Frequency of platelet type versus type 2B von Willebrand disease. An international registry-based study.

Less than 50 patients are reported with platelet type von Willebrand disease (PT-VWD) worldwide. Several reports have discussed the diagnostic challenge of this disease versus the closely similar disorder type 2B VWD. However, no systematic study has evaluated this dilemma globally. Over three years, a total of 110 samples/data from eight countries were analysed. A molecular approach was utilised, analysing exon 28 of the von Willebrand factor (VWF) gene, and in mutation negative cases the platelet GP1BA gene. Our results show that 48 cases initially diagnosed as putative type 2B/PT-VWD carried exon 28 mutations consistent with type 2B VWD, 17 carried GP1BA mutations consistent with a PT-VWD diagnosis, three had other VWD types (2A and 2M) and five expressed three non-previously published exon 28 mutations. Excluding 10 unaffected family members and one acquired VWD, 26 cases did not have mutations in either genes. Based on our study, the percentage of type 2B VWD diagnosis is 44% while the percentage of misdiagnosis of PT-VWD is 15%. This is the first large international study to investigate the occurrence of PT-VWD and type 2B VWD worldwide and to evaluate DNA analysis as a diagnostic tool for a large cohort of patients. The study highlights the diagnostic limitations due to unavailability/poor application of RIPA and related tests in some centres and proposes genetic analysis as a suitable tool for the discrimination of the two disorders worldwide. Cases that are negative for both VWF and GP1BA gene mutations require further evaluation for alternative diagnoses.

Internet com a laboratori virtual dins de l'ensenyament d'Enginyeria de Materials

L’objectiu principal d’aquest projecte és seleccionar i exposar una sèrie de recursos disponibles a Internet, que permetin complementar o facilitar l’aprenentatge dels continguts de la titulació d’Enginyeria de Materials. Al llarg del treball es presenten un total de 124 animacions de diferents temàtiques. La majoria d’elles provenen de pàgines web d’universitats i centres involucrats en l’estudi de materials. També s’exposa una animació d’elaboració pròpia, així com el fonament teòric en què es basa. Cal destacar que l’ús d’applets en l’ensenyament d’Enginyeria de Materials no implica una despesa econòmica extra, i que està d’acord amb les directrius de generar els mínims residus i de fomentar la reducció en origen, establertes en el Pla d’Acció Local per a la Sostenibilitat de la UAB. Gràcies també a la facilitat d’ús, els applets tenen un futur prometedor en l’ensenyament de moltes matèries.

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Induction chemotherapy, as part of an integrated plan of management for locally advanced cancers, is being practised throughout the world in independent, isolated departments in universities, teaching hospitals, and clinical schools. Frequently, however, teams are relatively unaware of the work being undertaken in other institutions, and this situation may slow further progress. This book aims to present the full range of management techniques and practices used in induction chemotherapy within one accessible volume. It provides up-to-date information on the pioneering and cutting edge practices employed in different institutions and documents the advantages of integrated treatment schedules. Patient selection is discussed, and each of the cancer types for which induction therapy has proved important is considered in detail. All who are responsible for the treatment of patients with locally advanced cancers will find this book to be an invaluable source of information. It will be particularly interesting for specialist oncologists aiming to set up and develop fully comprehensive cancer centres and for health administrators wishing to learn about the benefits of establishing such centres in strategic locations.

Task force IV: Clinical use of ambulatory blood pressure monitoring. Participants of the 1999 Consensus Conference on Ambulatory Blood Pressure Monitoring

