Sonorous memory in Jonathan Perel’s El predio (2010) and Los murales (2011)

Throughout his filmic production, Argentine director Jonathan Perel has demonstrated strict adherence to a unique aesthetic programme in which human agents appear to have only a minimal role. Each film contains only diegetic sounds and consists of fixed shots of architectural spaces and objects closely associated with the most recent Argentine military dictatorship (1976–1983) and recent attempts to memorialise the atrocities they committed. Through the close analysis of Perel’s first two films – El predio (2010) and Los murales (2011) – this article focusses on Perel’s highly distinctive use of environmental sound and argues that they are, in fact, uniquely musical works. Drawing on the work of John Cage, Michel Chion, Deleuze and Guattari, and Doreen Massey, the article proposes that Perel manipulates sound in order to situate debates over the memorialisation of recent atrocities in a perpetual present and thus critique contemporary abuses of power in Argentina

Impact of Vutrisiran on Quality of Life and Physical Function in Patients with Hereditary Transthyretin-Mediated Amyloidosis with Polyneuropathy

INTRODUCTION: Hereditary transthyretin (ATTRv; v for variant) amyloidosis, also known as hATTR amyloidosis, is a progressive and fatal disease associated with rapid deterioration of physical function and patients' quality of life (QOL). Vutrisiran, a subcutaneously administered RNA interference (RNAi) therapeutic that reduces hepatic production of transthyretin, was assessed in patients with ATTRv amyloidosis with polyneuropathy in the pivotal HELIOS-A study. METHODS: The phase 3 open-label HELIOS-A study investigated the efficacy and safety of vutrisiran in patients with ATTRv amyloidosis with polyneuropathy, compared with an external placebo group from the APOLLO study of the RNAi therapeutic patisiran. Measures of QOL and physical function were assessed. RESULTS: At month 18, vutrisiran improved Norfolk Quality of Life-Diabetic Neuropathy (Norfolk QOL-DN) total score (least squares mean difference [LSMD] in change from baseline [CFB]: –21.0; p = 1.84 × 10–10) and Norfolk QOL-DN domain scores, compared with external placebo. This benefit relative to external placebo was evident across all baseline polyneuropathy disability (PND) scores and most pronounced in patients with baseline PND scores I–II. Compared with external placebo, vutrisiran also demonstrated benefit in EuroQoL-Visual Analog Scale (EQ-VAS) score (LSMD in CFB: 13.7; nominal p = 2.21 × 10–7), 10-m walk test (LSMD in CFB: 0.239 m/s; p = 1.21 × 10–7), Rasch-built Overall Disability Score (LSMD in CFB: 8.4; p = 3.54 × 10–15), and modified body mass index (mBMI) (LSMD in CFB: 140.7; p = 4.16 × 10–15) at month 18. Overall, Norfolk QOL-DN, EQ-VAS, and mBMI improved from pretreatment baseline with vutrisiran, whereas all measures worsened from baseline in the external placebo group. At month 18, Karnofsky Performance Status was stable/improved from baseline in 58.2/13.1% with vutrisiran versus 34.7/8.1% with external placebo. CONCLUSION: Vutrisiran treatment provided significant clinical benefits in multiple measures of QOL and physical function in patients with ATTRv amyloidosis with polyneuropathy. Benefits were most pronounced in patients with earlier-stage disease, highlighting the importance of early diagnosis and treatment

Diagnosis and Treatment of Hereditary Transthyretin Amyloidosis (hATTR) Polyneuropathy: Current Perspectives on Improving Patient Care

Marco Luigetti,1,2 Angela Romano,2 Andrea Di Paolantonio,2 Giulia Bisogni,3 Mario Sabatelli2,3 1Neurology Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy; 2Università Cattolica del Sacro Cuore, Rome, Italy; 3Centro Clinico NEMO Adulti, Rome, ItalyCorrespondence: Marco LuigettiNeurology Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo A Gemelli 8, Rome 00168, ItalyTel +39-06-3015 4435Fax +39-06-3550 1909Email [email protected]: Hereditary transthyretin amyloidosis (hATTR) with polyneuropathy (formerly known as Familial Amyloid Polyneuropathy) is a rare disease due to mutations in the gene encoding transthyretin (TTR) and characterized by multisystem extracellular deposition of amyloid, leading to dysfunction of different organs and tissues. hATTR amyloidosis represents a diagnostic challenge for neurologists considering the great variability in clinical presentation and multiorgan involvement. Generally, patients present with polyneuropathy, but clinicians should consider the frequent cardiac, ocular and renal impairment. Especially a hypertrophic cardiomyopathy, even if usually latent, is identifiable in at least 50% of the patients. Therapeutically, current available options act at different stages of TTR production, including synthesis inhibition (liver transplantation and/or gene-silencing drugs) or tetramer TTR stabilization (TTR stabilizers), increasing survival at different disease stages.Keywords: amyloid, polyneuropathy, clinical care, therapy, transthyreti

R-CMMN: A Tool to Design Resilient Aware Multi-party Business Processes

Today business organizations operate in digital ecosystems that can be conceptualized in terms of multi-party business processes, where co-operation among parties is mandatory. Being every party a potential source of failures with impacts on the entire ecosystem, resilience is a feature that should be enforced by multi-party business processes directly at design-time, to anticipate what should be done in case of possible occurring failures at run-time. In this direction, this paper presents R-CMMN, a modeling tool that implements a maturity model to support process designers in the definition of resilient aware business processes at design-time, using OMG CMMN as modeling notation

