Megalencephalic Leukoencephalopathy With Subcortical Cysts (mlc) – A Case With Clinical And Magnetic Resonance Imaging (mri) Dissociation [leucoencefalopatia Megalencefálica Com Cistos Subcorticais (mlc) – Um Caso Com Dissociação Clínica E De Resonância Magnética]

[No abstract available]732171172Nunes, R.H., Pacheco, F.T., Rocha, A.J., Magnetic resonance imaging of anterior temporal lobe cysts in children: Discriminating special imaging features in a particular group of diseases (2014) Neuroradiology, 56, pp. 569-577. , http://dx.doi.org/10.1007/s00234-014-1356-9Olivier, M., Lenard, H.G., Aksu, F., Gärtner, J., A new leukoencephlapathy with bilateral anterior temporal lobe cysts (1998) Neuropediatrics, 29 (5), pp. 225-228. , http://dx.doi.org/10.1055/s-2007-973566Knaap, M.S., Boor, I., Estévez, R., Megalencephalic leukoencephalopathy with subcortical cysts: Chronic white matter oedema due to a defect in brain ion and water homoeostasis (2012) Lancet Neurol, 11 (11), pp. 973-985. , http://dx.doi.org/10.1016/S1474-4422(12)70192-8Sinhal, B.S., Gorospe, J.R., Naidu, S., Megalencephalic leukoencephalopathy with subcotical cysts (2003) J Child Neurol, 18 (9), pp. 645-652. , http://dx.doi.org/10.1177/0883073803018009120

Challenges and opportunities for a South America Waterway System

South America has been developed from its coast to its hinterlands since the beginning of its Western colonization. However, to this point, no significant effort has been made to integrate its interior. Waterways transportation can be considered the most sustainable inland mode of transportation due to its low CO2 emissions per ton of cargo transport. With this in mind, this paper investigates the history, challenges and opportunities of the past proposals for the construction of the South America Waterway System (SAWS) connecting the La Plata, Amazon, and Orinoco river basins. It focuses on particular challenges of the proposed waterway. (i) a comparison between the deforestation surrounding existing road and waterway infrastructure in the Amazon, (ii) the large water level variation in the Amazon basin, (iii) and the alternatives for storing water to reduce the impacts of floods and droughts in the proposed waterway. We conclude that deforestation surrounding existing waterways is practically zero and that groundwater storage has an important role in storing water for the basin and reservoirs, a limited one. The SAWS can significantly foster South American integration, encourage sustainable extraction of natural resources in the region and help the conservation of the Amazon forest

Effectiveness and safety of obeticholic acid in a Southern European multicenter cohort of patients with primary biliary cholangitis and suboptimal response to ursodeoxycholic acid

Background Obeticholic acid (OCA) was recently approved as the only on-label alternative for patients with primary biliary cholangitis (PBC) with intolerance or suboptimal response to ursodeoxycholic acid (UDCA). However, few data are available outside clinical trials. Aim To assess the effectiveness and safety of OCA in a real-world cohort of patients with non-effective UDCA therapy. Methods Open-label, prospective, real-world, multicentre study, enrolling consecutive patients who did not meet Paris II criteria, from 18 institutions in Spain and Portugal. Effectiveness was assessed by the changes in GLOBE and UK-PBC scores from baseline. POISE and Paris II criteria were evaluated after 12 months of OCA . Liver fibrosis was evaluated by FIB-4 and AST to platelet ratio index (APRI). Results One hundred and twenty patients were eligible, median time since PBC diagnosis 9.3 (4.0-13.8) years, 21.7% had cirrhosis, and 26.7% received had previous or concomitant treatment with fibrates. Seventy-eight patients completed at least 1 year of OCA. The Globe-PBC score decreased to 0.17 (95% CI 0.05 to 0.28; P = 0.005) and the UK-PBC score decreased to 0.81 (95% CI -0.19 to 1.80; P = 0.11). There was a significant decrease in alkaline phosphatase of 81.3 U/L (95% CI 42.5 to 120; P < 0.001), ALT 22.1 U/L (95% CI 10.4 to 33.8; P < 0.001) and bilirubin 0.12 mg/dL (95% CI 0 to 0.24; P = 0.044). FIB-4 and APRI remained stable. According to the POISE criteria, 29.5% (23 out of 78) achieved response. The adverse events rate was 35%; 11.67% discontinued (8.3% due to pruritus). Conclusions This study supports data from phase III trials with significant improvement of PBC-Globe continuous prognostic marker score among OCA-treated patients with good tolerability