OBJECTIVE: To reach a consensus on the clinical use of ambulatory blood pressure monitoring (ABPM). METHODS: A task force on the clinical use of ABPM wrote this overview in preparation for the Seventh International Consensus Conference (23-25 September 1999, Leuven, Belgium). This article was amended to account for opinions aired at the conference and to reflect the common ground reached in the discussions. POINTS OF CONSENSUS: The Riva Rocci/Korotkoff technique, although it is prone to error, is easy and cheap to perform and remains worldwide the standard procedure for measuring blood pressure. ABPM should be performed only with properly validated devices as an accessory to conventional measurement of blood pressure. Ambulatory recording of blood pressure requires considerable investment in equipment and training and its use for screening purposes cannot be recommended. ABPM is most useful for identifying patients with white-coat hypertension (WCH), also known as isolated clinic hypertension, which is arbitrarily defined as a clinic blood pressure of more than 140 mmHg systolic or 90 mmHg diastolic in a patient with daytime ambulatory blood pressure below 135 mmHg systolic and 85 mmHg diastolic. Some experts consider a daytime blood pressure below 130 mmHg systolic and 80 mmHg diastolic optimal. Whether WCH predisposes subjects to sustained hypertension remains debated. However, outcome is better correlated to the ambulatory blood pressure than it is to the conventional blood pressure. Antihypertensive drugs lower the clinic blood pressure in patients with WCH but not the ambulatory blood pressure, and also do not improve prognosis. Nevertheless, WCH should not be left unattended. If no previous cardiovascular complications are present, treatment could be limited to follow-up and hygienic measures, which should also account for risk factors other than hypertension. ABPM is superior to conventional measurement of blood pressure not only for selecting patients for antihypertensive drug treatment but also for assessing the effects both of non-pharmacological and of pharmacological therapy. The ambulatory blood pressure should be reduced by treatment to below the thresholds applied for diagnosing sustained hypertension. ABPM makes the diagnosis and treatment of nocturnal hypertension possible and is especially indicated for patients with borderline hypertension, the elderly, pregnant women, patients with treatment-resistant hypertension and patients with symptoms suggestive of hypotension. In centres with sufficient financial resources, ABPM could become part of the routine assessment of patients with clinic hypertension. For patients with WCH, it should be repeated at annual or 6-monthly intervals. Variation of blood pressure throughout the day can be monitored only by ABPM, but several advantages of the latter technique can also be obtained by self-measurement of blood pressure, a less expensive method that is probably better suited to primary practice and use in developing countries. CONCLUSIONS: ABPM or equivalent methods for tracing the white-coat effect should become part of the routine diagnostic and therapeutic procedures applied to treated and untreated patients with elevated clinic blood pressures. Results of long-term outcome trials should better establish the advantage of further integrating ABPM as an accessory to conventional sphygmomanometry into the routine care of hypertensive patients and should provide more definite information on the long-term cost-effectiveness. Because such trials are not likely to be funded by the pharmaceutical industry, governments and health insurance companies should take responsibility in this regard.

Less frequent dosing of erythropoiesis stimulating agents in patients undergoing dialysis: a European multicentre cost study.

OBJECTIVE: To calculate the variable costs involved with the process of delivering erythropoiesis stimulating agents (ESA) in European dialysis practices. METHODS: A conceptual model was developed to classify the processes and sub-processes followed in the pharmacy (ordering from supplier, receiving/storing/delivering ESA to the dialysis unit), dialysis unit (dose determination, ordering, receipt, registration, storage, administration, registration) and waste disposal unit. Time and material costs were recorded. Labour costs were derived from actual local wages while material costs came from the facilities' accounting records. Activities associated with ESA administration were listed and each activity evaluated to determine if dosing frequency affected the amount of resources required. RESULTS: A total of 21 centres in 8 European countries supplied data for 142 patients (mean) per hospital (range 42-648). Patients received various ESA regimens (thrice-weekly, twice-weekly, once-weekly, once every 2 weeks and once-monthly). Administering ESA every 2 weeks, the mean costs per patient per year for each process and the estimates of the percentage reduction in costs obtainable, respectively, were: pharmacy labour (10.1 euro, 39%); dialysis unit labour (66.0 euro, 65%); dialysis unit materials (4.11 euro, 61%) and waste unit materials (0.43 euro, 49%). LIMITATION: Impact on financial costs was not measured. CONCLUSION: ESA administration has quantifiable labour and material costs which are affected by dosing frequency.

Genetic analysis of the breeding system of an invasive subterranean termite, Reticulitermes santonensis, in urban and natural habitats.

Reticulitermes santonensis is a subterranean termite that invades urban areas in France and elsewhere where it causes damage to human-built structures. We investigated the breeding system, colony and population genetic structure, and mode of dispersal of two French populations of R. santonensis. Termite workers were sampled from 43 and 31 collection points, respectively, from a natural population in west-central France (in and around the island of Oleron) and an urban population (Paris). Ten to 20 workers per collection point were genotyped at nine variable microsatellite loci to determine colony identity and to infer colony breeding structure. There was a total of 26 colonies, some of which were spatially expansive, extending up to 320 linear metres. Altogether, the analysis of genotype distribution, F-statistics and relatedness coefficients suggested that all colonies were extended families headed by numerous neotenics (nonwinged precocious reproductives) probably descended from pairs of primary (winged) reproductives. Isolation by distance among collection points within two large colonies from both populations suggested spatially separated reproductive centres with restricted movement of workers and neotenics. There was a moderate level of genetic differentiation (F(ST) = 0.10) between the Oleron and Paris populations, and the number of alleles was significantly higher in Oleron than in Paris, as expected if the Paris population went through bottlenecks when it was introduced from western France. We hypothesize that the diverse and flexible breeding systems found in subterranean termites pre-adapt them to invade new or marginal habitats. Considering that R. santonensis may be an introduced population of the North American species R. flavipes, a breeding system consisting primarily of extended family colonies containing many neotenic reproductives may facilitate human-mediated spread and establishment of R. santonensis in urban areas with harsh climates.

