Systemic-onset juvenile idiopathic arthritis: the changing life of a rare disease.

Systemic-onset juvenile idiopathic arthritis (SoJIA), sometimes called Still's disease, is a systemic inflammatory disease classified within the spectrum of juvenile idiopathic arthritis (JIA). It is an orphan disease with often a chronic course and a major impact on the affected children and their families. This disorder is unique in terms of clinical manifestations, prognosis and response to conventional immunosuppressants. The objectives of this review are to describe SoJIA and emphasise the recent advances in the pathogenesis and treatment, which have transformed the care and the prognosis of this potentially life-threatening paediatric condition.

Long diagnostic delay in Crohn's disease is associated with complicated disease course and increased operation rate

Background: We have recently shown that the median diagnostic delay to establish Crohn's disease (CD) diagnosis in the Swiss IBD Cohort (SIBDC) was 9 months. Seventy five percent of all CD patients were diagnosed within 24 months. The clinical impact of a long diagnostic delay on the natural history of CD is unknown. Aim: To compare the frequency and type of CD-related complications in the patient groups with long diagnostic delay (>24 months) vs. the ones diagnosed within 24 months. Methods: Retrospective analysis of data from the SIBDCS, comprising a large sample of CD patients followed in hospitals and private practices across Switzerland. Results: Two hundred CD patients (121 female, mean age 44.9 ± 15.0 years, 38% smokers, 71% ever treated with immunomodulators and 35% with anti-TNF) with long diagnostic delay were compared to 697 CD patients (358 female, mean age 39.1 ± 14.9 years, 33% smokers, 74% ever treated with immunomodulators and 33% with anti-TNF) diagnosed within 24 months. No differences in the outcomes were observed between the two patient groups within year one after CD diagnosis. Among those diagnosed 2-5 years ago, CD patients with long diagnostic delay (n = 45) presented more frequently with internal fistulas (11.1% vs. 3.1%, p = 0.03) and bowel stenoses (28.9% vs. 15.7%, p = 0.05), and they more frequently underwent CD-related operations (15.6% vs. 5.0%, p = 0.02) compared to the patients diagnosed within 24 months (n = 159). Among those diagnosed 6-10 years ago, CD patients with long diagnostic delay (n = 48) presented more frequently with extraintestinal manifestations (60.4% vs. 34.6%, p = 0.001) than those diagnosed within 24 months (n = 182). For the patients diagnosed 11-15 years ago, no differences in outcomes were found between the long diagnostic delay group (n = 106) and the one diagnosed within 24 months (n = 32). Among those diagnosed >= 16 years ago, the group with long diagnostic delay (n = 71) more frequently underwent CD-related operations (63.4% vs. 46.5%, p = 0.01) compared to the group diagnosed with CD within 24 months (n = 241). Conclusions: A long diagnostic delay in CD patients is associated with a more complicated disease course and higher number of CD-related operations in the years following the diagnosis. Our results indicate that efforts should be undertaken to shorten the diagnostic delay in CD patients in order to reduce the risk for progression towards a complicated disease phenotype.

Dissecting the compression of mortality in Switzerland, 1876-2005

This paper aims to examine changes in common longevity and variability of the adult life span, and attempts to answer whether or not the compression of mortality continues in Switzerland in the years 1876-2005. The results show that the negative relationships between the large increase in the adult modal age at death, observed at least from the 1920s, and the decrease in the standard deviation of the ages at deaths occurring above it, illustrate a significant compression of adult mortality. Typical adult longevity increased by about 10% during the last fifty years in Switzerland, and adult heterogeneity in the age at death decreased in the same proportion. This analysis has not found any evidence suggesting that we are approaching longevity limits in term of modal or even maximum life spans. It ascertains a slowdown in the reduction of adult heterogeneity in longevity, already observed in Japan and other low mortality countries.

Addressing current treatment challenges in Crohn's disease in real life: A physician's survey.

