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

Wide area microprobe analyser (WAMPA)

Wide area microprobe analyser (WAMPA) represents a new scientific instrument concept for planetary exploration. WAMPA builds on recently published research such as sensor webs and distributed microsensors [The sensor web: a new instrument concept, SPIE Symposium on Integrated Optics, 20–26 January 2001, San Jose, CA; Design considerations for distributed microsensor systems, Proceedings of the IEEE 1999 Custom Integrated Circuits Conference (CICC ’99), May 1999, pp. 279–286] but adds new sensor and localisation concepts. WAMPA is driven by the recurrent theme in spacecraft and sensor design to achieve smaller, lighter and lower cost systems. The essential characteristics of the WAMPA design that differentiates it from other space science instruments are that WAMPA is both a wide area instrument, consisting of a distributed set of sensors, and that each probe is designed to use little, if any, power. It achieves the former by being utilised in large numbers (>10), requiring that the individual probes be low mass and low volume . It is envisaged that the probes would be dispersed by landers or rovers as mission support instruments rather than primary science instruments and would be used in hostile environments and rugged terrains where the lander/rover could not be risked (see Fig. 1)

Evolution of aeronautical communications for personal and multimedia services

The demand to make air travel more pleasant, secure, and productive for passengers is one of the winning factors for both airlines and the aircraft manufacturing industry for which aeronautical communications is one of the enablers. This article describes current trends in the area of aeronautical passenger communication toward personal and wireless in-cabin communications and multimedia data networks. Technological challenges are summarized as well as market potentials and regulatory issues

Aeronautical communications for personal and multimedia services via satellite

The demand for making air travel more pleasant, secure and productive for passengers is one of the winning factors for both airlines and the aircraft manufacturing industry for which aeronautical communications is one of the enablers. This article describes current trends in the area of aeronautical passenger communication towards personal and wireless in-cabin communications and multimedia data networks. Technological challenges are summarized as well as market potentials