Intracerebroventricular Delivery as a Safe, Long-Term Route of Drug Administration

Intrathecal delivery methods have been used for many decades to treat a broad range of central nervous system disorders. A literature review demonstrated that intracerebroventricular route is an established and well-tolerated method for prolonged central nervous system drug delivery in pediatric and adult populations. Intracerebroventricular devices were present in patients from one to 7156 days. The number of punctures per device ranged from 2 to 280. Noninfectious complication rates per patient (range, 1.0% to 33.0%) were similar to infectious complication rates (0.0% to 27.0%). Clinician experience and training and the use of strict aseptic techniques have been shown to reduce the frequency of complications.(VLID)485637

Best practices for the use of intracerebroventricular drug delivery devices

For decades, intracerebroventricular (ICV), or intraventricular, devices have been used in the treatment of a broad range of pediatric and adult central nervous system (CNS) disorders. Due to the limited permeability of the blood brain barrier, diseases with CNS involvement may require direct administration of drugs into the brain to achieve full therapeutic effect. A recent comprehensive literature review on the clinical use and complications of ICV drug delivery revealed that device-associated complication rates are variable, and may be as high as 33% for non-infectious complications and 27% for infectious complications. The variability in reported safety outcomes may be driven by a lack of consensus on best practices of device use. Numerous studies have demonstrated that employing strict aseptic techniques and following stringent protocols can dramatically reduce complications. Key practices to be considered in facilitating the safe, long-term use of these devices are presented.(VLID)469777

Management Strategies for Cln2 Disease

CLN2 disease (neuronal ceroid lipofuscinosis type 2) is a rare, autosomal recessive, pediatric-onset, rapidly progressive neurodegenerative lysosomal storage disorder caused by tripeptidyl peptidase 1 (TPP1) enzyme deficiency, and is characterized by language delay, seizures, rapid cognitive and motor decline, blindness, and early death. No management guidelines exist and there is a paucity of published disease-specific evidence to inform clinical practice, which currently draws upon experience from the field of childhood neurodisability. Twenty-four disease experts were surveyed on CLN2 disease management and a subset met to discuss current practice. Management goals and strategies are consistent among experts globally and are guided by the principles of pediatric palliative care. Goals and interventions evolve as the disease progresses, with a shift in focus from maintenance of function early in the disease to maintenance of quality of life. A multidisciplinary approach is critical for optimal patient care. This work represents an initial step toward the development of consensus-based management guidelines for CLN2 disease.WoSScopu

The effect of specificity of experience on a firm's perceived importance of institutional knowledge in an ongoing business

We study how three types of firm experience, ranging from the specific to the general, influence the perceived importance of institutional knowledge in the ongoing business of internationalising firms based on a sample of 101 small-to-medium-sized firms. The three types of firm experiences are international, country and ongoing business. The results show that firm experience within the ongoing business, and the experience from multiple past business deals in various countries, develop institutional knowledge, whereas experience from multiple past business deals in a specific country does not. The theoretical contribution of this paper is that it establishes a link between different kinds of experience and managerial cognition in terms of institutional knowledge. In addition, it emphasises that firms develop institutional knowledge from multiple diverse country experiences, and experience in the specific ongoing business rather than experiences at the level of the country. Journal of International Business Studies (2006) 37, 699–712. doi:10.1057/palgrave.jibs.8400214; (2006)

Communities and change in the anthropocene: understanding social-ecological vulnerability and planning adaptations to multiple interacting exposures

The majority of vulnerability and adaptation scholarship, policies and programs focus exclusively on climate change or global environmental change. Yet, individuals, communities and sectors experience a broad array of multi-scalar and multi-temporal, social, political, economic and environmental changes to which they are vulnerable and must adapt. While extensive theoretical-and increasingly empirical-work suggests the need to explore multiple exposures, a clear conceptual framework which would facilitate analysis of vulnerability and adaptation to multiple interacting socioeconomic and biophysical changes is lacking. This review and synthesis paper aims to fill this gap through presenting a conceptual framework for integrating multiple exposures into vulnerability analysis and adaptation planning. To support applications of the framework and facilitate assessments and comparative analyses of community vulnerability, we develop a comprehensive typology of drivers and exposures experienced by coastal communities. Our results reveal essential elements of a pragmatic approach for local-scale vulnerability analysis and for planning appropriate adaptations within the context of multiple interacting exposures. We also identify methodologies for characterizing exposures and impacts, exploring interactions and identifying and prioritizing responses. This review focuses on coastal communities; however, we believe the framework, typology and approach will be useful for understanding vulnerability and planning adaptation to multiple exposures in various social-ecological contexts