PMU31 early cost-effectiveness analysis of continuous monitoring of lung-aeration with electrical impedance tomography in preterm neonates with respiratory distress syndrome

Objectives Respiratory distress syndrome (RDS) is relatively common in preterm neonates due to lung immaturity. Clinical management by respiratory support is associated with high complications rates. Guidance on appropriate lung-aeration is limited using conventional thorax X-ray monitoring. Electrical impedance tomography (EIT) allows radiation-free, continuous lung-aeration monitoring to guide effective respiratory support. EIT produces dynamic images of air volume changes whereas X-ray shows 2-D structure. Clinicians expect EIT implementation to reduce the number of patients requiring mechanical ventilation, overall complication rates and hospitalisation length. We conducted an early cost-effectiveness analysis of EIT-monitoring in preterm neonates with RDS versus standard care in the Netherlands. Methods A decision-analytic model was constructed comparing costs and effects of conventional X-ray versus EIT-monitoring for preterm neonates with RDS from the healthcare perspective with a time horizon of two years. Input parameters were based on literature and cost databases. The effects of EIT-monitoring were based on consensus by 6 clinical experts for two scenarios, (1) a conservative scenario assuming only a decrease of patients on mechanical ventilation under EIT-monitoring, and (2) an optimistic scenario including scenario (1) and assuming an additional 10% relative complication rate decrease in comparison to standard care. Main outcomes were total average costs per patient, number of patients with bronchopulmonary dysplasia (BPD), and mortality. One-way sensitivity analyses were conducted. Results EIT-monitoring was estimated to be cost-saving in both scenarios, mainly due to a shorter average hospital length of stay. Total incremental costs per patient for EIT-monitored care versus standard care were -€929 and -€10,706 for scenario (1) and (2), respectively. The number of patients with BPD and deaths were reduced. Results were robust to changes in input parameters. Conclusions EIT lung-aeration monitoring in preterm neonates is expected to result in cost-savings and lower mortality and BPD rates, in comparison to standard care, in a Dutch hospital setting. Copyright © 2019 Published by Elsevier Inc

The Administrative State en de noodzaak van constitutioneel onderhoud

The Legitimacy and Effectiveness of Law & Governance in a World of Multilevel Jurisdiction

Efficiënt wetgeven is meer dan snel wetten maken

Effective Protection of Fundamental Rights in a pluralist worl

Combining Efficiency and Transparency in Legislative Processes

In this contribution, we will illustrate the modern-day dynamics of the interplay between the need for expedience and efficiency on the one hand, and the demand for openness, inclusiveness and transparency on the other by looking into one of government’s main decision-making processes: the legislative process. Particularly in the field of legislation, the balancing of both efficiency and transparency is of the essence for modern legislatures in parliamentary democracies: laws expressed by acts and legislative instruments can only be truly effective if they rest on broad societal support. As we will argue, a transparent and inclusive legislative process functions as a kind of democratic check on government action: it guarantees sufficient deliberative activity before a government may act. Throughout our contribution, a 2012 comparative study commissioned by the Dutch Ministry of Security and Justice, and carried out by an interdisciplinary team of researchers from Leiden University will be used as a guiding rail to illustrate some the ways in which different jurisdictions in Europe have managed to combine, or at least balance, the need for legislative efficiency and transparency. We will use this study to demonstrate how traditional legislative processes nowadays grapple to translate the will of the citizens into effective legislation, how modern administrations still need democratically underpinned legislative procedures as the basis for the legitimation of (their) decisions, how efficient delivery of decisions and careful (lengthy) scrutiny interact. On the basis of this material we will further discuss concepts of, respectively, efficiency and transparency and especially the way modern legislatures examined in the study use information and communication technology (ICT) to overcome the sometimes opposing demands on their legislative processes. Insofar as possible we will try to highlight a few ‘best practices’ that show how legislative processes can (and cannot) adapt to new present day demands.The Legitimacy and Effectiveness of Law & Governance in a World of Multilevel Jurisdiction

Mastication and Oral Motor Function in McArdle Disease: Patient Reported Complaints