Radio-frequency ablation as primary management of well-tolerated sustained monomorphic ventricular tachycardia in patients with structural heart disease and left ventricular ejection fraction over 30%.

AIMS: Patients with well-tolerated sustained monomorphic ventricular tachycardia (SMVT) and left ventricular ejection fraction (LVEF) over 30% may benefit from a primary strategy of VT ablation without immediate need for a 'back-up' implantable cardioverter-defibrillator (ICD). METHODS AND RESULTS: One hundred and sixty-six patients with structural heart disease (SHD), LVEF over 30%, and well-tolerated SMVT (no syncope) underwent primary radiofrequency ablation without ICD implantation at eight European centres. There were 139 men (84%) with mean age 62 ± 15 years and mean LVEF of 50 ± 10%. Fifty-five percent had ischaemic heart disease, 19% non-ischaemic cardiomyopathy, and 12% arrhythmogenic right ventricular cardiomyopathy. Three hundred seventy-eight similar patients were implanted with an ICD during the same period and serve as a control group. All-cause mortality was 12% (20 patients) over a mean follow-up of 32 ± 27 months. Eight patients (40%) died from non-cardiovascular causes, 8 (40%) died from non-arrhythmic cardiovascular causes, and 4 (20%) died suddenly (SD) (2.4% of the population). All-cause mortality in the control group was 12%. Twenty-seven patients (16%) had a non-fatal recurrence at a median time of 5 months, while 20 patients (12%) required an ICD, of whom 4 died (20%). CONCLUSION: Patients with well-tolerated SMVT, SHD, and LVEF > 30% undergoing primary VT ablation without a back-up ICD had a very low rate of arrhythmic death and recurrences were generally non-fatal. These data would support a randomized clinical trial comparing this approach with others incorporating implantation of an ICD as a primary strategy.

La Accesibilidad en los Bienes de Interés cultural: situación y necesidades

Anàlisi normativa d'àmbit europeu, nacional, autonòmic i municipal existent sobre Accessibilitat en l'edificació, Accessibilitat al patrimoni i Protecció de Béns Culturals i Patrimoni Històric. Identificació dels conflictes d'aplicació entre les normatives d'accessibilitat i la conservació del patrimoni històric

Análisis normativo

Anàlisi normativa d'àmbit europeu, nacional, autonòmic i municipal existent sobre Accessibilitat en l'edificació, Accessibilitat al patrimoni i Protecció de Béns Culturals i Patrimoni Històric. Identificació dels conflictes d'aplicació entre les normatives d'accessibilitat i la conservació del patrimoni històric

Edufree – Aplicació per a l’ensenyament

Sistema d’informació creat amb l’objectiu de gestionar via Web les matriculacions i avaluacions de l’alumnat per part dels centres docents. Entre altres funcionalitats, les principals són: manteniment de mòduls, matriculació d’alumnes al centre per la posterior inscripció als mòduls desitjats, creació de grups d’alumnes per tal de distribuir-los en classes per nivells i en un número adequat, assignació del professorat als mòduls i grups creats, avaluació dels alumnes i generació de documents que ens permet realitzar llistats dels alumnes i butlletins trimestrals amb les avaluacions realitzades.

La salut laboral dels docents estudi de les causes psicosocials del malestar docent

El treball de recerca que he realitzat porta per títol: "LA SALUT LABORAL DELS DOCENTS. ESTUDI DE LES CAUSES PSICOSOCIALS DEL MALESTAR DOCENT", perquè tenir una bona salut laboral i arribar cada dia a la feina amb il•lusió i optimisme són essencials per poder ser un bon professional en el camp de l’educació i saber transmetre als alumnes i als companys de treball: energia, vitalitat, entusiasme i ganes d’aprendre. La salut laboral és l’element clau de la satisfacció professional, de la qualitat de la feina educativa i de la consolidació dels centres com a veritables entorns d’aprenentatge. La qualitat personal dels docents i, per tant, la seva salut laboral, contribueixen a la qualitat educativa i a garantir uns veritables entorns inclusius d'aprenentatge. D'aquest treball inicial en sorgeix un segon volum que porta per títol: "CURS DE FORMACIÓ EN EDUCACIÓ EMOCIONAL PER A LA PREVENCIÓ DEL MALESTAR DOCENT", on es suggereix un currículum per treballar l'educació emocional dels docents i diverses activitats per fer un veritable creixement personal, que portin cap a la satisfacció professional dels docents.