BACKGROUND: In recent years several trials have addressed treatment challenges in Crohn's disease. Clinical trials however, represent a very special situation. AIMS: To perform a cross-sectional survey among gastroenterologists on the current clinical real life therapeutic approach focussing on the use of biologics. METHODS: A survey including six main questions on clinical management of loss of response, diagnostic evaluation prior to major treatment changes, preference for anti-tumour necrosis factor (TNF) agent, (de-)escalation strategies as well as a basic section regarding personal information was sent by mail to all gastroenterologists in Switzerland (n=318). RESULTS: In total, 120 questionnaires were analysed (response rate 37.7%). 90% of gastroenterologists in Switzerland use a thiopurine as the first step-up strategy (anti-TNF alone 7.5%, combination 2.5%). To address loss of response, most physicians prefer shortening the interval of anti-TNF administration followed by dose increase, switching the biologic and adding a thiopurine. In case of prolonged remission on combination therapy, the thiopurine is stopped first (52.6%) after a mean treatment duration of 15.7 months (biologic first in 41.4%). CONCLUSIONS: Everyday clinical practice in Crohn's disease patients appears to be incongruent with clinical data derived from major trials. Studies investigating reasons underlying these discrepancies are of need to optimize and harmonize treatment.

Good outcomes of Lyme arthritis in 24 patients in an endemic area of Switzerland

RESUME : But : Décrire l'évolution après traitement de l'arthrite de Lyme dans une zone d'endémie de l'Ouest de la Suisse, région où certains parmi les premiers cas d'arthrite de Lyme en dehors des Etats-Unis (USA) ont été rapportés. Patients et méthodes : Une évaluation rétrospective a été faite à partir d'un groupe de 24 patients (15 H, 9 F; âge moyen :38,7 ans) qui ont présenté une arthrite monoarticulaire (20 cas) ou oligoarticulaire (4 cas) causée par une infection à Borrelia burgdorferi (Bb). Ces patients ont été recrutés par l'intermédiaire de rhumatologues entre 1994 et 1999. Le genou était touché dans 85 % des cas. Une histoire de piqûre de tique était décrite dans 9 cas et un érythème chronique migrant dans 4 cas. Tous les patients avaient un titre d'anticorps pour Bb élevé, dosé par ELISA.Dans 20 cas, un immunoblot était positif pour Bb, la majorité étant positif pour les 3 sous-types de Bb présents en Suisse ; un seul cas était positif pour Bb sensu stricto. Neuf liquides synoviaux ont été examinés par PCR afin de détecter la présence de BbDNA (6 cas positifs). Résultats : Tous les patients ont reçu des antibiotiques soit oralement (10 cas), soit par voie parentérale (14 cas). Une deuxième cure d'antibiotiques a été administrée à 4 patients en raison de persistance de l'arthrite. On a observé une évolution rapidement favorable chez 13 patients et dans 9 cas, il a fallu, pour obtenir la guérison, réaliser une injection intraarticulaire de glucocorticoïdes ou une synoviorthèse. Après une période d'observation de 40 mois en moyenne (de 6 à 84 mois), aucun patient n'a présenté de signe d'arthrite chronique, mais 2 patients se plaignaient encore de myalgies ou d'arthralgies. Conclusion : Nous n'avons pas trouvé dans notre étude d'arthrite récidivante ou chronique après traitement comme on l'a décrit aux USA. Ceci est peut-être lié au fait que les types de Bb observés en Europe sont différents des USA où on trouve seulement Bb sensu stricto. ABSTRACT: Objective: To describe outcomes of treated Lyme arthritis in an endemic area of western Switzerland, where some of the first cases of Lyme disease outside the United States were reported. Patients and methods: We retrospectively studied 24 patients (15 males and nine females, mean age 38.7 years) managed by rheumatologists between 1994 and 1999 for Borrelia burgdotferi arthritis manifesting as monoarthritis (a = 20), oligoarthritis (a = 3), or polyarthritis (a = 1). The knee was affected in 20 (85%) patients. Nine patients reported a history of tick bite and four of erythema chronicum migrans. All the patients but one had a high titer of antibodies to B. burgdoiferi by ELISA and all but two had a positive immunoblot test (22 positive for all three types of B. burgdorferi found in Switzerland and one positive only for B. burgdoiferi sensu stricto). Joint fluid PCR for B. burgdorferi was done in nine patients and was positive in six. Results: All 24 patients received antibiotic therapy, orally (a= 10) or parenterally (n= 14). A second course of antibiotic therapy was used in four patients with persistent arthritis. A rapid response was noted in 13 patients. IntraarticUlar glucocorticoid therapy or a synoviorthesis was required in nine patients. After a mean follow-up of 40 months (range, 6-84 months), none of the patients had chronic arthritis but two reported persistent muscle or joint pain. Conclusion: Recurrent or chronic arthritis, which has been reported in treated patients in the United States, did not occur in our series. This may be ascribable to differences in B. burgdolferi subtypes, as in the United States only B. burgdoiferi sensu stricto is found.

