Familial frontotemporal dementia with ubiquitin inclusion bodies and without motor neuron disease.

Frontotemporal dementia (FTD) is the second most common degenerative dementia after Alzheimer's disease and its Lewy body variant. Clinical pathology can be subdivided in three main neuropathological subtypes: frontal lobe dementia, Pick's disease and FTD with motor neuron disease (MND), all characterised by distinct histological features. Until recently the presence of ubiquitin-positive intraneuronal inclusions in the dentate gyrus, and the temporal and frontal cortex was usually associated with the MND type. Such inclusions were also observed in a few sporadic cases of FTD without or with parkinsonism (FTDP) in the absence of MND. We present here clinical, neuropathological and immunohistochemical data about a Swiss FTD family with FTDP-like features but without MND. Spongiosis and mild gliosis were observed in the grey matter. No neurofibrillary tangles, Pick bodies, Lewy bodies, senile plaques or prion-positive signals were present. However, ubiquitin-positive intracytoplasmic inclusions were detected in various structures but predominantly in the dentate gyrus. These observations support the existence of a familial form of FTDP with ubiquitin-positive intracytoplasmic inclusions (Swiss FTDP family).

Effect of Mechanical and Geometric Mismatching on Fatigue and Damage of Welded Joints

The future of high technology welded constructions will be characterised by higher strength materials and improved weld quality with respect to fatigue resistance. The expected implementation of high quality high strength steel welds will require that more attention be given to the issues of crack initiation and mechanical mismatching. Experiments and finite element analyses were performed within the framework of continuum damage mechanics to investigate the effect of mismatching of welded joints on void nucleation and coalescence during monotonic loading. It was found that the damage of undermatched joints mainly occurred in the sandwich layer and the damageresistance of the joints decreases with the decrease of the sandwich layer width. The damage of over-matched joints mainly occurred in the base metal adjacent to the sandwich layer and the damage resistance of the joints increases with thedecrease of the sandwich layer width. The mechanisms of the initiation of the micro voids/cracks were found to be cracking of the inclusions or the embrittled second phase, and the debonding of the inclusions from the matrix. Experimental fatigue crack growth rate testing showed that the fatigue life of under-matched central crack panel specimens is longer than that of over-matched and even-matched specimens. Further investigation by the elastic-plastic finite element analysis indicated that fatigue crack closure, which originated from the inhomogeneousyielding adjacent to the crack tip, played an important role in the fatigue crack propagation. The applicability of the J integral concept to the mismatched specimens with crack extension under cyclic loading was assessed. The concept of fatigue class used by the International Institute of Welding was introduced in the parametric numerical analysis of several welded joints. The effect of weld geometry and load condition on fatigue strength of ferrite-pearlite steel joints was systematically evaluated based on linear elastic fracture mechanics. Joint types included lap joints, angle joints and butt joints. Various combinations of the tensile and bending loads were considered during the evaluation with the emphasis focused on the existence of both root and toe cracks. For a lap joint with asmall lack-of-penetration, a reasonably large weld leg and smaller flank angle were recommended for engineering practice in order to achieve higher fatigue strength. It was found that the fatigue strength of the angle joint depended strongly on the location and orientation of the preexisting crack-like welding defects, even if the joint was welded with full penetration. It is commonly believed that the double sided butt welds can have significantly higher fatigue strength than that of a single sided welds, but fatigue crack initiation and propagation can originate from the weld root if the welding procedure results in a partial penetration. It is clearly shown that the fatigue strength of the butt joint could be improved remarkably by ensuring full penetration. Nevertheless, increasing the fatigue strength of a butt joint by increasing the size of the weld is an uneconomical alternative.

Structural factors impacting carrier transport and electroluminescence from Si nanocluster-sensitized Er ions.

