Long-term data from the Swiss pulmonary hypertension registry.

BACKGROUND Registries are important for real-life epidemiology on different pulmonary hypertension (PH) groups. OBJECTIVE To provide long-term data of the Swiss PH registry of 1998-2012. METHODS PH patients have been classified into 5 groups and registered upon written informed consent at 5 university and 8 associated hospitals since 1998. New York Heart Association (NYHA) class, 6-min walk distance, hemodynamics and therapy were registered at baseline. Patients were regularly followed, and therapy and events (death, transplantation, endarterectomy or loss to follow-up) registered. The data were stratified according to the time of diagnosis into prevalent before 2000 and incident during 2000-2004, 2005-2008 and 2009-2012. RESULTS From 996 (53% female) PH patients, 549 had pulmonary arterial hypertension (PAH), 36 PH due to left heart disease, 127 due to lung disease, 249 to chronic thromboembolic PH (CTEPH) and 35 to miscellaneous PH. Age and BMI significantly increased over time, whereas hemodynamic severity decreased. Overall, event-free survival was 84, 72, 64 and 58% for the years 1-4 and similar for time periods since 2000, but better during the more recent periods for PAH and CTEPH. Of all PAH cases, 89% had target medical therapy and 43% combination therapy. Of CTEPH patients, 14 and 2% underwent pulmonary endarterectomy or transplantation, respectively; 87% were treated with PAH target therapy. CONCLUSION Since 2000, the incident Swiss PH patients registered were older, hemodynamically better and mostly treated with PAH target therapies. Survival has been better for PAH and CTEPH diagnosed since 2008 compared with earlier diagnosis or other classifications.

The Impact of Human Asset Specificity on the Sourcing of Application Services

The question concerning the circumstances under which it is advantageous for a company to outsource certain information systems functions has been a controversial issue for the last decade. While opponents emphasize the risks of outsourcing based on the loss of strategic potentials and increased transaction costs, proponents emphasize the strategic benefits of outsourcing and high potentials of cost-savings. This paper brings together both views by examining the conditions under which both the strategic potentials as well as savings in production and transaction costs of developing and maintaining software applications can better be achieved in-house as opposed to by an external vendor. We develop a theoretical framework from three complementary theories and test it empirically based on a mail survey of 139 German companies. The results show that insourcing is more cost efficient and advantageous in creating strategic benefits through IS if the provision of application services requires a high amount of firm specific human assets. These relationships, however, are partially moderated by differences in the trustworthiness and intrinsic motivation of internal versus external IS professionals. Moreover, capital shares with an external vendor can lower the risk of high transaction costs as well the risk of loosing the strategic opportunities of an IS.

The Swiss National Registry for Primary Immunodeficiencies: report on the first 6 years' activity from 2008 to 2014

The Swiss National Registry for Primary Immunodeficiency Disorders (PID) was established in 2008, constituting a nationwide network of paediatric and adult departments involved in the care of patients with PID at university medical centres, affiliated teaching hospitals and medical institutions. The registry collects anonymized clinical and genetic information on PID patients and is set up within the framework of the European database for PID, run by the European Society of Immunodeficiency Diseases. To date, a total of 348 patients are registered in Switzerland, indicating an estimated minimal prevalence of 4·2 patients per 100 000 inhabitants. Distribution of different PID categories, age and gender are similar to the European cohort of currently 19 091 registered patients: 'predominantly antibody disorders' are the most common diseases observed (n = 217/348, 62%), followed by 'phagocytic disorders' (n = 31/348, 9%). As expected, 'predominantly antibody disorders' are more prevalent in adults than in children (78 versus 31%). Within this category, 'common variable immunodeficiency disorder' (CVID) is the most prevalent PID (n = 98/217, 45%), followed by 'other hypogammaglobulinaemias' (i.e. a group of non-classified hypogammaglobulinaemias) (n = 54/217, 25%). Among 'phagocytic disorders', 'chronic granulomatous disease' is the most prevalent PID (n = 27/31, 87%). The diagnostic delay between onset of symptoms and diagnosis is high, with a median of 6 years for CVID and more than 3 years for 'other hypogammaglobulinaemias'.

Patient outcomes after laminotomy, hemilaminectomy, laminectomy and laminectomy with instrumented fusion for spinal canal stenosis: a propensity score-based study from the Spine Tango registry.