Superior flow for bridge to life with self-expanding venous cannulas.

BACKGROUND: Recently, a compact cardiopulmonary support (CPS) system designed for quick set-up for example, during emergency cannulation, has been introduced. Traditional rectilinear percutaneous cannulas are standard for remote vascular access with the original design. The present study was designed to assess the potential of performance increase by the introduction of next-generation, self-expanding venous cannulas, which can take advantage of the luminal width of the venous vasculature despite a relatively small access orifice. METHODS: Veno-arterial bypass was established in three bovine experiments (69+/-10 kg). The Lifebridge (Lifebridge GmbH, Munich, Germany) system was connected to the right atrium in a trans-jugular fashion with various venous cannulas; and the oxygenated blood was returned through the carotid artery with a 17 F percutaneous cannula. Two different venous cannulas were studied, and the correlation between the centrifugal pump speed (1500-3900 RPM), flow and the required negative pressure on the venous side was established: (A) Biomedicus 19 F (Medtronic, Tolochenaz, Switzerland); (B) Smart canula 18 F/36 F (Smartcanula LLC, Lausanne, Switzerland). RESULTS: At 1500 RPM, the blood flow was 0.44+/-0.26 l min(-1) for the 19 F rectilinear cannula versus 0.73+/-0.34 l min(-1) for the 18/36 F self-expanding cannula. At 2500 RPM the blood flow was 1.63+/-0.62 l min(-1) for the 19F rectilinear cannula versus 2.13+/-0.34 l min(-1) for the 18/36 F self-expanding cannula. At 3500 RPM, the blood flow was 2.78+/-0.47 l min(-1) for the 19 F rectilinear cannula versus 3.64+/-0.39 l min(-1) for the 18/36 F self-expanding cannula (p<0.01 for 18/36 F vs 19 F). At 1500 RPM, the venous line pressure was 18+/-8 mmHg for the 19F rectilinear cannula versus 19+/-5 mmHg for the 18/36 F self-expanding cannula. At 2500 RPM the venous line pressure accounted for -22+/-32 mmHg for the 19 F rectilinear cannula versus 2+/-5 mmHg for the 18/36 F self-expanding cannula. At 3500 RPM, the venous line pressure was -112+/-42 mmHg for the rectilinear cannula versus 28+/-7 mmHg for the 18/36 F self-expanding cannula (p<0.01 for 18 F/36 F vs 19 F). Conclusions: The negative pressure required to achieve adequate venous drainage with the self-expanding venous cannula accounts for approximately 31% of the pressure necessary with the 19 F rectilinear cannula. In addition, a pump flow of more than 4 l min(-1) can be achieved with the self-expanding design and a well-accepted negative inlet pressure for minimal blood trauma of less than 50 mmHg.

Propionic acidemia: clinical course and outcome in 55 pediatric and adolescent patients.

