3,414 research outputs found
The Highly Miniaturised Radiation Monitor
We present the design and preliminary calibration results of a novel highly
miniaturised particle radiation monitor (HMRM) for spacecraft use. The HMRM
device comprises a telescopic configuration of active pixel sensors enclosed in
a titanium shield, with an estimated total mass of 52 g and volume of 15
cm. The monitor is intended to provide real-time dosimetry and
identification of energetic charged particles in fluxes of up to 10
cm s (omnidirectional). Achieving this capability with such a
small instrument could open new prospects for radiation detection in space.Comment: 17 pages, 15 figure
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Boosting MultiFarm track with Turkish dataset
The evolution of semantic structured data, such as those behind the deep web or socialnetworks, requires mapping between sources to enable a high level integration. Sev-eral ontology matching systems have been developed to establish mappings betweenmultilingual ontologies, however, employing these systems in real world requires anassessment of the ontologies capability and performance which is conducted by theMultiFarm Track. Yet, this track still lacks of ontologies from different language fami-lies. In this paper, we contribute to the OAEI initiative with a Turkish dataset to extendthe coverage of languages for the matching systems
Identification versus CBCD: a comparison of different evaluation techniques
Fingerprint techniques have a significant advantage in respect of watermarking: a fingerprint can be extracted in each moment of the lifetime of a multimedia content. This aspect is fundamental to solve the problem of copy detection mainly because many copies can be available in huge amount of data in circulation and because each copy can be attacked in several ways (compression, re-encoding, text-overlay, etc.). In this paper the problem of copy detection is studied and tested from two different point of views: content based and identification approaches. The results show that the proposed system is quite robust to some copy modifications and most of all show that the overall results depend on the evaluation method used for testing
Evaluation of Core Experiment for MPEG-7 Video Signature Tool
The present document evaluates and discusses the experiment conditions proposed in the VCE-7 CE on Video Signature, discussed in the previous MPEG meetings [MPEG-VS-09-01] [MPEG-VS-09-02]. In particular we present some simulation results that show the weaknesses of the evaluation procedure, proposed in the VCE-7 CE on Video Signature, and that highlight some aspects that could impair the performance evaluation of the here considered technology in a realistic environment. Therefore, we propose a new Core Experiment which will consider this issues
A proposal for Video Signature Tool and Video Fingerprinting
In this document we present and evaluate a video signature system, proposed by Signals and Communications Laboratory – Department of Electronic for Automation, University of Brescia (Italy). The proposed Video Signature is MPEG-7 compliant. The technology is based on the use of many different features (color, motion, etc.). The preliminary studies show that these features have relevant characteristics in their development in time. The testing results prove that the features act differently with respect of the type and modification the query suffered
GNAO1 encephalopathy: broadening the phenotype and evaluating treatment and outcome
OBJECTIVE:
To describe better the motor phenotype, molecular genetic features, and clinical course of GNAO1-related disease.
METHODS:
We reviewed clinical information, video recordings, and neuroimaging of a newly identified cohort of 7 patients with de novo missense and splice site GNAO1 mutations, detected by next-generation sequencing techniques.
RESULTS:
Patients first presented in early childhood (median age of presentation 10 months, range 0-48 months), with a wide range of clinical symptoms ranging from severe motor and cognitive impairment with marked choreoathetosis, self-injurious behavior, and epileptic encephalopathy to a milder phenotype, featuring moderate developmental delay associated with complex stereotypies, mainly facial dyskinesia and mild epilepsy. Hyperkinetic movements were often exacerbated by specific triggers, such as voluntary movement, intercurrent illnesses, emotion, and high ambient temperature, leading to hospital admissions. Most patients were resistant to drug intervention, although tetrabenazine was effective in partially controlling dyskinesia for 2/7 patients. Emergency deep brain stimulation (DBS) was life saving in 1 patient, resulting in immediate clinical benefit with complete cessation of violent hyperkinetic movements. Five patients had well-controlled epilepsy and 1 had drug-resistant seizures. Structural brain abnormalities, including mild cerebral atrophy and corpus callosum dysgenesis, were evident in 5 patients. One patient had a diffuse astrocytoma (WHO grade II), surgically removed at age 16.
CONCLUSIONS:
Our findings support the causative role of GNAO1 mutations in an expanded spectrum of early-onset epilepsy and movement disorders, frequently exacerbated by specific triggers and at times associated with self-injurious behavior. Tetrabenazine and DBS were the most useful treatments for dyskinesia
Dinâmica do nitrogênio em função da aplicação de lodos de esgoto em sistema de plantio direto.