We present an analysis of factors influencing carrier transport and electroluminescence (EL) at 1.5 µm from erbium-doped silicon-rich silica (SiOx) layers. The effects of both the active layer thickness and the Si excess content on the electrical excitation of erbium are studied. We demonstrate that when the thickness is decreased from a few hundred to tens of nanometers the conductivity is greatly enhanced. Carrier transport is well described in all cases by a Poole-Frenkel mechanism, while the thickness-dependent current density suggests an evolution of both density and distribution of trapping states induced by Si nanoinclusions. We ascribe this observation to stress-induced effects prevailing in thin films, which inhibit the agglomeration of Si atoms, resulting in a high density of sub-nm Si inclusions that induce traps much shallower than those generated by Si nanoclusters (Si-ncs) formed in thicker films. There is no direct correlation between high conductivity and optimized EL intensity at 1.5 µm. Our results suggest that the main excitation mechanism governing the EL signal is impact excitation, which gradually becomes more efficient as film thickness increases, thanks to the increased segregation of Si-ncs, which in turn allows more efficient injection of hot electrons into the oxide matrix. Optimization of the EL signal is thus found to be a compromise between conductivity and both number and degree of segregation of Si-ncs, all of which are governed by a combination of excess Si content and sample thickness. This material study has strong implications for many electrically driven devices using Si-ncs or Si-excess mediated EL.

Immersioultraäänitutkimuksen kehittäminen

Teräksenvalmistajilta edellytetään jatkuvasti panostusta laadun ja laadunvarmistuksen kehittämiseen. Teräksen laatu ja puhtaus korostuvat varsinkin silloin, kun terästä käytetään vaativiin käyttökohteisiin, kuten autoteollisuuden tarpeisiin. Ultraäänitarkastusmenetelmää käytetään laadun-varmistuksessa teräksen sisävikojen etsimiseen. Ultraäänitarkastuksessa lähetetään suuritaajuuksista ääntä kappaleeseen. Ääni etenee materiaalissa ja heijastuu erilaisista epäjatkuvuuskohdista. Luotaimeen palaavaa ääntä analysoimalla saadaan tietoa teräksestä ja sen sisävioista. Ultraäänitarkastuksen ongelmana on vian tyypin määrittäminen hankaluus sekä herkkyys tutkittavan materiaalin aineominaisuuksille. Työn tavoitteena oli immersioultraäänitarkastuksen kehittäminen sovellettuna teräksenvalmistajan tarpeisiin. Materiaalin aineominaisuuksista tutkittiin seostuksen vaikutusta. Teräslajit tarkastettiin valssitilaisena, karkaistuna ja normalisoituna. Lisäksi tutkittiin kappaleen pinnankarheuden ja -muodon vaikutusta tarkastukseen. Vikatyyppien tunnistamisen mahdollisuuksia ultra-äänitarkastuksessa selvitettiin käyttäen FFT- taajuusanalyysiä. Erilailla lämpökäsitellyillä teräslajeilla näytti eniten tuloksiin vaikuttavan terästen raekoko. Valssitilaisilla teräksillä raekoko on suuri, jolloin ultraääni vaimenee voimakkaasti teräksessä. Huomattavaa kuitenkin oli, että mikäli lämpö-käsittelyillä ei teräksen raekokoa saada juuri pienennettyä, ei myöskään vaimeneminen vähene. Tämän vuoksi lämpökäsittely ei välttämättä ole aina tarpeellinen valmisteltaessa ultraääninäytteitä. Fourier’n taajuusanalyysissä huomattiin olevan eroavaisuuksia verrattaessa huokosista ja sulkeumista palaavien kaikujen taajuusspektrejä. Näiden tulosten perusteella näyttäisi olevan mahdollista käyttää FFT- menetelmää ultraääni-tarkastuksessa vikojen luokitteluun.

Fluid flow processes at basin scale

S u b s u r face fluid flow plays a significant role in many geologic processes and is increasingly being studied in the scale of sedimentary basins and geologic time perspective. Many economic resources such as petroleum and mineral deposits are products of basin scale fluid flow operating over large periods of time. Such ancient flow systems can be studied through analysis of diagenetic alterations and fluid inclusions to constrain physical and chemical conditions of fluids and rocks during their paleohy d r og e o l ogic evolution. Basin simulation models are useful to complement the paleohy d r og e o l ogic record preserved in the rocks and to derive conceptual models on hydraulic basin evolution and generation of economic resources. Different types of fluid flow regimes may evo l ve during basin evolution. The most important with respect to flow rates and capacity for transport of solutes and thermal energy is gr avitational fluid flow driven by the topographic configuration of a basin. Such flow systems require the basin to be elevated above sea level. Consolidational fluid flow is the principal fluid migration process in basins below sea level, caused by loading of compressible rocks. Flow rates of such systems are several orders of magnitude below topogr a p hy driven flow. Howeve r, consolidation may create significant fluid ove rpressure. Episodic dewatering of ove rpressured compart m e n t s m ay cause sudden fluid release with elevated flow velocities and may cause a transient local thermal and chemical disequilibrium betwe e n fluid and rock. This paper gives an ove rv i ew on subsurface fluid flow processes at basin scale and presents examples related to the Pe n e d è s basin in the central Catalan continental margin including the offshore Barcelona half-graben and the compressive South-Pyrenean basin.

