Damage Detection System with Sub-microsecond Resolution

Fiber optic grating sensors have been used to measure multi-dimensional strain, pressure, temperature, corrosion and moisture. This paper presents a method of using fiber grating sensors to measure the position and velocity of a very fast event associated with a blast wave. A chirped fiber grating of 50 mm length is placed in a highly energetic material. The action of the shock wave is to destroy the fiber grating as it propagates along it. By using a spectral filter such as a chirped fiber grating in combination with high speed detectors the position and velocity of the shock wave may be determined. A layout of a system used to experimentally verify this technique is described and results presented for two different highly energetic materials

Continuous monitoring of mining induced strain in a road pavement using fiber Bragg grating sensors

Abstrakt Få samhällsproblem har under de senaste åren mött så allmän uppmärksamhet som ungdomsbrottsligheten. Den polisanmälda ungdomsbrottsligheten har i stort sett följt brottslighetens utvecklingskurva avseende antal begångna brott, denna kurva visar en tydlig ökning sedan efterkrigstiden. Detta är en komparativ uppsats som är inriktad på ungdomsbrottsligheten i två av Malmö stadsdelar där vi undersöker antalet polisanmälda ungdomar till socialtjänsten mellan åren 2007-2009. Vi har studerat vilka brott ungdomarna var anmälda, analyserat brottstrender och i den mån det var möjligt granskat hur könsfördelningen har sett ut. Vi har undersökt vilka insatser som tillämpas från socialtjänstens håll för att motverka ungdomsbrottslighet samt förhindra fortsatt brottslighet hos redan polisanmälda ungdomar samt granska hur socialsekreterare vid våra undersökningsverksamheter upplever och uppfattar ungdomsbrottsligheten. Våra resultat har delvis ställts i relation till hur ungdomsbrottsligheten enligt tidigare forskning och redan befintlig statistik ser ut i Sverige. Vi har i uppsatsen använt oss av kvalitativ och kvantitativ datainsamlingsmetod samt ställt analysavsnitt och diskussionsavsnitt i relation till tidigare forskning och den av Travis Hirschis konstruerade teorin om sociala band. Studiens resultat visar bland annat att antalet polisanmälningar till socialtjänsterna har ökat, att killar begår fler brott än tjejer, att brottstyperna skiljer sig åt beroende på kön samt att ett mindre antal ungdomar ansvarar för en stor del av socialtjänsternas inkomna polisanmälningar

In-Situ Continuous Detonation Velocity Measurements Using Fiber-optic Bragg Grating Sensors

In order to fully calibrate hydrocodes and dynamic chemistry burn models, initiation and detonation research requires continuous measurement of low order detonation velocities as the detonation runs up to full order detonation for a given density and initiation pressure pulse. A novel detector of detonation velocity is presented using a 125 micron diameter optical fiber with an integral chirped fiber Bragg grating as an intrinsic sensor. This fiber is embedded in the explosive under study and interrogated during detonation as the fiber Bragg grating scatters light back along the fiber to a photodiode, producing a return signal dependant on the convolution integral of the grating reflection bandpass, the ASE intensity profile and the photodetector response curve. Detonation velocity is measured as the decrease in reflected light exiting the fiber as the grating is consumed when the detonation reaction zone proceeds along the fiber sensor axis. This small fiber probe causes minimal perturbation to the detonation wave and can measure detonation velocities along path lengths tens of millimeters long. Experimental details of the associated equipment and preliminary data in the form of continuous detonation velocity records within nitromethane and PBX-9502 are presented

Solving unsolved rare neurological diseases—a Solve-RD viewpoint

Funding Information: Funding The Solve-RD project has received funding from the European Union’s Horizon 2020 research and innovation programme under Grant Agreement No. 779257. Data were analysed using the RD‐Connect Genome‐Phenome Analysis Platform, which received funding from EU projects RD‐Connect, Solve-RD and EJP-RD (Grant Numbers FP7 305444, H2020 779257, H2020 825575), Instituto de Salud Carlos III (Grant Numbers PT13/0001/0044, PT17/0009/0019; Instituto Nacional de Bioinformática, INB) and ELIXIR Implementation Studies. The study was further funded by the Federal Ministry of Education and Research, Germany, through the TreatHSP network (01GM1905 to RS and LS), the National Institute of Neurological Diseases and Stroke (R01NS072248 to SZ and RS), the European Joint Program on Rare Diseases-EJP-RD COFUND-EJP N° 825575 through funding for the PROSPAX consortium (441409627 to MS, RS and BvW). CW was supported by the PATE program of the Medical Faculty, University of Tübingen. CEE received support from the Dutch Princess Beatrix Muscle Fund and the Dutch Spieren voor Spieren Muscle fund. Authors on this paper are members of the European Reference Network for Rare Neurological Diseases (ERN-RND, Project ID 739510). Funding Information: Conflict of interest HG receives/has received research support from the Deutsche Forschungsgemeinschaft (DFG), the Bundesministerium für Bildung und Forschung (BMBF), the Bundesministerium für Gesundheit (BMG) and the European Union (EU). He has received consulting fees from Roche. He has received a speaker honorarium from Takeda. The authors declare no competing interests.Peer reviewe

Dominantly inherited distal nemaline/cap myopathy caused by a large deletion in the nebulin gene

