se describe la técnica de estudio de la neurografía por resonancia magnética y las principales patologías que pueden afectar al plexo braquial (AU)

The study of the structures that make up the brachial plexus has benefited particularly from the high resolution images provided by 3 T magnetic resonance scanners. The brachial plexus can have mononeuropathies or polyneuropathies. The mononeuropathies include traumatic injuries and trapping, such as occurs in thoracic outlet syndrome due to cervical ribs, prominent transverse apophyses, or tumors. The polyneuropathies include inflammatory processes, in particular chronic inflammatory demyelinating polyneuropathy, Parsonage-Turner syndrome, granulomatous diseases, and radiation neuropathy. Vascular processes affecting the brachial plexus include diabetic polyneuropathy and the vasculitides (AU)

High resolution neurography of the brachial plexus by 3 Tesla magnetic resonance imaging.

The study of the structures that make up the brachial plexus has benefited particularly from the high resolution images provided by 3T magnetic resonance scanners. The brachial plexus can have mononeuropathies or polyneuropathies. The mononeuropathies include traumatic injuries and trapping, such as occurs in thoracic outlet syndrome due to cervical ribs, prominent transverse apophyses, or tumors. The polyneuropathies include inflammatory processes, in particular chronic inflammatory demyelinating polyneuropathy, Parsonage-Turner syndrome, granulomatous diseases, and radiation neuropathy. Vascular processes affecting the brachial plexus include diabetic polyneuropathy and the vasculitides. This article reviews the anatomy of the brachial plexus and describes the technique for magnetic resonance neurography and the most common pathologic conditions that can affect the brachial plexus.

Influence of white matter MRI hyper-intensities on acute l-dopa response in patients with Parkinson's disease.

OBJECTIVE: To determine whether brain white matter hyperintensities (WMH) influence l-dopa response in Parkinson's disease (PD) patients. METHODS: We prospectively evaluated 60 PD patients with an acute l-dopa challenge test, and assessed motor performance with the Movement Disorders Society revised Unified Parkinson's Disease Rating Scale (MDS-UPDRS) during "ON" and "OFF" medication states. Magnetic resonance images were examined using a visual semi-quantitative rating scale for quantification and distribution analysis of WMH. l-dopa challenge test response was correlated to extent and location of WMH, to determine a potential association between them. RESULTS: Subjects with greater deep WMH burden, showed less response to l-dopa on axial motor symptoms (R = -0.35; p < 0.027), when tested with Part III of the MDS-UPDRS before and after acute levodopa challenge. CONCLUSIONS: Results suggest WMH may affect response to l-dopa on axial function of PD patients, which could be due to either non-dopaminergic (cortico-basal ganglia) motor pathway disruption, or postsynaptic nigrostriatal pathway involvement.

I-C-SEA Change: A participatory tool for rapid assessment of vulnerability of tropical coastal communities to climate change impacts.

We present a synoptic, participatory vulnerability assessment tool to help identify the likely impacts of climate change and human activity in coastal areas and begin discussions among stakeholders on the coping and adaptation measures necessary to minimize these impacts. Vulnerability assessment tools are most needed in the tropical Indo-Pacific, where burgeoning populations and inequitable economic growth place even greater burdens on natural resources and support ecosystems. The Integrated Coastal Sensitivity, Exposure, and Adaptive Capacity for Climate Change (I-C-SEA Change) tool is built around a series of scoring rubrics to guide non-specialists in assigning scores to the sensitivity and adaptive capacity components of vulnerability, particularly for coral reef, seagrass, and mangrove habitats, along with fisheries and coastal integrity. These scores are then weighed against threat or exposure to climate-related impacts such as marine flooding and erosion. The tool provides opportunities for learning by engaging more stakeholders in participatory planning and group decision-making. It also allows for information to be collated and processed during a "town-hall" meeting, facilitating further discussion, data validation, and even interactive scenario building.

Modelling coral reef futures to inform management: can reducing local-scale stressors conserve reefs under climate change?

