16 research outputs found
Gamification: a key determinant of massive open online course (MOOC) success
Massive open online courses (MOOCs), contribute significantly to individual empowerment because they can help people learn about a wide range of topics. To realize the full potential of MOOCs, we need to understand their factors of success, here defined as the use, user satisfaction, along the individual and organizational performance resulting from the user involvement. We propose a theoretical framework to identify the determinants of successful MOOCs, and empirically measure these factors in a real MOOC context. We put forward the role of gamification and suggest that, together with information system (IS) theory, gamification proved to play a crucial role in the success of MOOCs.info:eu-repo/semantics/acceptedVersio
Herpes Simplex Encephalitis Does Interferon Care?
Introduction and aims: Herpes simplex encephalitis (HSE) is an acute, life-threatening disease, requiring prompt intervention. TLR3-interferon (IFN) axis defects in the antiviral innate immune response against HSV-1 and some genes (TLR3, UNC93B1 and TRAF3) probably play an important role in HSE pathogenesis.
Methods: Descriptive study between January 2007 and December 2012 from HSE patients treated with acyclovir (initiated between D2 to D3 of illness) and INF alpha-2b. HSV-1 was detected by PCR from CSF. PBMC and fibroblasts were studied for their IFN responses to TLR3 and virus stimulations. Coding exons of the known HSE-associated genes were sequenced.
Results: Six cases, aged between 7 months and 11 years, with seizures and extensive brain injury. Interferon was initiated between D3 and D18. Patient 1 initiated IFN on D18 and stopped 7 days later for bicytopenia. Patient 2 started on D3 and has no sequelae. Patient 4 started on D5 and has persistent right sided hemiparesis. Patient 3, 5 and 6 started on D5, D3 and D7 respectively remain with epilepsy under medical control. Only Patient 1, who started IFN later than D7, has sequelar tetraparesis. None of the other patients have severe neurological deficits. The functional studies were normal, except for patient 1 whose fibroblasts displayed impaired IFN-lambda production after stimulations of poly(I:C), thought to be TLR3-dependent. No mutation was found in the sequenced coding exons of UNC93B1, TLR3 and TRAF3.
Conclusions: Although a small sample, our results suggest that IFN therapy should be considered in the treatment of HSE.info:eu-repo/semantics/publishedVersio
TANGO2 Deficiency Disorder: Two Cases of Developmental Delay Preceding Metabolic Crisis
Background: TANGO2 deficiency disorder is a rare genetic disease caused by biallelic defects in TANGO2 gene.
Methods: We report the clinical phenotype of two children with TANGO2 deficiency disorder.
Results: Patient 1 is a female child presenting with developmental delay and microcephaly during the second year of life, who evolved with severe cognitive impairment, facial dysmorphisms, spastic paraparesis, and atonic seizures. At age 13 years, she was hospitalized due to an episode of rhabdomyolysis complicated with cardiac arrhythmia and hypothyroidism. Patient 2 is a female child with dysmorphic facial features, cleft palate, and developmental delay who was diagnosed with DiGeorge syndrome. At age three years, she presented with an acute episode of severe rhabdomyolysis in the context of human herpesvirus 6 infection. After the resolution of this acute episode, she maintained recurrent muscle weakness with axial hypotonia and progressive spasticity of the lower extremities. In both patients, diagnosis of TANGO2 deficiency disorder was only confirmed after an acute metabolic crisis.
Conclusions: A high index of suspicion for TANGO2 deficiency disorder is needed in patients with developmental delay or other neurological symptoms and episodic rhabdomyolysis.info:eu-repo/semantics/publishedVersio
Retinopathy and Bone Marrow Failure Revealing Coats Plus Syndrome
info:eu-repo/semantics/publishedVersio
Ecological risk assessment of sediment management areas : application to Sado Estuary, Portugal
The purpose of this work was to integrate different
methodologies to assess the potential ecological risk
of estuarine sedimentary management areas, using the Sado
Estuary in Portugal as case study. To evaluate the environmental
risk of sediment contamination, an integrative
and innovative approach was used involving assessment of
sediment chemistry, sediment toxicity, benthic community
structure, human driving forces and pressures and management
areas organic load levels. The basis for decisionmaking
for overall assessment was a statistical multivariate
analysis appended into a score matrix tables, using a best
expert judgment. The integrated approach allowed to
identify from the 19 management areas analyzed, three
with no risk but other three with high risk to cause adverse
effects in the biota, related with the contaminants analyzed.
The methodologies used showed to be effective as a support
for decision making leading to future estuarine management
recommendations.peerreviewe
Non-Invasive Ventilation: Three Years of Clinical Practice in a Paediatric Intermediate Care Unit
Introdução: A ventilação não invasiva tem sido utilizada de forma crescente em crianças com insuficiência respiratória aguda,evitando as complicações associadas à ventilação invasiva. Pretendeu-se descrever a experiência de ventilação não invasiva numa unidade de cuidados intermédios e avaliar a sua eficácia em evitar a transferência das crianças para uma unidade de cuidados intensivos pediátricos.
Métodos: Estudo longitudinal retrospetivo de todas as crianças admitidas numa unidade de cuidados intermédios e submetidas a ventilação não invasiva (2012-2015). Foram analisados dados demográficos, ocorrência de complicações, frequência respiratória, frequência cardíaca, pH, pressão parcial de dióxido de carbono e relação entre a saturação periférica de oxigénio e fração inspiratória de oxigénio, antes e duas, quatro, seis, 12 e 24 horas após inicio da ventilação não invasiva. Os doentes foram
divididos em dois grupos, sucesso ou insucesso da ventilação não invasiva, de acordo com a necessidade de transferência.
Resultados: Foram incluídas 35 crianças com mediana de idade 42 dias. O diagnóstico principal foi bronquiolite em 28 doentes e a indicação para iniciar a ventilação não invasiva foi insuficiência respiratória aguda em 32. Foram eficazmente ventilados 29 (82,9%) e seis necessitaram de ser transferidos. Verificou-se uma melhoria da frequência respiratória, frequência cardíaca, pH e pressão parcial de dióxido de carbono a partir das duas horas de ventilação não invasiva no grupo de sucesso (p < 0,05). Não houve complicações major.
Discussão: Confirmou-se a efectividade e segurança da ventilação não invasiva em evitar o agravamento clínico das crianças com insuficiência respiratória aguda numa unidade de cuidados intermédios, evitando a ventilação invasiva e/ou transferência para uma unidade de cuidados intensivos pediátricos.info:eu-repo/semantics/publishedVersio
Cross-Roads of Tourism: A Complex Spatial Systems Analysis of Tourism and Urban Sprawl in the Algarve
Urban development combined with city expansion, has brought irreversible consequences for land use and environmental degradation. The balance between stability in urban areas and biodiversity, relates in essence to sustainability and economic development. This economic development in southern Europe is especially affected by service industries such as tourism. Preventing future damage is particularly necessary in coastal zones, where contributing factors of a human or natural nature require important strategies to be designed for regional and urban planning. The application of the spatial realities of land use within temporal dynamics allows the ex-ante assessment of spatial planning policies. The combination of economic, social, and natural consequences questions the application of complex system theory within spatio-temporal dynamics supporting regional decision making. In this context, geographic information systems, combined with spatial data inventories, are used for a systemic analysis of the dynamics of urban change within land-use dynamics and complex systems. Copyright © 2011 Inderscience Enterprises Ltd