Hardness of FRHC-Cu determined by statistical analysis

A statistical indentation method has been employed to study the hardness value of fire-refined high conductivity copper, using nanoindentation technique. The Joslin and Oliver approach was used with the aim to separate the hardness (H) influence of copper matrix, from that of inclusions and grain boundaries. This approach relies on a large array of imprints (around 400 indentations), performed at 150 nm of indentation depth. A statistical study using a cumulative distribution function fit and Gaussian simulated distributions, exhibits that H for each phase can be extracted when the indentation depth is much lower than the size of the secondary phases. It is found that the thermal treatment produces a hardness increase, due to the partly re-dissolution of the inclusions (mainly Pb and Sn) in the matrix.

Opettajan monikulttuurinen työ

Teacher's multicultural work The purpose of the present study is to explore teachers’ conceptions of their work as teachers of multicultural students. Teachers’ experiences of multicultural work and conceptions derived from them are part of the teacher’s multicultural competence which is seen as a key component of the teacher’s multicultural teachership. The teacher’s multicultural competence consists of the teacher’s cultural knowledge, pedagogical skills and experiences and attitudes related to multiculturalism. The teacher’s multicultural competence constitutes the basis on which the teacher implements multicultural education. The foundation for the teacher’s work is laid by laws and decrees, curricula, regulations issued by authorities in charge of the education of immigrant students, resources available and other demands and expectations set by the ambient society. The study was conducted in the city of Turku, Finland. The sample consisted of class teachers who taught both immigrant and majority students. Main objects of study in the theoretical part are the multicultural and pluralistic school and the multicultural teachership. The basic assumption is that the multicultural and pluralistic school forms the frame of activity in which the teacher implements multicultural teaching. The research strategy is based on methodological triangulation. The quantitative part of the study was carried out using a questionnaire typical of survey methods. The questionnaire was returned by 71 teachers. The qualitative part was conducted using theme-based interviews typical of phenomenological philosophical research. Of the total of teachers who returned the questionnaire, twelve (12) teachers were selected for interviews. According to the results, the participating teachers enjoyed their work regardless of the ample extra work caused by the students with immigrant backgrounds. The teachers experienced their work as teachers of multicultural student groups as strenuous, yet challenging. Students with immigrant backgrounds had caused many changes in the teacher’s work. The teachers regarded their multicultural skills as inadequate in relation to the demands of the work. They had not received education related to teaching students with immigrant backgrounds, but they were ready for in-service education. The teachers’ previous attitudes concerning immigrant students had been enforced. Teaching experiences strengthened the earlier, both positive and negative, attitudes. The central problems related to multiculturalism in the teacher’s work were caused by the deficient Finnish skills of the students with immigrant background. This was apparent in both teaching and learning as well as in contacts with parents. The teachers reported on relatively few inclusions of multicultural angles in their teaching. However, they believed that they could aid students with different cultural backgrounds in their integration process. At the same time they felt that their own chances to enhance the students’ cultural identities were slim. On the basis of the interviews conducted in connection with the teacher’s multicultural competence, the teachers were divided into three groups: assimilative, indeterminate and integrating multicultural teachers. The present study provides a strong indication that teachers tend to interpret multiculturalism in narrow terms. School activities, such as Finnish as a second language, first language and religious instruction, which were targeted exclusively at immigrant students were in most cases considered adequate. A holistic, cross-disciplinary, all-inclusive multicultural education that would permeate all school activities remains largely unimplemented.

Permissivity of insect cells to Waddlia chondrophila, Estrella lausannensis and Parachlamydia acanthamoebae.