Climate change has emerged as a principal threat to coral reefs, and is expected to exacerbate coral reef degradation caused by more localised stressors. Management of local stressors is widely advocated to bolster coral reef resilience, but the extent to which management of local stressors might affect future trajectories of reef state remains unclear. This is in part because of limited understanding of the cumulative impact of multiple stressors. Models are ideal tools to aid understanding of future reef state under alternative management and climatic scenarios, but to date few have been sufficiently developed to be useful as decision support tools for local management of coral reefs subject to multiple stressors. We used a simulation model of coral reefs to investigate the extent to which the management of local stressors (namely poor water quality and fishing) might influence future reef state under varying climatic scenarios relating to coral bleaching. We parameterised the model for Bolinao, the Philippines, and explored how simulation modelling can be used to provide decision support for local management. We found that management of water quality, and to a lesser extent fishing, can have a significant impact on future reef state, including coral recovery following bleaching-induced mortality. The stressors we examined interacted antagonistically to affect reef state, highlighting the importance of considering the combined impact of multiple stressors rather than considering them individually. Further, by providing explicit guidance for management of Bolinao's reef system, such as which course of management action will most likely to be effective over what time scales and at which sites, we demonstrated the utility of simulation models for supporting management. Aside from providing explicit guidance for management of Bolinao's reef system, our study offers insights which could inform reef management more broadly, as well as general understanding of reef systems.

Identification of Races 0 and 2 of Cladosporium fulvum (syn Passalora fulva) on Tomato in the Cinturón Hortícola de La Plata, Argentina.

Surveys aimed at evaluating the incidence and severity of a new disease that developed in greenhouses cultivated with tomato (Solanum lycopersicum L.) were performed during 2009 and 2010 in greenhouses of the cultivars Elpida (Enza Zaden) and Colibrí (Clause) in an area of tomato production known as the Cinturón Hortícola de La Plata (the "horticultural belt of La Plata"). The disease had a 100% prevalence and 90% incidence within the ten 250 m2 greenhouses that were monitored in 2009, 2010, and 2011. In two consecutive assays, severity was 40%. The wide distribution of the disease suggests that the tomato hybrids under use lack resistance genes. The upper surface of diseased leaves had pale green to yellowish, 1- to 1.5-cm spots with undefined margins that progressed to a yellowish brown color, while on the lower side they had pale brown to brown sporulation of fungal conidiophores and conidia. Monosporic fungal cultures were obtained by needle transfer of conidia from sporulating areas of leaves (n = 20) to water agar medium. On 2% potato dextrose agar (PDA) the colonies of the relatively low growing fungus were strongly pigmented, greenish grey, and black on the reverse of the plate. The fungus developed one-celled, pale olive-green, ovoid conidia on long, branched chains, which arose from pigmented conidiophores, corresponding to the description of Cladosporium fulvum made by Joosten and de Wit (1). The identity of two isolates was confirmed by amplifying the 580-bp ITS sequences by means of primers ITS4 (5'-TCCTCCGCTTATTGATATGC-3') and ITS5 (5'-GAATTCGGAAGTAAAAGTCGTAACAAGG-3') (ITS sequence Race 0 JQ768324.1 and Race 2 JQ768325.1). Both were 100% homologous to the ITS sequences of C. fulvum strains ATCC44962 (AF393700) and ATCC44960 (AF303701). Monosporic cultures of four isolates, each obtained from leaves collected from different plants growing in different greenhouses, were inoculated on a set of differential genotypes of tomato: cvs. Moneymaker, Cf-0, Cf-2, Cf-4, Cf-5, and Cf-9 (kindly provided by the Laboratory of Phytopathology of Wageningen University). Three plants of each tomato genotype at the 5 to 6 true leaf stage were inoculated by spraying a 105 conidia/ml conidial suspension of C. fulvum on the leaflets of the 3rd and 4th leaf. Inoculation tests of each isolate were repeated at least twice. After inoculation, plants were grown in the greenhouse at 13 to 29°C and 99% relative humidity. However, for the first 20 h after inoculation, plants were kept in the dark. They were regularly monitored and were scored as resistant or susceptible at 20 days after inoculation. Susceptible genotypes developed pale green to yellow spots on the abaxial leaf surface and pale brown to olivaceous brown sporulation on the adaxial side. Plants lacking disease symptoms were considered resistant. Inoculated fungi were reisolated from infected tissue and the identity of the fungal cultures confirmed based on morphology and the ITS sequence. Based on the reactions of the tomato genotypes, two races were identified, three isolates (race 2) developed symptoms only in cv. MM Cf-2, while the remaining isolate (race 0) provoked symptoms only in cv. MM Cf-0. Reference: (1) M. Joosten and P. de Wit. Annu Rev Phytopathol. 37:335, 1999.