PURPOSE To compare patient outcomes and complication rates after different decompression techniques or instrumented fusion (IF) in lumbar spinal stenosis (LSS). METHODS The multicentre study was based on Spine Tango data. Inclusion criteria were LSS with a posterior decompression and pre- and postoperative COMI assessment between 3 and 24 months. 1,176 cases were assigned to four groups: (1) laminotomy (n = 642), (2) hemilaminectomy (n = 196), (3) laminectomy (n = 230) and (4) laminectomy combined with an IF (n = 108). Clinical outcomes were achievement of minimum relevant change in COMI back and leg pain and COMI score (2.2 points), surgical and general complications, measures taken due to complications, and reintervention on the index level based on patient information. The inverse propensity score weighting method was used for adjustment. RESULTS Laminotomy, hemilaminectomy and laminectomy were significantly less beneficial than laminectomy in combination with IF regarding leg pain (ORs with 95% CI 0.52, 0.34-0.81; 0.25, 0.15-0.41; 0.44, 0.27-0.72, respectively) and COMI score improvement (ORs with 95% CI 0.51, 0.33-0.81; 0.30, 0.18-0.51; 0.48, 0.29-0.79, respectively). However, the sole decompressions caused significantly fewer surgical (ORs with 95% CI 0.42, 0.26-0.69; 0.33, 0.17-0.63; 0.39, 0.21-0.71, respectively) and general complications (ORs with 95% CI 0.11, 0.04-0.29; 0.03, 0.003-0.41; 0.25, 0.09-0.71, respectively) than laminectomy in combination with IF. Accordingly, the likelihood of required measures was also significantly lower after laminotomy (OR 0.28, 95% CI 0.17-0.46), hemilaminectomy (OR 0.28, 95% CI 0.15-0.53) and after laminectomy (OR 0.39, 95% CI 0.22-0.68) in comparison with laminectomy with IF. The likelihood of a reintervention was not significantly different between the treatment groups. DISCUSSION As already demonstrated in the literature, decompression in patients with LSS is a very effective treatment. Despite better patient outcomes after laminectomy in combination with IF, caution is advised due to higher rates of surgical and general complications and consequent required measures. Based on the current study, laminotomy or laminectomy, rather than hemilaminectomy, is recommendable for minimum relevant pain relief.

Superior outcomes of decompression with an interlaminar dynamic device versus decompression alone in patients with lumbar spinal stenosis and back pain: a cross registry study.

INTRODUCTION Surgical decompression for lumbar spinal stenosis (LSS) has been associated with poorer outcomes in patients with pronounced low back pain (LBP) as compared to patients with predominant leg pain. This cross registry study assessed potential benefits of the interlaminar coflex® device as an add-on to bony decompression alone. METHODS Patients with lumbar decompression plus coflex® (SWISSspine registry) were compared with decompressed controls (Spine Tango registry). Inclusion criteria were LSS and a preoperative back pain level of ≥5 points. 1:1 propensity score-based matching was performed. Outcome measures were back and leg pain relief, COMI score improvement, patient satisfaction, complication, and revision rates. RESULTS 50 matched pairs without residual significant differences but age were created. At the 7-9 months follow-up interval the coflex® group had higher back (p=0.014) and leg pain relief (p<0.001) and COMI score improvement (p=0.029) than the decompression group. Patient satisfaction was 90% in both groups. No revision was documented in the coflex® and one in the decompression group (2.0%). DISCUSSION In the short-term, lumbar decompression with coflex® compared with decompression alone in patients with LSS and pronounced LBP at baseline is a safe and effective treatment option that appears beneficial regarding clinical and functional outcomes. However, residual confounding of non-measured covariables may have partially influenced our findings. Also, despite careful inclusion and exclusion of cases the cross registry approach introduces a potential for selection bias that we could not totally control for and that makes additional studies necessary.

Pelvic Fractures in Children Results from the German Pelvic Trauma Registry: A Cohort Study.