BACKGROUND: Propionic acidemia is an inherited disorder caused by deficiency of propionyl-CoA carboxylase. Although it is one of the most frequent organic acidurias, information on the outcome of affected individuals is still limited. STUDY DESIGN/METHODS: Clinical and outcome data of 55 patients with propionic acidemia from 16 European metabolic centers were evaluated retrospectively. 35 patients were diagnosed by selective metabolic screening while 20 patients were identified by newborn screening. Endocrine parameters and bone age were evaluated. In addition, IQ testing was performed and the patients' and their families' quality of life was assessed. RESULTS: The vast majority of patients (>85%) presented with metabolic decompensation in the neonatal period. Asymptomatic individuals were the exception. About three quarters of the study population was mentally retarded, median IQ was 55. Apart from neurologic symptoms, complications comprised hematologic abnormalities, cardiac diseases, feeding problems and impaired growth. Most patients considered their quality of life high. However, according to the parents' point of view psychic problems were four times more common in propionic acidemia patients than in healthy controls. CONCLUSION: Our data show that the outcome of propionic acidemia is still unfavourable, in spite of improved clinical management. Many patients develop long-term complications affecting different organ systems. Impairment of neurocognitive development is of special concern. Nevertheless, self-assessment of quality of life of the patients and their parents yielded rather positive results.

Involvement of family physicians in structured programs for chronic diseases or multi-morbidity in Switzerland.

The increasing prevalence of chronic diseases and multi-morbidity represents challenges for health systems worldwide. In that perspective, the current organization of healthcare delivery, fragmentation of care, limited use of evidence-based guidelines and patients'insufficient empowerment are some reasons explaining the current limited effectiveness of the management of chronically ill patients. Based on theoretical models such as the Chronic Care Model (CCM), initiatives targeting improvements in the care of patients with chronic diseases have been implemented worldwide since more than a decade. Their development in Switzerland, a health system where more than half of practices are still single handed [6], is only recent and infrequent. Structured programs for patients with chronic diseases or multimorbidity usually propose patient-centered interventions and consider an integrative multidisciplinary approach. Currently, little is known on the existence of such programs and on the role of family physicians (FPs)within these programs, in Switzerland. The objective of this study was to identify and describe current structured programs targeting chronic diseases or multi-morbidity in Switzerland. This may help in examining innovative approaches that are only developed locally but would deserve wider interest for further implementation. We conducted a telephone-based survey between June and November 2013 and contacted systematically key institutions, informants and stakeholders nationwide and in the 26 cantons...

The real estate markets : Players, institutions and territories

Revealing the parties, the processes and the institutions and, consequently, both the diversity and contingency of the real estate markets, the existing increasing literature emphasises the contemporary numerous links and interdependencies between real estate, land value, planning and town planning policy and even the financial system. This paper is an attempt to understand all the real estate markets, from the most peripheral ones, where the urban rent is the lowest, to the most dense city centres. To gain a better understanding of the real estate market, a process of firstly deconstruction and then reconstruction is used. The process of deconstruction involves identifying various market trends according to property type (principally residential buildings), players and institutions, territorial situations and temporalities based on research conducted in Switzerland. We then developed a meta-synthesis inspired by Fernand Braudel whose works put as much emphasis on day-to-day economic activity as on long-term activity, and on local as well as global issues.

Impact of case management on frequent users quality of life : a randomized controlled trial

BACKGROUND: Frequent emergency department users represent a small number of patients but account for a large number of emergency department visits. They should be a focus because they are often vulnerable patients with many risk factors affecting their quality of life (QoL). Case management interventions have resulted in a significant decrease in emergency department visits, but association with QoL has not been assessed. One aim of our study was to examine to what extent an interdisciplinary case management intervention, compared to standard emergency care, improved frequent emergency department users' QoL. METHODS: Data are part of a randomized, controlled trial designed to improve frequent emergency department users' QoL and use of health-care resources at the Lausanne University Hospital, Switzerland. In total, 250 frequent emergency department users (≥5 attendances during the previous 12 months; ≥ 18 years of age) were interviewed between May 2012 and July 2013. Following an assessment focused on social characteristics; social, mental, and somatic determinants of health; risk behaviors; health care use; and QoL, participants were randomly assigned to the control or the intervention group (n=125 in each group). The final sample included 194 participants (20 deaths, 36 dropouts, n=96 in the intervention group, n=99 in the control group). Participants in the intervention group received a case management intervention by an interdisciplinary, mobile team in addition to standard emergency care. The case management intervention involved four nurses and a physician who provided counseling and assistance concerning social determinants of health, substance-use disorders, and access to the health-care system.