Superoxide dismutase (SOD) and neutrophil infiltration in intestinal ischaemia-revascularization.

SOD is the main detoxifying enzyme of OFRs which have been mainly purported to participate in ischaemia revascularization lesions. A study was made of the behaviour of SOD during ischaemia and the response to pharmacological doses of SOD in Wistar rats in which ischaemia was induced by 90 min of clamping and followed by revascularization. SOD levels were determined in the intestinal wall, evaluating the degree of infiltration of neutrophils, leucocytes and monocytes by immunohistochemical methods. Ischaemia led to a significant decrease in intestinal wall SOD levels (p = 0.003). The administration of pharmacological doses of SOD was observed to improve survival of the animals (p = 0.001) and significantly decreased the infiltration of leucocytes only during revascularization measured by MPO and LCA. Beneficial effects of SOD could be explained by its effect as scavenger of OFRs and by its action on the neutrophil infiltration.

Improved survival in intestinal ischemia by allopurinol not related to xanthine-oxidase inhibition.

Allopurinol, a xanthine-oxidase (XO) inhibitor, has been used to improve the resistance to ischemia with disappointing results that have been attributed to administration regimen of the drug. Our aim was to investigate the effect of different administration schedules of allopurinol on the survival in rats undergoing intestinal ischemia testing the blockade of XO. Intestinal ischemia was achieved by 90 min of clamping the superior mesenteric artery (SMA) close to its origin from the aorta. Three groups of animals were evaluated: A-group: only the allopurinol solvent was given; B-group: the full dose of allopurinol (100 mg/k b.w.) was given iv and C-group: the 75% dose was administered orally 24 hr before and the remaining 25% was administered 30 min before. Survival was evaluated at 48 hr and the blockade of XO was assayed by High Efficacy Liquid Chromatography (HELC) in homogenate of intestinal wall. Survival was only improved in the C-group (P = 0.02). Levels of hypoxanthine were significantly increased both in B-group and C-group (P = 0.003) when compared with the A-group. Levels of uric acid in B-group (P = 0.0003) and C-group (P = 0.0009) were significantly decreased with respect to A-group. That means that an effective blockade of XO is achieved whichever the regimen of administration. Allopurinol and oxypurinol levels were significantly increased (P = 0.05 and P = 0.008) in C-group when compared with B-group. We conclude that the protective effect of allopurinol on survival in intestinal ischemia in rats is not related to the blockade of XO but rather to the allopurinol and oxypurinol levels in intestinal wall.

[Effect of xanthine oxidase inhibitors on the prognosis of acute intestinal ischemia]. / Efecto de los inhibidores de la xantino-oxidasa sobre el pronóstico de la isquemia intestinal aguda.

During the last few years, the scientific field has focused its attention on the pathogenic role of free radicals in the process of ischemia-revascularization. It is a well-known fact that xanthine oxidase is an important source of tissular free radicals. Bearing this in mind, we designed an experimental protocol to analyse the effect of allopurinol (a xanthine oxidase inhibitor) in the survival of rats after the occlusion of the superior mesenteric artery during a period of 90 minutes and its action on the superoxide radical liberation. The concentration of oxipurinol and allopurinol in the ischemic area (intestine), liver and blood were measured. We concluded that the administration of allopurinol increased the survival rate, which is correlated to higher concentrations of allopurinol and oxipurinol in the inner part of the intestinal cells. A correlation between the survival rate and superoxide radicals was not found.