We report the first family with a dominantly inherited mutation of the nebulin gene (NEB). This 100kb in-frame deletion encompasses NEB exons 14-89, causing distal nemaline/cap myopathy in a three-generation family. It is the largest deletion characterized in NEB hitherto. The mutated allele was shown to be expressed at the mRNA level and furthermore, for the first time, a deletion was shown to cause the production of a smaller mutant nebulin protein. Thus, we suggest that this novel mutant nebulin protein has a dominant-negative effect, explaining the first documented dominant inheritance of nebulin-caused myopathy. The index patient, a young man, was more severely affected than his mother and grandmother. His first symptom was foot drop at the age of three, followed by distal muscle atrophy, slight hypomimia, high-arched palate, and weakness of the neck and elbow flexors, hands, tibialis anterior and toe extensors. Muscle biopsies showed myopathic features with type 1 fibre predominance in the index patient and nemaline bodies and cap-like structures in biopsies from his mother and grandmother. The muscle biopsy findings constitute a further example of nemaline bodies and cap-like structures being part of the same spectrum of pathological changes. (C) 2019 Elsevier B.V. All rights reserved.Peer reviewe

Vascular Complications of Pancreatitis: Role of Interventional Therapy

Major vascular complications related to pancreatitis can cause life-threatening hemorrhage and have to be dealt with as an emergency, utilizing a multidisciplinary approach of angiography, endoscopy or surgery. These may occur secondary to direct vascular injuries, which result in the formation of splanchnic pseudoaneurysms, gastrointestinal etiologies such as peptic ulcer disease and gastroesophageal varices, and post-operative bleeding related to pancreatic surgery. In this review article, we discuss the pathophysiologic mechanisms, diagnostic modalities, and treatment of pancreatic vascular complications, with a focus on the role of minimally-invasive interventional therapies such as angioembolization, endovascular stenting, and ultrasound-guided percutaneous thrombin injection in their management

Out-of-Frame Mutations in ACTN2 Last Exon Cause a Dominant Distal Myopathy With Facial Weakness

Background and Objectives To clinically, genetically, and histopathologically characterize patients presenting with an unusual combination of distal myopathy and facial weakness, without involvement of upper limb or shoulder girdle muscles. Methods Two families with a novel form of actininopathy were identified. Patients had been followed up over 10 years. Their molecular genetic diagnosis was not clear after extensive investigations, including analysis of candidate genes and FSHD1-related D4Z4 repeats. Results Patients shared a similar clinical phenotype and a common pattern of muscle involvement. They presented with a very slowly progressive myopathy involving anterior lower leg and facial muscles. Muscle MRI finding showed complete fat replacement of anterolateral compartment muscles of the lower legs with variable involvement of soleus and gastrocnemius but sparing thigh muscles. Muscle biopsy showed internalized nuclei, myofibrillar disorganization, and rimmed vacuoles. High-throughput sequencing identified in each proband a heterozygous single nucleotide deletion (c.2558del and c.2567del) in the last exon of the ACTN2 gene. The deletions are predicted to lead to a novel but unstructured slightly extended C-terminal amino acid sequence. Discussion Our findings indicate an unusual form of actininopathy with specific molecular and clinical features. Actininopathy should be considered in the differential diagnosis of distal myopathy combined with facial weakness.Peer reviewe

Delay in diagnosis of muscle disorders depends on the subspecialty of the initially consulted physician

<p>Abstract</p> <p>Background</p> <p>New therapeutic strategies in muscular dystrophies will make a difference in prognosis only if they are begun early in the course of the disease. Therefore, we investigated factors that influence the time to diagnosis in muscle dystrophy patients.</p> <p>Methods</p> <p>A sample of 101 patients (mean age 49 years; range 19-80; 44% women) with diagnosed muscle dystrophies from neurological practices and the neuromuscular specialty clinic in Berlin, Germany, was invited to participate. Time from first consultation to diagnosis, subspecialty of physician, and sociodemographic data were assessed with self-report questionnaires. The association between time to diagnosis and potential predictors (subspecialty of initially consulted physician, diagnoses, gender, and age at onset) was modeled with linear regression analysis.</p> <p>Results</p> <p>The mean time span between first health-care contact and diagnosis was 4.3 years (median 1). The diagnostic delay was significantly longer if patients were initially seen by a non-neurological specialist compared to a general practitioner (5.2 vs. 3.5 years, p = 0.047). Other factors that were independently associated with diagnostic delay were female gender and inherited muscle disease.</p> <p>Conclusion</p> <p>Action to improve clinical awareness of muscle diseases in non-neurological specialists is needed.</p

Unique Exercise Lactate Profile in Muscle phosphofructokinase Deficiency (Tarui Disease); Difference Compared with McArdle Disease

Introduction: Glycogen storage disease V (GSDV, McArdle disease) and GSDVII (Tarui disease) are the most common of the rare disorders of glycogen metabolism. Both are associated with low lactate levels on exercise. Our aim was to find out whether lactate response associated with exercise testing could distinguish between these disorders. Methods: Two siblings with Tarui disease, two patients with McArdle disease and eight healthy controls were tested on spiroergometric exercise tests with follow-up of venous lactate and ammonia. Results: A late increase of lactate about three times the basal level was seen 10-30 min after exercise in patients with Tarui disease being higher than in McArdle disease and lower than in the controls. Ammonia was increased in Tarui disease. Discussion: Our results suggest that follow-up of lactate associated with exercise testing can be utilized in diagnostics to distinguish between different GSD diseases.Peer reviewe