Mucopolysaccharidosis I, II, and VI: Brief review and guidelines for treatment

Mucopolysaccharidoses (MPS) are rare genetic diseases caused by the deficiency of one of the lysosomal enzymes involved in the glycosaminoglycan (GAG) breakdown pathway. This metabolic block leads to the accumulation of GAG in various organs and tissues of the affected patients, resulting in a multisystemic clinical picture, sometimes including cognitive impairment. Until the beginning of the XXI century, treatment was mainly supportive. Bone marrow transplantation improved the natural course of the disease in some types of MPS, but the morbidity and mortality restricted its use to selected cases. The identification of the genes involved, the new molecular biology tools and the availability of animal models made it possible to develop specific enzyme replacement therapies (ERT) for these diseases. At present, a great number of Brazilian medical centers from all regions of the country have experience with ERT for MPS I, II, and VI, acquired not only through patient treatment but also in clinical trials. Taking the three types of MPS together, over 200 patients have been treated with ERT in our country. This document summarizes the experience of the professionals involved, along with the data available in the international literature, bringing together and harmonizing the information available on the management of these severe and progressive diseases, thus disclosing new prospects for Brazilian patients affected by these conditions

Climate Change Response for Inclusive WASH: A guidance note for Plan International Indonesia

Prepared for Plan International Indonesi

Re-thinking the creative economy through informality and social inclusion: changing policy directions from Latin America

This chapter examines the extent to which a focus on the informal creative economy can support peripheral cultural scenes that remain invisible to policy and society. The first part briefly outlines the key features of informal settlements in Latin America; the second looks at policy initiatives in Rio de Janeiro and Buenos Aires that have sought to develop, encourage and support creative economies in disadvantaged or peripheral areas; the third part reflects on how these initiatives might help expand, challenge or rethink predominant views and policies on the creative economy from other contexts, assessing whether they might also help overcome ‘creative injustice’ (Banks, 2017) in the sector. Overall, the chapter shows how Latin American countries are starting to launch initiatives for the creative sector that are motivated by concerns with social inclusion and development in marginalised locations. However, these goals can be at odds with the frameworks from which the programmes develop, as they are often underpinned by a market-oriented logic based on individualistic entrepreneurialism and commercialisation