As pelvic fractures in children and adolescents are very rare, the surgical management is not well delineated nor are the postoperative complications. The aim of this study using the prospective data from German Pelvic Trauma Registry study was to evaluate the various treatment approaches compared to adults and delineated the differences in postoperative complications after pelvic injuries.Using the prospective pelvic trauma registry established by the German Society of Traumatology and the German Section of the Arbeitsgemeinschaft für Osteosynthesefragen (AO), International in 1991, patients with pelvic fractures over a 12-year time frame submitted by any 1 of the 23 member level I trauma centers were reviewed.We identified a total of 13,525 patients including pelvic fractures in 13,317 adults and 208 children aged ≤14 years and compared these 2 groups. The 2 groups' Injury Severitiy Score (ISS) did not differ statistically. Lethality in the pediatric group was 6.3%, not statistically different from the adults' 4.6%. In all, 18.3% of the pediatric pelvic fractures were treated surgically as compared to 22.7% in the adult group. No child suffered any thrombosis/embolism, acute respiratory distress syndrome (ARDS), multiorgan failure (MOF), or neurologic deficit, nor was any septic MOF detected. The differences between adults and children were statistically significant in that the children suffered less frequently from thrombosis/embolism (P = 0.041) and ARDS and MOF (P = 0.006).This prospective multicenter study addressing patients with pelvic fractures reveals that the risk for a thrombosis/embolism, ARDS, and MOF is significant lower in pediatric patients than in adults. No statistical differences could be found in the ratios of operative therapy of the pelvic fractures in children compared to adults.

An international registry for primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder leading to chronic upper and lower airway disease. Fundamental data on epidemiology, clinical presentation, course and treatment strategies are lacking in PCD. We have established an international PCD registry to realise an unmet need for an international platform to systematically collect data on incidence, clinical presentation, treatment and disease course.The registry was launched in January 2014. We used internet technology to ensure easy online access using a web browser under www.pcdregistry.eu. Data from 201 patients have been collected so far. The database is comprised of a basic data form including demographic and diagnostic information, and visit forms designed to monitor the disease course.To establish a definite PCD diagnosis, we used strict diagnostic criteria, which required two to three diagnostic methods in addition to classical clinical symptoms. Preliminary analysis of lung function data demonstrated a mean annual decline of percentage predicted forced expiratory volume in 1 s of 0.59% (95% CI 0.98-0.22).Here, we present the development of an international PCD registry as a new promising tool to advance the understanding of this rare disorder, to recruit candidates for research studies and ultimately to improve PCD care.

Racial Disparities in Access to and Outcomes of Kidney Transplantation in Children, Adolescents, and Young Adults: Results From the ESPN/ERA-EDTA (European Society of Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association) Registry.

BACKGROUND Racial disparities in kidney transplantation in children have been found in the United States, but have not been studied before in Europe. STUDY DESIGN Cohort study. SETTING & PARTICIPANTS Data were derived from the ESPN/ERA-EDTA Registry, an international pediatric renal registry collecting data from 36 European countries. This analysis included 1,134 young patients (aged ≤19 years) from 8 medium- to high-income countries who initiated renal replacement therapy (RRT) in 2006 to 2012. FACTOR Racial background. OUTCOMES & MEASUREMENTS Differences between racial groups in access to kidney transplantation, transplant survival, and overall survival on RRT were examined using Cox regression analysis while adjusting for age at RRT initiation, sex, and country of residence. RESULTS 868 (76.5%) patients were white; 59 (5.2%), black; 116 (10.2%), Asian; and 91 (8.0%), from other racial groups. After a median follow-up of 2.8 (range, 0.1-3.0) years, we found that black (HR, 0.49; 95% CI, 0.34-0.72) and Asian (HR, 0.54; 95% CI, 0.41-0.71) patients were less likely to receive a kidney transplant than white patients. These disparities persisted after adjustment for primary renal disease. Transplant survival rates were similar across racial groups. Asian patients had higher overall mortality risk on RRT compared with white patients (HR, 2.50; 95% CI, 1.14-5.49). Adjustment for primary kidney disease reduced the effect of Asian background, suggesting that part of the association may be explained by differences in the underlying kidney disease between racial groups. LIMITATIONS No data for socioeconomic status, blood group, and HLA profile. CONCLUSIONS We believe this is the first study examining racial differences in access to and outcomes of kidney transplantation in a large European population. We found important differences with less favorable outcomes for black and Asian patients. Further research is required to address the barriers to optimal treatment among racial minority groups.

Total disc arthroplasty versus anterior cervical interbody fusion: use of the Spine Tango registry to supplement the evidence from randomized control trials.