Recent large scale studies questioning the presence of intracellular bacteria of the Chlamydiales order in ticks and fleas revealed that arthropods, similarly to mammals, reptiles, birds or fishes, can be colonized by Chlamydia-related bacteria with a predominant representation of the Rhabdochlamydiaceae and Parachlamydiaceae families. We thus investigated the permissivity of two insect cell lines towards Waddlia chondrophila, Estrella lausannensis and Parachlamydia acanthamoebae, three bacteria representative of three distinct families within the Chlamydiales order, all documented in ticks and/or in other arthropods. We demonstrated that W. chondrophila and E. lausannensis are able to very efficiently multiply in these insect cell lines. E. lausannensis however induced a rapid cytopathic effect, which somehow restricted its replication. P. acanthamoebae was not able to grow in these cell lines even if inclusions containing a few replicating bacteria could occasionally be observed.

Trafficking of Estrella lausannensis in human macrophages.

Estrella lausannensis is a new member of the Chlamydiales order. Like other Chlamydia-related bacteria, it is able to replicate in amoebae and in fish cell lines. A preliminary study investigating the pathogenic potential of Chlamydia-related bacteria found a correlation between antibody response to E. lausannensis and pneumonia in children. To further investigate the pathogenic potential of E. lausannensis, we determined its ability to grow in human macrophages and its intracellular trafficking. The replication in macrophages resulted in viable E. lausannensis; however, it caused a significant cytopathic effect. The intracellular trafficking of E. lausannensis was analyzed by determining the interaction of the Estrella-containing inclusions with various endocytic markers as well as host organelles. The E. lausannensis inclusion escaped the endocytic pathway rapidly avoiding maturation into phagolysosomes by preventing both EEA-1 and LAMP-1 accumulation. Compared to Waddlia chondrophila, another Chlamydia-related bacteria, the recruitment of mitochondria and endoplasmic reticulum was minimal for E. lausannensis inclusions. Estrella lausannensis appears to use a distinct source of nutrients and energy compared to other members of the Chlamydiales order. In conclusion, we hypothesize that E. lausannensis has a restricted growth in human macrophages, due to its reduced capacity to control programmed cell death.

Interdisciplinary Medication Adherence Program: The Example of a University Community Pharmacy in Switzerland.

The Community Pharmacy of the Department of Ambulatory Care and Community Medicine (Policlinique Médicale Universitaire, PMU), University of Lausanne, developed and implemented an interdisciplinary medication adherence program. The program aims to support and reinforce medication adherence through a multifactorial and interdisciplinary intervention. Motivational interviewing is combined with medication adherence electronic monitors (MEMS, Aardex MWV) and a report to patient, physician, nurse, and other pharmacists. This program has become a routine activity and was extended for use with all chronic diseases. From 2004 to 2014, there were 819 patient inclusions, and 268 patients were in follow-up in 2014. This paper aims to present the organization and program's context, statistical data, published research, and future perspectives.

Le contrôle glycémique modéré est-il sans danger chez les patients adultes grièvement brûlés? = Is moderate glycemic control safe in major burns? : a 15 year cohort study

