Mortality associated with neurofibromatosis type 1: A study based on Italian death certificates (1995-2006)

<p>Abstract</p> <p>Background</p> <p>Persons affected by neurofibromatosis type 1 (NF1) have a decreased survival, yet information on NF1-associated mortality is limited.</p> <p>Methods/Aim</p> <p>The National Mortality Database and individual Multiple-Causes-of-Death records were used to estimate NF1-associated mortality in Italy in the period 1995-2006, to compare the distribution of age at death (as a proxy of survival) to that of the general population and to evaluate the relation between NF1 and other medical conditions by determining whether the distribution of underlying causes of NF1-associated deaths differs from that of general population.</p> <p>Results</p> <p>Of the nearly 6.75 million deaths in the study period, 632 had a diagnosis of NF1, yet for nearly three-fourths of them the underlying cause was not coded as neurofibromatosis. The age distribution showed that NF1-associated deaths also occurred among the elderly, though mortality in early ages was high. The mean age for NF1-associated death was approximately 20 years lower than that for the general population. The gender differential may suggest that women are affected by more severe NF1-related complications, or they may simply reflect a greater tendency for NF1 to be reported on the death certificates of young women. Regarding the relation with other medical conditions, we found an excess, as the underlying cause of death, for malignant neoplasm of connective and other soft tissue and brain, but not for other sites. We also found an excess for obstructive chronic bronchitis and musculoskeletal system diseases among elderly persons.</p> <p>Conclusion</p> <p>This is the first nationally representative population-based study on NF1-associated mortality in Italy. It stresses the importance of the Multiple-Causes-of-Death Database in providing a more complete picture of mortality for conditions that are frequently not recorded as the underlying cause of death, or to study complex chronic diseases or diseases that have no specific International Classification of Diseases code, such as NF1. It also highlights the usefulness of already available data when a surveillance system is not fully operational.</p

Sudden Unexpected Deaths and Vaccinations during the First Two Years of Life in Italy: A Case Series Study

Background The signal of an association between vaccination in the second year of life with a hexavalent vaccine and sudden unexpected deaths (SUD) in the two days following vaccination was reported in Germany in 2003. A study to establish whether the immunisation with hexavalent vaccines increased the short term risk of SUD in infants was conducted in Italy. Methodology/Principal Findings The reference population comprises around 3 million infants vaccinated in Italy in the study period 1999–2004 (1.5 million received hexavalent vaccines). Events of SUD in infants aged 1–23 months were identified through the death certificates. Vaccination history was retrieved from immunisation registries. Association between immunisation and death was assessed adopting a case series design focusing on the risk periods 0–1, 0–7, and 0–14 days after immunisation. Among the 604 infants who died of SUD, 244 (40%) had received at least one vaccination. Four deaths occurred within two days from vaccination with the hexavalent vaccines (RR = 1.5; 95% CI 0.6 to 4.2). The RRs for the risk periods 0–7 and 0–14 were 2.0 (95% CI 1.2 to 3.5) and 1.5 (95% CI 0.9 to 2.4). The increased risk was limited to the first dose (RR = 2.2; 95% CI 1.1 to 4.4), whereas no increase was observed for the second and third doses combined. Conclusions The RRs of SUD for any vaccines and any risk periods, even when greater than 1, were almost an order of magnitude lower than the estimates in Germany. The limited increase in RRs found in Italy appears confined to the first dose and may be partly explained by a residual uncontrolled confounding effect of age

Rationale and design of an independent randomised controlled trial evaluating the effectiveness of aripiprazole or haloperidol in combination with clozapine for treatment-resistant schizophrenia

