Proposal of a new clinical protocol for evaluating fatigability in adult SMA patients

Objective. Spinal Muscular Atrophy (SMA) is a genetic neuromuscular disease affecting the lower motor neuron, carrying a significant burden on patients’ general motor skills and quality of life, characterized by a great variability in phenotypic expression. As new therapeutic options make their appearance on the scene, sensitive clinical tools and outcome measures are needed, especially in adult patients undergoing treatment, in which the expected clinical response is a mild improvement or stabilization of disease progression. Methods. Here, we describe a new functional motor scale specifically designed for evaluating the endurance dimension for the upper and lower limbs in adult SMA patients. Results. The scale was first tested in eight control healthy subjects and then validated in ten adult SMA patients, proving intra- and inter-observer reliability. We also set up an evaluation protocol by using wearable devices including surface EMG and accelerometer. Conclusions. The endurance evaluation should integrate the standard clinical monitoring in the management and follow-up of SMA adult patients

The clinical relevance of oliguria in the critically ill patient : Analysis of a large observational database

Funding Information: Marc Leone reports receiving consulting fees from Amomed and Aguettant; lecture fees from MSD, Pfizer, Octapharma, 3 M, Aspen, Orion; travel support from LFB; and grant support from PHRC IR and his institution. JLV is the Editor-in-Chief of Critical Care. The other authors declare that they have no relevant financial interests. Publisher Copyright: © 2020 The Author(s). Copyright: Copyright 2020 Elsevier B.V., All rights reserved.Background: Urine output is widely used as one of the criteria for the diagnosis and staging of acute renal failure, but few studies have specifically assessed the role of oliguria as a marker of acute renal failure or outcomes in general intensive care unit (ICU) patients. Using a large multinational database, we therefore evaluated the occurrence of oliguria (defined as a urine output 16 years) patients in the ICON audit who had a urine output measurement on the day of admission were included. To investigate the association between oliguria and mortality, we used a multilevel analysis. Results: Of the 8292 patients included, 2050 (24.7%) were oliguric during the first 24 h of admission. Patients with oliguria on admission who had at least one additional 24-h urine output recorded during their ICU stay (n = 1349) were divided into three groups: transient - oliguria resolved within 48 h after the admission day (n = 390 [28.9%]), prolonged - oliguria resolved > 48 h after the admission day (n = 141 [10.5%]), and permanent - oliguria persisting for the whole ICU stay or again present at the end of the ICU stay (n = 818 [60.6%]). ICU and hospital mortality rates were higher in patients with oliguria than in those without, except for patients with transient oliguria who had significantly lower mortality rates than non-oliguric patients. In multilevel analysis, the need for RRT was associated with a significantly higher risk of death (OR = 1.51 [95% CI 1.19-1.91], p = 0.001), but the presence of oliguria on admission was not (OR = 1.14 [95% CI 0.97-1.34], p = 0.103). Conclusions: Oliguria is common in ICU patients and may have a relatively benign nature if only transient. The duration of oliguria and need for RRT are associated with worse outcome.publishersversionPeer reviewe

The Multilateral Trade Regime: Which Way Forward? <i>A Look at the Warwick Report</i>

In the light of the complex negotiations of the Doha Development Agenda, the Warwick Commission has been charged with carrying out independent analysis with the goal of making recommendations about how the World Trade Organization (WTO) should move forward. In December 2007, the Commission has released its fi rst report, entitled The Multilateral Trade Regime: Which Way Forward? This article attempts to review the most significant findings and proposals of the Report, and to criticize some of its shortcomings. The composition of the Commission and its focus almost exclusively on procedural issues constitute serious defects of the Report albeit not altering the value of the proposals. The author calls for a more representative study, carried out on more substantial issues. Importantly, multidisciplinary approaches would promote creative and constructive thinking about the future of the world trade.</jats:p

Investing in Culture: Underwater Cultural Heritage and International Investment Law

Underwater cultural heritage (UCH), which includes evidence of past cultures preserved in shipwrecks, enables the relevant epistemic communities to open a window to the unknown past and enrich their understanding of history. Recent technologies have allowed the recovery of more and more shipwrecks by private actors who often retrieve materials from shipwrecks to sell them. Not all salvors conduct proper scientific inquiry, conserve artifacts, and publish the results of the research; more often, much of the salvaged material is sold and its cultural capital dispersed. Because states rarely have adequate funds to recover ancient shipwrecks and manage this material, however, commercial actors seem to be necessary components of every regulatory framework governing UCH. In this context, this Article aims to reconcile private interests with the public interest in cultural heritage protection. Such reconciliation requires that international law be reinterpreted and reshaped in order to better protect and preserve UCH and that preservation of cultural heritage be recognized as a key component of economic, social, and cultural development