BACKGROUND CONTEXT Several randomized controlled trials (RCTs) have compared patient outcomes of anterior (cervical) interbody fusion (AIF) with those of total disc arthroplasty (TDA). Because RCTs have known limitations with regard to their external validity, the comparative effectiveness of the two therapies in daily practice remains unknown. PURPOSE This study aimed to compare patient-reported outcomes after TDA versus AIF based on data from an international spine registry. STUDY DESIGN AND SETTING A retrospective analysis of registry data was carried out. PATIENT SAMPLE Inclusion criteria were degenerative disc or disc herniation of the cervical spine treated by single-level TDA or AIF, no previous surgery, and a Core Outcome Measures Index (COMI) completed at baseline and at least 3 months' follow-up. Overall, 987 patients were identified. OUTCOME MEASURES Neck and arm pain relief and COMI score improvement were the outcome measures. METHODS Three separate analyses were performed to compare TDA and AIF surgical outcomes: (1) mimicking an RCT setting, with admission criteria typical of those in published RCTs, a 1:1 matched analysis was carried out in 739 patients; (2) an analysis was performed on 248 patients outside the classic RCT spectrum, that is, with one or more typical RCT exclusion criteria; (3) a subgroup analysis of all patients with additional follow-up longer than 2 years (n=149). RESULTS Matching resulted in 190 pairs with an average follow-up of 17 months that had no residual significant differences for any patient characteristics. Small but statistically significant differences in outcome were observed in favor of TDA, which are potentially clinically relevant. Subgroup analyses of atypical patients and of patients with longer-term follow-up showed no significant differences in outcome between the treatments. CONCLUSIONS The results of this observational study were in accordance with those of the published RCTs, suggesting substantial pain reduction both after AIF and TDA, with slightly greater benefit after arthroplasty. The analysis of atypical patients suggested that, in patients outside the spectrum of clinical trials, both surgical interventions appeared to work to a similar extent to that shown for the cohort in the matched study. Also, in the longer-term perspective, both therapies resulted in similar benefits to the patients.

[Influence of the pelvic trauma registry of the DGU on treatment of pelvic ring fractures].

Fractures of the pelvic ring are comparatively rare with an incidence of 2-8 % of all fractures depending on the study in question. The severity of pelvic ring fractures can be very different ranging from simple and mostly "harmless" type A fractures up to life-threatening complex type C fractures. Although it was previously postulated that high-energy trauma was necessary to induce a pelvic ring fracture, over the past decades it became more and more evident, not least from data in the pelvic trauma registry of the German Society for Trauma Surgery (DGU), that low-energy minor trauma can also cause pelvic ring fractures of osteoporotic bone and in a rapidly increasing population of geriatric patients insufficiency fractures of the pelvic ring are nowadays observed with no preceding trauma.Even in large trauma centers the number of patients with pelvic ring fractures is mostly insufficient to perform valid and sufficiently powerful monocentric studies on epidemiological, diagnostic or therapeutic issues. For this reason, in 1991 the first and still the only registry worldwide for the documentation and evaluation of pelvic ring fractures was introduced by the Working Group Pelvis (AG Becken) of the DGU. Originally, the main objectives of the documentation were epidemiological and diagnostic issues; however, in the course of time it developed into an increasingly expanding dataset with comprehensive parameters on injury patterns, operative and conservative therapy regimens and short-term and long-term outcome of patients. Originally starting with 10 institutions, in the meantime more than 30 hospitals in Germany and other European countries participate in the documentation of data. In the third phase of the registry alone, which was started in 2004, data from approximately 15,000 patients with pelvic ring and acetabular fractures were documented. In addition to the scientific impact of the pelvic trauma registry, which is reflected in the numerous national and international publications, the dramatically changing epidemiology of pelvic ring fractures, further developments in diagnostics and the changes in operative procedures over time could be demonstrated. Last but not least the now well-established diagnostic and therapeutic algorithms for pelvic ring fractures, which could be derived from the information collated in registry studies, reflect the clinical impact of the registry.

First Annual IMACS Report: A global International Society for Heart and Lung Transplantation Registry for Mechanical Circulatory Support

The first annual report of the International Society for Heart and Lung Transplantation (ISHLT) Mechanically Assisted Circulatory Support (IMACS) registry provides global data on 5,942 patients from 31 countries. This initial report focuses on patient demographics, survival, device types, adverse events, competing outcomes, and a risk factor analysis.