<p>Abstract</p> <p>Background</p> <p>One third to two thirds of people with schizophrenia have persistent psychotic symptoms despite clozapine treatment. Under real-world circumstances, the need to provide effective therapeutic interventions to patients who do not have an optimal response to clozapine has been cited as the most common reason for simultaneously prescribing a second antipsychotic drug in combination treatment strategies. In a clinical area where the pressing need of providing therapeutic answers has progressively increased the occurrence of antipsychotic polypharmacy, despite the lack of robust evidence of its efficacy, we sought to implement a pre-planned protocol where two alternative therapeutic answers are systematically provided and evaluated within the context of a pragmatic, multicentre, independent randomised study.</p> <p>Methods/Design</p> <p>The principal clinical question to be answered by the present project is the relative efficacy and tolerability of combination treatment with clozapine plus aripiprazole compared with combination treatment with clozapine plus haloperidol in patients with an incomplete response to treatment with clozapine over an appropriate period of time. This project is a prospective, multicentre, randomized, parallel-group, superiority trial that follow patients over a period of 12 months. Withdrawal from allocated treatment within 3 months is the primary outcome.</p> <p>Discussion</p> <p>The implementation of the protocol presented here shows that it is possible to create a network of community psychiatric services that accept the idea of using their everyday clinical practice to produce randomised knowledge. The employed pragmatic attitude allowed to randomly allocate more than 100 individuals, which means that this study is the largest antipsychotic combination trial conducted so far in Western countries. We expect that the current project, by generating evidence on whether it is clinically useful to combine clozapine with aripiprazole rather than with haloperidol, provides physicians with a solid evidence base to be directly applied in the routine care of patients with schizophrenia.</p> <p>Trial Registration</p> <p><b>Clincaltrials.gov Identifier</b>: NCT00395915</p

Oblique grain boundaries: analysis of light and electron beam induced current profiles in silicon

Asymmetric light and electron-beam induced current profiles have been previously attributed to a difference of the diffusion length on the two sides of the grain boundary. This feature is instead due to the grain boundary not being perpendicular to the surface. In this geometry, the equations describing the minority-carrier diffusion are not solvable by analytical procedures. A method for their numerical solution is introduced, fast enough to allow the use of minimization procedures. A good agreement is found between fitted values of the grain boundary inclination and those directly measured

Amenable mortality as a performance indicator of Italian health-care services

Abstract Background Mortality amenable to health-care services (‘amenable mortality’) has been defined as “premature deaths that should not occur in the presence of timely and effective health care” and as “conditions for which effective clinical interventions exist.” We analyzed the regional variability in health-care services using amenable mortality as a performance indicator. Convergent validity was examined against other indicators, such as health expenditure, GDP per capita, life expectancy at birth, disability-free life expectancy at age 15, number of diagnostic and laboratory tests per 1,000 inhabitants, and the prevalence of cancer and cardiovascular diseases. Methods Amenable mortality rate was calculated as the average annual number of deaths in the population aged 0–74 years per 100,000 inhabitants, and it was then stratified by gender and region. Data were drawn from national mortality statistics for the period 2006–08. Results During the study period (2006–08), the age-standardized death rate (SDR) amenable to health-care services in Italy was 62.6 per 100,000 inhabitants: 66.0 per 100,000 for males and 59.1 per 100,000 for females. Significant regional variations ranged from 54.1 per 100,000 inhabitants in Alto Adige to 76.3 per 100,000 in Campania. Regional variability in SDR was examined separately for male and females. The variability proved to be statistically significant for both males and females (males: Q-test = 638.5, p p = 0.002; female: r = −0.88, p p p Conclusions Amenable mortality shows a wide variation across Italian regions and an inverse relationship with life expectancy and GDP per capita, as expected.</p

Follow-up short and long-term mortalities of tracheostomized critically ill patients in an Italian multi-center observational study

Abstract The effects of tracheostomy on outcome as well as on intra or post-operative complications is yet to be defined. Admission of patients with tracheostomy to rehabilitation facility is at higher risk of suboptimal care and increased mortality. The aim of the study was to investigate ICU mortality, clinical outcome and quality of life up to 12 months after ICU discharge in tracheostomized critically ill patients. This is a prospective, multi-center, cohort study endorsed by Italian Society of Anesthesia, Analgesia, Reanimation, and Intensive Care (SIAARTI Prot. n° 643/13) registered in Clinicaltrial.gov (NCT01899352). Patients admitted to intensive care unit (ICU) and requiring elective tracheostomy according to physician in charge decision were included in the study. The primary outcome was ICU mortality. Secondary outcomes included risk factors for ICU mortality, prevalence of mortality at follow-up, rate of discharge from the hospital and rehabilitation, quality of life, performance status, and management of tracheostomy cannula at 3-, 6, 12-months from the day of tracheostomy. 694 critically ill patients who were tracheostomized in the ICU were included. ICU mortality was 15.8%. Age, SOFA score at the day of the tracheostomy, and days of endotracheal intubation before tracheostomy were risk factors for ICU mortality. The regression tree analysis showed that SOFA score at the day of tracheostomy and age had a preeminent role for the choice to perform the tracheostomy. Of the 694 ICU patients with tracheostomy, 469 completed the 12-months follow-up. Mortality was 33.51% at 3-months, 45.30% at 6-months, and 55.86% at 12-months. Patients with tracheostomy were less likely discharged at home but at hospital facilities or rehabilitative structures; and quality of life of patients with tracheostomy was severely compromised at 3–6 and 12 months when compared with patients without tracheostomy. In patients admitted to ICU, tracheostomy is associated with high mortality, difficult rehabilitation, and decreased quality of life. The choice to perform a tracheostomy should be carefully weighed on family burden and health-related quality of life. Clinical trial registration: Clinicaltrial.gov (NCT01899352)