Incidence and outcomes of symptomatic neonatal arterial ischemic stroke.

BACKGROUND AND OBJECTIVES: Neonatal arterial ischemic stroke (NAIS) is associated with considerable lifetime burdens such as cerebral palsy, epilepsy, and cognitive impairment. Prospective epidemiologic studies that include outcome assessments are scarce. This study aimed to provide information on the epidemiology, clinical manifestations, infarct characteristics, associated clinical variables, treatment strategies, and outcomes of NAIS in a prospective, population-based cohort of Swiss children. METHODS: This prospective study evaluated the epidemiology, clinical manifestations, vascular territories, associated clinical variables, and treatment of all full-term neonates diagnosed with NAIS and born in Switzerland between 2000 and 2010. Follow-up was performed 2 years (mean 23.3 months, SD 4.3 months) after birth. RESULTS: One hundred neonates (67 boys) had a diagnosis of NAIS. The NAIS incidence in Switzerland during this time was 13 (95% confidence interval [CI], 11-17) per 100 000 live births. Seizures were the most common symptom (95%). Eighty-one percent had unilateral (80% left-sided) and 19% had bilateral lesions. Risk factors included maternal risk conditions (32%), birth complications (68%), and neonatal comorbidities (54%). Antithrombotic and antiplatelet therapy use was low (17%). No serious side effects were reported. Two years after birth, 39% were diagnosed with cerebral palsy and 31% had delayed mental performance. CONCLUSIONS: NAIS in Switzerland shows a similar incidence as other population-based studies. About one-third of patients developed cerebral palsy or showed delayed mental performance 2 years after birth, and children with normal mental performance may still develop deficits later in life.

"Ici la Suisse-Do isch t Schwyz-Switzerland calling!" : la société suisse de radiodiffusion (SSR) au service du rayonnement culturel helvetique (1932-1949)

La dialectique entre radiodiffusion et histoire des relations culturelles internationales est un domaine largement inexploré. L'objectif de cette thèse est d'analyser le rôle de la Société suisse de radiodiffusion (SSR), société privée qui exerce jusqu'en 1983 le monopole sur l'ensemble des stations de radio suisses, dans l'intensification des relations culturelles internationales de la Confédération. Pour examiner cette dimension des activités de la SSR, je me suis prioritairement penchée sur l'étude de la radio internationale helvétique, dénommée alors « Service suisse d'ondes courtes » (SOC). A l'instar de plusieurs organismes similaires à l'étranger, le SOC remplit dès ses débuts une double mission : resserrer les liens avec la diaspora et faire rayonner le pays hors des frontières nationales. Cette recherche met sur le devant de la scène un acteur médiatique aujourd'hui totalement oublié, le Service suisse d'ondes courtes. Par rapport à l'historiographie des radios internationales, elle mêle approche institutionnelle et, dans la mesure des sources disponibles, l'analyse de la programmation. Elle complète aussi l'histoire de la diplomatie culturelle suisse en rappelant la place du service public audiovisuel parmi les institutions chargées de promouvoir le pays à l'étranger. Pour finir, cette étude constitue également un apport à l'histoire des organisations internationales liées à la radiodiffusion (UIR, UIT). L'analyse du volet international des activités de la SSR a permis de dépasser la seule notion de « puissance » qui a été jusqu'à ces dernières années au coeur des ouvrages dévolus aux radios internationales. L'objectif poursuivi par la SSR ne réside pas tellement dans la diplomatie d'influence (l'exercice d'un « soft power »), qui tend à imposer ses valeurs et un mode de vie, mais plutôt dans la volonté de faire comprendre et reconnaître la culture politique de la Suisse dans le but de renforcer et pérenniser la place de celle-ci dans le concert des nations. Dans cette perspective, la culture devient un moyen utilisé pour transmettre à l'étranger une représentation valorisante du pays, une image de marque (une forme de « Nation Branding » avant l'heure) également utile au secteur touristique et à l'industrie d'exportation. Le Service suisse d'ondes courtes fait ainsi avant tout des relations publiques, un avant-goût de ce que les Américains appelleront dans les années 1960 la « public diplomacy »