Background: Exertional myalgia and cramps of the limb and trunk muscles are typical in McArdle disease, but mastication and oral motor limitations have not been systematically investigated before. Objective: Determine the reported prevalence and characteristics of limitations on oral motor activities, mastication, swallowing, and other oral motor activities in patients with McArdle disease. Methods: An observational study was carried out in 28 patients using a standardised questionnaire on mastication and oral motor function. Results: 57% of the participants reported difficulties with mastication. Muscle cramps during mastication occurred in 57% of the patients. A feeling that food remains in the throat occurred in 32%. Adaptations during mastication were needed in 42% of the patients. Mastication difficulties, muscle cramps during mastication and mastication adjustments were inversely correlated with age (r2 = –0.445, p < 0.05; r2 = –0.509, p < 0.01; r2 = –0.456, p < 0.05). Feeling of food remaining in throat, cramps during mastication and during other oral motor muscle activities, were correlated with disease severity (r2 = 0.476, p < 0.01; r2 = 0.463, p < 0.01; r2 = 0.461, p < 0.01; r2 = 0.432, p < 0.05). Conclusions: In short, reported mastication difficulties are prevalent in people with McArdle disease, most frequently in younger people. Therefore, awareness of mastication and oral motor problems is require

Early onset as a marker for disease severity in facioscapulohumeral muscular dystrophy

Contains fulltext : 202651.pdf (publisher's version ) (Open Access)OBJECTIVE: To assess the relation between age at onset and disease severity in facioscapulohumeral muscular dystrophy (FSHD). METHODS: In this prospective cross-sectional study, we matched adult patients with FSHD with an early disease onset with 2 sex-matched FSHD control groups with a classic onset; the first group was age matched, and the second group was disease duration matched. Genetic characteristics, muscle performance, respiratory functioning, hearing loss, vision loss, epilepsy, educational level, and work status were compared with the 2 control groups. RESULTS: Twenty-eight patients with early-onset FSHD were age (n = 28) or duration (n = 27) matched with classic-onset patients. Patients with early-onset FSHD had more severe muscle weakness (mean FSHD clinical score 11 vs 5 in the age-matched and 9 in the duration-matched group, p < 0.05) and a higher frequency of wheelchair dependency (57%, 0%, and 30%, respectively, p < 0.05). In addition, systemic features were more frequent in early-onset FSHD, most important, hearing loss, decreased respiratory function and spinal deformities. There was no difference in work status. Genetically, the shortest D4Z4 repeat arrays (2-3 units) were found exclusively in the early-onset group, and the largest repeat arrays (8-9 units) were found only in the classic-onset groups. De novo mutations were more frequent in early-onset patients (46% vs 4%). CONCLUSIONS: Patients with early-onset FSHD more often have severe muscle weakness and systemic features. The disease severity is greater than in patients with classic-onset FSHD who are matched for disease duration, suggesting that the progression is faster in early-onset patients

Monocyte migration to the synovium in rheumatoid arthritis patients treated with adalimumab

Objectives The mechanism of action of treatment with tumour necrosis factor (TNF) blockers in rheumatoid arthritis (RA) is still not completely understood. The aim of this study was to test if adalimumab treatment could affect the influx of monocytes into the synovium. Methods A novel technique was used to analyse the migration of labelled autologous monocytes before and 14 days after initiation of adalimumab treatment using scintigraphy. CD14 monocytes were isolated from patients with RA, using a positive selection procedure with magnetic-activated cell sorting, and labelled with technetium-99m-hexamethylpropylene-amino-oxime. Scintigraphic scans were made 1, 2 and 3 h after re-infusion. Results As early as 14 days after the start of treatment with adalimumab a significant decrease in disease activity score evaluated in 28 joints was shown. There was no significant decrease in the influx of monocytes into the joint at this time. Conclusions This study indicates that adalimumab treatment does not reduce the influx of monocytes into the synovium early after initiation of treatment. As previous studies showed a rapid decrease in macrophage infiltration after TNF-antibody therapy, which could not be explained by increased cell death, this points to an important role for enhanced efflux of inflammatory cells from the synoviu

Legislative Processes in Transition. Comparative Study of the Legislative Processes in Finland, Slovenia and the United Kingdom as a Source of Inspiration for Enhancing the Efficiency of the Dutch Legislative Process

Effective Protection of Fundamental Rights in a pluralist worl