Introduction: L'hyperglycémie est un phénomène connu chez les patients gravement agressés, et surtout chez ceux nécessitant un séjour aux soins intensifs, alors que l'hypoglycémie est une complication menaçante. Des valeurs de glycémies anormales sont associées avec une mortalité et morbidité augmentées chez les patients de soins intensifs, y compris les grands brûlés. Des glycémies jusqu'à 15mmol/l ont longtemps été tolérées sans traitement. En 2001, une grande étude randomisée a complètement changé les pratiques du contrôle glycémique aux soins intensifs. Van den Berghe et al. ont montré qu'un contrôle glycémique strict atteint au moyen d'une « intensive insulin therapy » (HT) visant une glycémie 4.1-6.0 mmol/l réduisait la mortalité chez les patients chirurgicaux traités plus que 5. Par la suite plusieurs études contradictoires ont questionné la validité externe de l'étude de Louvain: avec la publication de l'étude « NICE-SUGAR » en 2009 enrôlant plus de 6000 patients cette hypothèse a été réfutée, aboutissant à un contrôle modéré de la glycémie (6-8 mmol/l). Bien que plusieurs études sur le contrôle glycémique aient également inclus quelques patients brûlés, à ce jour il n'y a pas de recommandation ferme concernant la gestion de la glycémie chez les patients brûlés adultes. Le but de l'étude était d'évaluer la sécurité du protocole de contrôle de la glycémie qui avait été introduit aux soins intensifs adultes chez des patients grand brûlés nécessitant un traitement prolongé aux soins intensifs. Méthodes : 11 s'agit d'une étude rétrospective uni-centrique sur des patients brûlés admis aux soins intensifs du CHUV à Lausanne entre de 2000 à juin 2014. Critères d'inclusions : Age >16 ans, brûlures nécessitant un traitement aux soins intensifs >10 jours. Critères d'exclusion : Décès ou transfert hors des soins intensifs <10 jours. Les investigations ont été limitées aux 21 premiers jours de l'hospitalisation aux soins intensifs. Variables : Variables démographiques, surface brûlée (TBSA), scores de sévérité, infections, durée d'intubation, durée du séjour aux soins intensifs, mortalité. Variables métaboliques : Administration totale de glucides, énergie et insuline/2411, valeurs de glycémie artérielle et CRP. Quatre périodes (P) ont été analysées, correspondant à l'évolution du protocole de contrôle de glycémie du service. P1: Avant son introduction (2000-2001) ; P2: Contrôle glycémie serré géré par les médecins (2002-2006) ; P3: Contrôle glycémie serré géré par lés infirmières (2007-2010); P4: Contrôle modéré géré par les infirmières (2011-2014). Les limites glycémiques ont été définis de manière suivante: Hypoglycémie extrême <2.3mmol/l ; hypoglycémie modéré <4.0mmol/l ; hyperglycémie modérée 8.1-10.0mmol/l ; hyperglycémie sévère >10.0mmol/l. Toutes les valeurs de glycémies artérielles ont été extraites depuis le système informatisé des soins intensifs (MetaVision ®). Statistiques: Wilcoxon rank test, Two- way Anova, Tuckey Kramer test, area under the curve (AUC), Spearman's test et odds ratio. STATA 12 1 ' StataCorp, College station, TX, USA and JPM V 10.1 (SAS Institute, Cary, NC, USA). Résultats: Sur les 508 patients brûlés admis durant la période étudiée, 229 patients correspondaient aux critères d'inclusion, âgés de 45±20ans (X±SD) et brûlés sur 32±20% de la surface corporelle. Les scores de sévérité sont restés stables. Au total 28'690 glycémies artérielles ont été analysées. La valeur médiane de glycémie est restée stable avec une diminution progressive de la variabilité intra-patient. Après initiation du protocole, les valeurs normoglycémiques ont augmenté de 34.7% à 65.9% avec diminution des événements hypoglycémiques (pas d'hypoglycémie extrême en P4). Le nombre d'hyperglycémies sévères est resté stable durant les périodes 1 à 3, avec une diminution en P4 (9.25%) : les doses d'insuline ont aussi diminué. L'interprétation des résultats de P4 a été compliquée par une diminution concomitante des apports d'énergie et de glucose (p<0.0001). Conclusions: L'application du protocole destiné aux patients de soins intensifs non brûlés a amélioré le contrôle glycémique chez les patients adultes brûlés, aboutissant à une diminution significative de la variabilité des glycémies. Un contrôle modéré de la glycémie peut être appliqué en sécurité, considérant le nombre très faible d'hypoglycémies. La gestion du protocole par les infirmières s'avère plus sûre qu'un contrôle par les médecins, avec diminution des hypoglycémies. Cependant le nombre d'hyperglycémies reste trop élevé. L'hyperglycémie' n'est pas contrôlable uniquement par l'administration d'insuline, mais nécessite également une approche multifactorielle comprenant une optimisation de la nutrition adaptée aux besoins énergétiques élevés des grands brûlés. Plus d'études seront nécessaire pour mieux comprendre la complexité du mécanisme de l'hyperglycémie chez le patient adulte brûlé et pour en améliorer le contrôle glycémique.