CoDiab-VD: protocol of a prospective population-based cohort study on diabetes care in Switzerland.

BACKGROUND: Diabetes represents an increasing health burden worldwide. In 2010, the Public Health Department of the canton of Vaud (Switzerland) launched a regional diabetes programme entitled "Programme cantonal Diabète" (PcD), with the objectives to both decrease the incidence of diabetes and improve care for patients with diabetes. The cohort entitled CoDiab-VD emerged from that programme. It specifically aimed at following quality of diabetes care over time, at evaluating the coverage of the PcD within this canton and at assessing the impact of the PcD on care of patients with diabetes. METHODS/DESIGN: The cohort CoDiab-VD is a prospective population-based cohort study. Patients with diabetes were recruited in two waves (autumn 2011--summer 2012) through community pharmacies. Eligible participants were non-institutionalised adult patients (≥ 18 years) with diabetes diagnosed for at least one year, residing in the canton of Vaud and coming to a participating pharmacy with a diabetes-related prescription. Women with gestational diabetes, people with obvious cognitive impairment or insufficient command of French were not eligible. Self-reported data collected, included the following primary outcomes: processes-of-care indicators (annual checks) and outcomes of care such as HbA1C, (health-related) quality of life measures (Short Form-12 Health Survey--SF-12, Audit of Diabetes-Dependent Quality of Life 19--ADDQoL) and Patient Assessment of Chronic Illness Care (PACIC). Data on diabetes, health status, healthcare utilisation, health behaviour, self-management activities and support, knowledge of, or participation to, campaigns/activities proposed by the PcD, and socio-demographics were also obtained. For consenting participants, physicians provided few additional pieces of information about processes and laboratory results. Participants will be followed once a year, via a mailed self-report questionnaire. The core of the follow-up questionnaires will be similar to the baseline one, with the addition of thematic modules adapting to the development of the PcD. Physicians will be contacted every 2 years. DISCUSSION: CoDiab-VD will allow obtaining a broad picture of the care of patients with diabetes, as well as their needs regarding their chronic condition. The data will be used to evaluate the PcD and help prioritise targeted actions. TRIAL REGISTRATION: This study is registered with ClinicalTrials.gov, identifier NCT01902043, July 9, 2013.

Resident health-related quality of life in Swiss nursing homes.

BACKGROUND: Health-related quality of life (HRQOL) levels and their determinants in those living in nursing homes are unclear. The aim of this study was to investigate different HRQOL domains as a function of the degree of cognitive impairment and to explore associations between them and possible determinants of HRQOL. METHOD: Five HRQOL domains using the Minimum Data Set - Health Status Index (MDS-HSI) were investigated in a large sample of nursing home residents depending on cognitive performance levels derived from the Cognitive Performance Scale. Large effect size associations between clinical variables and the different HRQOL domains were looked for. RESULTS: HRQOL domains are impaired to variable degrees but with similar profiles depending on the cognitive performance level. Basic activities of daily living are a major factor associated with some but not all HRQOL domains and vary little with the degree of cognitive impairment. LIMITATIONS: This study is limited by the general difficulties related to measuring HRQOL in patients with cognitive impairment and the reduced number of variables considered among those potentially influencing HRQOL. CONCLUSION: HRQOL dimensions are not all linearly associated with increasing cognitive impairment in NH patients. Longitudinal studies are required to determine how the different HRQOL domains evolve over time in NH residents.