M-type asteroids: Rotational properties of 16 objects

We present the results of photometric observations of 16 M-type asteroids performed from 1984 through 1990. The observations have been carried out at the Teramo and Torino Astronomical Observatories, Italy, and at the European Southern Observatory (ESO), La Silla, Chile. We have determined for the first time a reliable rotational period for the asteroids 369 Aeria (PSyn = 4.h787), 785 Zwetana (PSyn = 8.h919) and 798 Ruth (PSyn = 10.h35). We have estimated the shape and the pole coordinates of 135 Hertha and 250 Bettina and also checked those of 16 Psyche, 21 Lutetia, 22 Kalliope, 129 Antigone and 216 Kleopatra. Composite lightcurves have been derived for nine of the observed objects (83 Beatrix, 97 Klotho, 110 Lydia, 129 Antigone, 135 Hertha, 216 Kleopatra, 369 Aeria, 785 Zwetana and 798 Ruth). Reasonably single lightcurve's coverage has been obtained for the majority of the reamining ones. For some asteroids transformations to the standard system have been performed

Healthcare workers’ willingness to receive influenza vaccination in the context of the covid-19 pandemic: A survey in southern Italy

This cross-sectional survey was designed to evaluate hospital healthcare workers’ (HCWs) willingness to receive the influenza vaccination during the COVID-19 pandemic and to identify the related determinants, since it is plausible that the two epidemics will coexist in future winters. Overall, 68% out of 490 participants expressed their willingness to receive influenza vaccination in the 2020/21 season, with 95% of those ever and 45.8% of those never vaccinated in the previous six influenza seasons. Belief that influenza vaccine is useful in distinguishing influenza symptoms from those of COVID-19 and that the influenza vaccine is useful to prevent influenza in hospital settings, willingness to receive COVID-19 vaccination, having no concern about influenza vaccine side effects, concern about the possibility to transmit influenza to hospitalized patients, and influenza vaccination in previous years were all predictors of willingness to receive influenza vaccination. In the context of the COVID-19 pandemic, a relevant increase in the willingness to undergo influenza vaccination was reported. Therefore, interventions focused primarily on enabling factors are needed to promote the adherence to influenza vaccination in future seasons among HCWs

The Challenge of Pharmacotherapy in Children and Adolescents with Epilepsy-ADHD Comorbidity

Epilepsy is common in children and adolescents where its prevalence is 3.2-5.5/1000. About one-third of patients also have attention deficit hyperactivity/impulsivity disorder (ADHD). The possible relationship between epilepsy and ADHD is still unclear, and ADHD symptoms (such as inattention, hyperactivity, behavioral disturbances) are frequently considered as adverse effects of antiepileptic drugs (AEDs). The literature was searched for data on the behavioral effects of AEDs. Phenobarbital is the most frequently reported medication to induce symptoms of ADHD, followed by topiramate and valproic acid. Phenytoin seems to exert modest effects, while for levetiracetam there are contrasting data. Lacosamide induces some beneficial effects on behavior; carbamazepine and lamotrigine exert favorable effects on attention and behavior. Gabapentin and vigabatrin have limited adverse effects on cognition. Oxcarbazepine, rufinamide, and eslicarbazepine do not seem to aggravate or induce ADHD symptoms, whereas perampanel can lead to a high incidence of hostile/aggressive behavior, which increases with higher dosages. Information about the behavioral effects of ethosuximide, zonisamide, tiagabine, pregabalin, stiripentol, and retigabine is still limited. Because ADHD significantly affects the quality of life of epilepsy patients, the clinical management of this neuropsychiatric disorder should be a priority. Methylphenidate is effective most children and adolescents with ADHD symptoms and comorbid epilepsy, without a significant increase of seizure risk, although data are still limited with few controlled trials

Periodontal regeneration by leukocyte and platelet-rich fibrin with autogenous bone graft versus enamel matrix derivative with autogenous bone graft in the treatment of periodontal intrabony defects: A randomized non-inferiority trial

Background: Aim of the present study was to ascertain if a combination of leukocyte and platelet-rich fibrin (L-PRF) + autogenous bone graft (ABG) may be a clinically “non-inferior” treatment modality as compared with the association of enamel matrix derivative (EMD) with ABG in the management of intrabony defects (IBDs). Methods: A total of forty-four patients, exhibiting at least one unfavorable intraosseous defect, were treated by L-PRF associated with ABG (22 patients; test group) or EMD+ABG (control group) in each defect. At baseline and 12 months, a complete clinical and radiographic examination was done. Pre- and post-therapy clinical (probing pocket depth [PPD], clinical attachment level [CAL], gingival recession [GR]) and radiographic (defect Bone level [(DBL)] parameters for the different treatments were compared. To guarantee the test treatment's efficacy 1mm was chosen as non-inferiority margin; for clinical relevance, a second non-inferiority margin = 0.5 mm was set. Results: Clinical and radiographic parameters significantly improved 12 months after surgery in both test and control sites, without inter-groups differences for each measurement. The control group – test group differences for the parameters CAL gain −0.248 mm (−0.618 to 0.122), PPD Reduction −0.397 mm (−0.810 to 0.015), GR Change 0.059 mm (−0.300 to 0.418), DBL Gain −0.250 mm (−0.746 to 0.246) were all within the non-inferiority margin of 0.5 mm. Conclusion: Our results suggest that the L-PRF+ABG combined treatment of non-contained IBDs produces non-inferior results in terms of CAL gain, PPD reduction, GR increase and DBL gain in comparison with the EMD+ABG combination