Leimuhitsauksen kokonaisvaltainen laadunhallinta päättymättömässä valssauksessa

Leimuhitsauksen laadunhallinnassa tärkeää on prosessimainen toiminta ja toimintojen kokonaisvaltaisuuden hahmottaminen. Leimuhitsauksen ollessa osana päättymätöntä valssausta hitsauksen laadunhallinta voidaan jakaa kolmeen päävaiheeseen: ennen hitsausta, hitsauksen aikana ja hitsauksen jälkeen vaikuttaviin laaduntuottotekijöihin. Leimuhitsauksen laaduntuottotekijöiden määritys ja jaottelu kaavioiksi on toteutettu tässä työssä. Leimuhitsin tekniseen laatuun vaikuttavat monet ilmiöt ja parametrit hitsauksen aikana. Tärkeimpiä hitsausparametreista ovat hitsausjännite, alustan liikenopeus, tyssäysmatka sekä leimutusaika. Väärät hitsausolosuhteet tai hitsausparametrit aiheuttavat erilaisia mekaanisia ja metallurgisia vikoja leimuhitsiin. Eräs metallurginen vikatyyppi ovat oksidisulkeumat hitsialueella. Nämä hapettuneet alueet voivat johtaa juurensa erilaisista syistä ja esimerkiksi leimutusajan ja epätasaisen liitospinnan yhteyttä sulkeumien syntyyn on epäilty. Pidennetyillä leimutusajoilla tehtyjen hitsauskokeiden ja hitsien rikkovien aineenkoetuskokeiden tuloksena todettiin tässä tutkittujen koehitsien oksidisulkeumien syntyvän hapettumalla leimutuksen aikana tai juuri hetkellä ennen tyssäystä, minkä lisäksi kosteat olosuhteet hitsausatmosfäärin ympärillä heikentävät lopputuloksen laatua. Leimutusajalla on tärkein osa leimutettavia pintoja tasaavana tekijänä ja mitä epätasaisempi liitettävä pinta on, sitä pidemmän leimutusajan se tarvitsee.

Influence of initial CaO/SiO2 ratio on the hydration of rice husk ash-Ca(OH)2 and sugar cane bagasse ash-Ca(OH)2 pastes

This work presents the results of a study on the hydration of pastes containing calcium hydroxide and either rice husk ash (RHA) or sugar cane bagasse ash (SCBA) in various initial CaO/SiO2 molar ratios. The products of the reactions were characterized by thermal analyses X-ray diffraction, and scanning electron microscopy. In the case of the RHA pastes, the product was composed of CaO-SiO2-H2O (type I C-S-H) or CaO-SiO2-H2O (type II C-S-H) according to the CaO/SiO2 ratio of the mixture. In contrast, in the case of the SBCA pastes, the product was composed primarily of CaO-SiO2-H2O that differed from both the previous types; the product also contained inclusions of calcium aluminate hydrates.

Mosaic in Senna occidentalis in southern Brazil induced by a new strain of Soybean mosaic virus

Plants of Senna occidentalis (sin. Cassia occidentalis) with mosaic symptoms were collected near a soybean (Glycine max) field where some plants exhibited symptoms of mosaic and blistering. A preliminary examination of leaf tissue from diseased S. occidentalis by electron microscopy revealed the presence of pinwheel inclusions as well as long flexuous particles, indicating the presence of a potyvirus. Host range, serology, and amino acid sequence from this potyvirus were similar to those from other Brazilian isolates of Soybean mosaic virus (SMV). The 3'- terminal region of the genomic RNA was cloned and a cDNA sequence of 1.9 kb upstream of the poly (A) tract was determined. The sequence contains a single open reading frame and a 3'- non-translated region (NTR) of 259 bp. The nucleotide sequence of the CP gene of SMV-Soc was 98% identical to that of Brazilian isolates SMV-B, SMV-L, and SMV-FT10. The percentage of nucleotide identity of their 3'-NTR's was 91, 98, and 99% in relation to SMV-L, SMV-B, and SMV-FT10, respectively. In contrast to other Brazilian SMV isolates studied, SMV-Soc was able to infect sunflower (Helianthus annuus). Based on these results, the S. occidentalis isolate was identified as a new strain of SMV belonging to the SMV strain, group G5 and was named SMV-Soc. This is the first report of naturaly occurring SMV infecting plants of S. occidentalis in Brazil, adding this weed as a new source of SMV in the field.