Projeto/Plano de Ação: 01.09.10103-04
Pengendalian Frekuensi Dengan Menggunakan Kontrol Fuzzy Prediktif Pada Simulator Plant Turbin-Generator Pada PLTU
Sistem pembangkit dirasakan sangat perlu guna memenuhi kebutuhan tenaga listrik yang semakin meningkat, kestabilan sangat dibutuhkan pada proses pembangkit sehingga sistem pengendalian digunakan untuk menjaga variabel proses tersebut tetap stabil. Salah satunya adalah dengan melakukan pengendali frekuensi pada tubin-generator suatu pembangkit listik, contohnya PLTU (Pembangkit Listrik Tenaga Uap). Frekuensi dari turbin uap harus dijaga kestabilannya agar keluaran daya listrik di generator berjalan dengan baik. Fluktuasi frekuensi adalah salah satu kendala penyampaian daya listrik ke beban, juga waktu kembali yang tidak segera ke kondisi normal akan mengakibatkan kerusakan pada sistem seperti patahnya poros turbin-generator dan kemungkinan terjadi gangguan pada jaringan listrik, sehingga perlu dilakukan pengaturan laju aliran uap yang masuk ke turbin. Kontroler yang digunakan untuk menjaga Perubahan frekuensi adalah kontrol fuzzy prediktif, dengan penambahan gain K1 pada kontrol fuzzy prediktif sebesar 42.35 yang bekerja secara sucsessive kontroler ini dapat mengurangi error sebesar 1,04% jika sistem hanya menggunakan kontroler fuzzy pada saat terjadi Perubahan beban
Idiopathic epilepsies with seizures precipitated by fever and SCN1A abnormalities.
Epilepsia. 2007 Sep;48(9):1678-85. Epub 2007 Jun 11.
Idiopathic epilepsies with seizures precipitated by fever and SCN1A abnormalities.
Marini C, Mei D, Temudo T, Ferrari AR, Buti D, Dravet C, Dias AI, Moreira A, Calado E, Seri S, Neville B, Narbona J, Reid E, Michelucci R, Sicca F, Cross HJ, Guerrini R.
SourceEpilepsy, Neurophysiology and Neurogenetic Unit, Institute of Child Neurology and Psychiatry, IRCCS Stella Maris Foundation, Calambrone, Pisa, Italy.
Abstract
PURPOSE: SCN1A is the most clinically relevant epilepsy gene, most mutations lead to severe myoclonic epilepsy of infancy (SMEI) and generalized epilepsy with febrile seizures plus (GEFS+). We studied 132 patients with epilepsy syndromes with seizures precipitated by fever, and performed phenotype-genotype correlations with SCN1A alterations.
METHODS: We included patients with SMEI including borderline SMEI (SMEB), GEFS+, febrile seizures (FS), or other seizure types precipitated by fever. We performed a clinical and genetic study focusing on SCN1A, using dHPLC, gene sequencing, and MLPA to detect genomic deletions/duplications on SMEI/SMEB patients.
RESULTS: We classified patients as: SMEI/SMEB = 55; GEFS+= 26; and other phenotypes = 51. SCN1A analysis by dHPLC/sequencing revealed 40 mutations in 37 SMEI/SMEB (67%) and 3 GEFS+ (11.5%) probands. MLPA showed genomic deletions in 2 of 18 SMEI/SMEB. Most mutations were de novo (82%). SMEB patients carrying mutations (8) were more likely to have missense mutations (62.5%), conversely SMEI patients (31) had more truncating, splice site or genomic alterations (64.5%). SMEI/SMEB with truncating, splice site or genomic alterations had a significantly earlier age of onset of FS compared to those with missense mutations and without mutations (p = 0.00007, ANOVA test). None of the remaining patients with seizures precipitated by fever carried SCN1A mutations.
CONCLUSION: We obtained a frequency of 71%SCN1A abnormalities in SMEI/SMEB and of 11.5% in GEFS+ probands. MLPA complements DNA sequencing of SCN1A increasing the mutation detection rate. SMEI/SMEB with truncating, splice site or genomic alterations had a significantly earlier age of onset of FS. This study confirms the high sensitivity of SCN1A for SMEI/SMEB phenotypes
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