La Transición de la mortalidad en el siglo XX: el caso de Tarragona

Resum L'estudi és una aproximació a l'anàlisi de l'evolució i de les característiques de la mortalitat a la província de Tarragona durant tot el segle XX. L'objectiu de la recerca és doble, per una part es descriu l'evolució de la mortalitat mitjançant la Transició Demogràfica i Epidemiològica i, per una altra, es desenvolupen algunes hipòtesis que tractaran d'explicar el procés de la Transició Sanitària mitjançant els factors determinants. En contra de l'argument monocausal (McKeown, 1976), els defensors de l'actuació multicausal (Livi Bacci, 1987; entre d'altres) podrien ser els promotors de la Teoria de la Transició Sanitària de Frenk en 1991. La multiplicitat de factors (culturals, educatius, socials, sanitaris...) i les variades interaccions entre ells, que protegeixen i resisteixen la malaltia, és el que podria explicar la diversitat de nivells de causes de malaltia i la mort entre les diferents àrees territorials i la seva cronologia. Resumen El estudio es una aproximación al análisis de la evolución y de las características de la mortalidad en la provincia de Tarragona durante todo el siglo XX. El objetivo de la investigación es doble, por una parte se describe la evolución de la mortalidad a través de la Transición Demográfica y Epidemiológica en la provincia y, por otra, se desarrollan algunas hipótesis que tratarán de explicar el proceso de la Transición Sanitaria a través de los factores determinantes. En contra del argumento monocausal (McKeown, 1976), los defensores de la actuación multicausal (Livi Bacci, 1987; entre otros) podrían ser los promotores de la Teoría de la Transición Sanitaria de Frenk en 1991. La multiplicidad de factores (culturales, educacionales, sociales, sanitarios…) y las variadas interacciones entre ellos, que protegen y resisten a la enfermedad, es lo que podría explicar la diversidad de niveles de causas de enfermedad y la muerte entre las diferentes áreas territoriales y su cronología. ---------- Palabras clave ---------- Demografía, mortalidad, Tarragona, transición epidemiológica, transición sanitaria, enfermedad, salud, esperanza de vida, administración pública

CAT (Curator Archiving Tool): improving access to web archives

PADICAT is the web archive created in 2005 in Catalonia (Spain ) by the Library of Catalonia (BC ) , the National Library of Catalonia , with the aim of collecting , processing and providing permanent access to the digital heritage of Catalonia . Its harvesting strategy is based on the hybrid model ( of massive harvesting . SPA top level domain ; selective compilation of the web site output of Catalan organizations; focused harvesting of public events) . The system provides open access to the whole collection , on the Internet . We consider necessary to complement the current search for new and visualization software with open source software tool, CAT ( Curator Archiving Tool) , composed by three modules aimed to effectively managing the processes of human cataloguing ; to publish directories where the digital resources and special collections ; and to offer statistical information of added value to end users. Within the framework of the International Internet Preservation Consortium meeting ( Vienna 2010) , the progress in the development of this new tool, and the philosophy that has motivated his design, are presented to the international community.

Severe traumatic brain injury in Switzerland - feasibility and first results of a cohort study.

BACKGROUND: We aimed to study the incidence and outcome of severe traumatic brain injury (TBI) in Switzerland and to test the feasibility of a large cohort study with case identification in the first 24 hours and 6-month follow-up. METHODS: From January to June 2005, we consecutively enrolled and followed up all persons with severe TBI (Abbreviated Injury Score of the head region >3 and Glasgow Coma Scale <9) in the catchment areas of 3 Swiss medical centres with neurosurgical facilities. The primary outcome was the Extended Glasgow Outcome Scale (GOSE) after 6 months. Secondary outcomes included survival, Functional Independence Mea - sure (FIM), and health-related quality of life (SF-12) at defined time-points up to 6 months after injury. RESULTS: We recruited 101 participants from a source population of about 2.47 million (ie, about 33% of Swiss population). The incidence of severe TBI was 8.2 per 100,000 person-years. The overall case fatality was 70%: 41 of 101 persons (41%) died at the scene of the accident. 23 of 60 hospitalised participants (38%) died within 48 hours, and 31 (53%) within 6 months. In all hospitalised patients, the median GOSE was 1 (range 1-8) after 6 months, and was 6 (2-8) in 6-month survivors. The median total FIM score was 125 (range 18-126); median-SF-12 component mea - sures were 44 (25-55) for the physical scale and 52 (32-65) for the mental scale. CONCLUSIONS: Severe TBI was associated with high case fatality and considerable morbidity in survivors. We demonstrated the feasibility of a multicentre cohort study in Switzerland with the aim of identifying modifiable determinants of outcome and improving current trauma care.