CIGR E-Journal Volume 4

Rosana G. Moreira, Editor-in-Chief; Texas A&M UniversityPeer Reviewed Technical Articles: (1) G. Brehme and K.-H. Krause. Compartmental Airflow Simulation in Stables with Natural Ventilation. Vol. IV. January 2002. (2) J.F. Ortega, J.M. Tarjuelo, and J.A. Juan. Evaluation of Irrigation Performance in Localized Irrigation Systems of Semiarid Regions(Castilla-La Mancha, Spain). Vol. IV. February 2002. (3) E.A. Ajav and O.A. Akingbehin. A Study of some Fuel Properties of Local Ethanol Blended with Diesel Fuel. Vol. IV. March 2002. (4) B. Bjerg, K. Svidt, S. Morsing, G. Zhang, and J.O. Johnson. Modelling of a Wall Inlet in Numerical Simulation of Airflow in Livestock Buildings. Vol. IV. March 2002. (5) V. Alchanatis, Y. Kashti, and R. Brikman. A Machine Vision System for Evaluation of Planter Seed Spatial Distribution. Vol. IV. April 2002. (6) O. Slobodzian-Ksenicz, and T. Kuczynski. Effect of Litter Type on Ammonia Emission in Turkey Housing. Vol. IV. May 2002. (7) H.M. Houszka. Thermal Conditions within a Piglet Creep Area with Different Cover Constructions and Different Surface of Cover Materials. Vol. IV. May 2002. (8) R. Tsheko. Discrimination of Plant Species Using Co Occurrence Matrix of Leaves. Vol. IV. May 2002. (9) C.K. Cavalaris and T.A. Gemtos. Evaluation of Four Conservation Tillage Methods in the Sugar Beet Crop. Vol. IV. June 2002. (10) L. Wang, C.B. Parnell and B.W. Shaw. A Study of the Cyclone Fractional Efficiency Curves. Vol. IV. June 2002. (11) H. Takai, K. Nekomoto, P.J. Dahl, E. Okamoto, S. Morita, and S. Hoshiba. Ammonia Contents and Desorption from Dusts Collected in Livestock Buildings. Vol. IV. June 2002. (12) L. Wang, C.B. Parnell and B.W. Shaw. Performance Characteristics of Cyclones in Cotton-Gin Dust Removal. Vol. IV. August 2002. (13) A. Ikeguchi. Ultra Sonic Sprayer Controlling Dust in Experimental Poultry Houses. Vol. IV. October 2002. (14) Y. Nagasaka, K. Taniwaki, R. Otani, and K. Shigeta. An Automated Rice Transplanter with RTKGPS and FOG. Vol. IV. October 2002. (15) Y. Hirai, E. Inoue, K. Mori, and K. Hashiguchi. Analysis of Reaction Forces and Posture of a Bunch of Crop Stalks During Reel Operations of a Combine Harvester. Vol. IV. October 2002. (16) A. Oida and M. Momozu. Simulation of Soil Behavior and Reaction by Machine Part by Means of DEM. Vol. IV. October 2002. (17) A. Oida, H. Shimizu, J. Miyasaka, H. Fujimoto and T. Ibuki. Study on the Performance of a Model Electric Off-Road Vehicle. Vol. IV. October 2002. (18)Y. Kaizu, T. Okamoto and K. Imou. Shape Recognition and Growth Measurement of Micropropagated Sugarcane Shoots. Vol. IV. October 2002. (19)D. Pochi and D. Vannucci. Prediction of Pesticide Distribution on the Ground Based on Boom Sprayer Movements. Vol. IV. December 2002. (20)A. Natsis, G. Papadakis, and I. Pitsilis. Experimental Investigation of the Influence of the Foreploughshare and the Disk Coulter on the Tillage Quality and the Tractor Fuel Consumption. Vol. IV. December 2002. (21)N. Isaac, R. Taylor, S. Staggenborg, M. Schrock, and D. Leikam. Using Cone Index Data to Explain Yield Variation Within a Field. Vol. IV. December 2002. (22)V.Y. Chertkov. Characteristic Crack Dimensions of Saturated Drying Soils: Theory and Applications. Vol. IV. December 2002. (23)E. Morimoto, M. Suguri and M. Umeda. Obstacle Avoidance System for Autonomous Transportation Vehicle based on Image Processing. Vol. IV. December 2002. (24) F. Curto, I. Naas, D. Pereira, D. Salagado, M. Murayama and F. Behrens. Predicting Broiler Breeder?s Behavior Using Electronic Identification. Vol. IV. December 2002. (25) H. Heege and B. Feldhaus. Site Specific Control of Seed-Numbers per Unit Area for Grain Drills. Vol. IV. December 2002... Invited Overview Articles: (1) J. Pawlak, G. Pellizzi and M. Fiala. On the Development of Agricultural Mechanization to Ensure a Long- Term World Food Supply. Club of Bologna. Vol. IV. June 2002 (2) F. Coolman. Developments in Dutch Farm Mechanization: Past and Future. Vol. IV. August 2002. (3) J. Pawlak. Farm Machinery Market in the Second Half of the XX Century. Vol. IV. July 2002. (4) J. Cuello. Foreword to Agricultural Engineering and International Development in the Third Millennium. Vol. IV. September 2002 (5) L. Clarke and C. Bishop. Farm Power/Present and Future Availability in Developing Countries. Vol. IV. October 2002. (6) T. Takakura. Food Production Strategy in East Asia-Engineering Perspective in the Third Millennium. Vol. IV. October 2002 (7) A. Tamimi. Cooperation Through Education: How Southern West Bank, Palestine, Can Be Developed Through Agricultural Engineering. Vol. IV. September 2002. (8) J. Cuello. Making the World a Better Place: What the Agricultural Engineering Professional Organizations Can Do in the New Century to Make Good on Their Age-Old Promise. Vol. IV. October 2002. (9) D. Slack. Engineering in a Shrinking World. Vol. IV. September 2002. (10) G. Feyereisen. Social and Engineering Aspects of an Aquacultural Development Project in the Nakasongola District of Uganda. Vol. IV. September 2002. (11) W. Clyma. Management Strategies for Sustainable Irrigated Agriculture with Organizational Change to Meet Urgent Needs. Vol. IV. September 2002. (12) A. Munack. Agriculture and the Environment: New Challenges for Engineers. Vol. IV. December 2002. (13) P. De Castro. Mechanization and Traceability of Agricultural Products: a Challenge for the Future-- Quality of Production, Reasons and Means for Traceability, Needs of Markets and Institutional and Prescriptive Aspects in the EU Context. Club of Bologna. Vol. IV. September 2002. (14) F. Pierce and R. Cavalieri. Globalization and Traceability of Agricultural Production: The Role of Mechanization. Club of Bologna. Vol. IV. September 2002. (15) I. Naas. Applications of Mechatronics to Animal Production. Club of Bologna. Vol. IV. October 2002. (16) H. Auernhammer. The Role of Mechatronics in Crop Product Traceability. Club of Bologna. Vol. IV. October 2002. (17) B. Douthwaite. How to Enable Innovation. Vol. IV. October 2002. (18) L. Opara. Engineering and Technological Outlook on Traceability of Agricultural Production and Products. Vol. IV. December 2002. (19) Club of Bologna. Conclusions and Recommendations (traceability of agricultural production). Vol. IV. December 2002. (20) J. Reid. Sensors and Data collection. Power Point presentation. Club of Bologna. November 16, 2002. Vol. IV. December 2002