La mortalita' in Italia nell'anno 1998

Consiglio Nazionale delle Ricerche - Biblioteca Centrale - P.le Aldo Moro, 7 , Rome / CNR - Consiglio Nazionale delle RichercheSIGLEITItal

Le cardinal Franz Ehrle (1845-1934)

Le cardinal Franz Ehrle (1845-1934), jésuite originaire de Souabe en Allemagne, est une figure majeure de l’histoire religieuse et intellectuelle de son temps. Il fut préfet de la Bibliothèque vaticane de 1895 à 1914 et devint, en 1922, cardinal puis bibliothécaire et archiviste de l’Église romaine en 1929. Savant de réputation internationale, il s’est intéressé aussi bien à l’histoire de l’Église médiévale qu’à celle de la scolastique, des ordres religieux et de la bibliothèque des papes. La Bibliothèque vaticane lui doit sa physionomie moderne. Proche des papes, de Léon XIII à Pie XI, il fut aussi un homme attentif aux problèmes de son temps. Son nom est resté familier aux historiens mais les divers aspects de sa vie et de son œuvre demeurent en fait inégalement et insuffisamment connus. En rassemblant quatorze contributions consacrées aux multiples facettes du savant, du préfet de la Bibliothèque vaticane et du cardinal, révélatrices de sa personnalité, de sa production historique et de ses orientations théologiques, ecclésiologiques et spirituelles, le présentvolume s’efforce de saisir, à la fois dans sa complexité et sa modernité, cette grande figure peut-être méconnue, témoindes défis intellectuels et religieux que l’Église eut à affronter au tournant des XIXe et XXe siècles.Der Jesuit Franz Kardinal Ehrle (1845-1934), aus Schwaben gebürtig, war eine herausragende Gestalt der Kirchengeschichteund des Geisteslebens seiner Zeit. Er leitete als Präfekt von 1895 bis 1914 die Vatikanische Bibliothek,wurde 1922 Kardinal und 1929 auch Bibliothekar und Archivar der Römischen Kirche. Das Interesse des Gelehrtenvon internationalem Rang richtete sich auf die Geschichte der mittelalterlichen Kirche, der Scholastik, derOrden und der Bibliothek der Päpste. Die Modernisierung der Vatikanischen Bibliothek mit bleibender Prägekraftist ihm zu verdanken, welcher das Vertrauen der Päpste, besonders Leos XIII. und Pius’ XI., gewann. Er standden Problemen seiner Zeit mit Aufmerksamkeit und Anteilnahme gegenüber. Der Name Ehrles begegnet in derGeschichtswissenschaft zwar heute noch, doch ist der Kenntnisstand seiner Person und seines Lebens wie auchseines Werkes bislang ungleichmäßig und unzureichend. Daher beleuchten die 14 Beiträge des vorliegenden Bandesdie vielfältigen Facetten des Gelehrten, Bibliothekspräfekten und Kardinals, seine Schriften, seine theologischen,ekklesiologischen und spirituellen Ausrichtungen. So stellt der Band die komplexen und modernen Züge diesergroßen Gestalt heraus, die zu einem wichtigen Zeugen der geistigen und geistlichen Herausforderungen wurde,denen sich die Kirche an der Wende vom 19. zum 20. Jahrhundert gegenübersah