Explore the vast range of titles available.

Background/Objectives: Oropharyngeal dysphagia (OD) is a prevalent symptom in patients with neuromuscular diseases (NMDs) and increases the risk of aspiration and malnutrition. Malnutrition is frequent in these patients and is not only related to dysphagia, but also generates a vicious circle that may result in worsening muscle atrophy and weakness. Texture modification is required to ensure safe and efficient swallowing. This study evaluates the acceptability of a ready-to-mix oral nutritional supplement (ONS) containing high-quality protein and a xanthan-based thickener in patients with OD and NMDs. Methods: This cross-sectional study includes adult patients with NMDs and OD who received a xanthan gum-based protein ONS. Patients completed a questionnaire to rate their satisfaction with the ONS. Results: Seventy-two patients were included (median age = 56 years; 51% were males). The percentage of patients with moderate-to-severe OD who rated the taste, consistency, and product quality as acceptable-to-very-satisfactory (score 3–5) were 76%, 80%, and 84%, respectively. After three months, 93% of patients continued consuming the ONS. Conclusions: Our findings support that the use of protein xanthan gum-based ONS can be as a palatable and an effective nutritional intervention, given their high satisfaction and acceptance rates among patients with NMDs and OD.

The impact of reperfusion therapies on cognition has been poorly explored and little knowledge exists. We explored the influence of endovascular treatment (EVT) on cognitive outcome in patients with anterior circulation ischemic stroke. Patients presenting with ischemic stroke due to anterior large vessel occlusion who underwent intravenous thrombolysis (IVT) alone or EVT plus IVT were recruited. Cognitive abilities were evaluated at 6 months from stroke through a neuropsychological test battery. A total of 88 patients with a mean age of 66.3 ± 12.9 years were included, of which 38 treated with IVT alone and 50 with IVT plus EVT. Compared to patients treated with IVT alone, patients who received EVT plus IVT performed significantly better at the neuropsychological tests exploring executive functions, attention, abstract reasoning, visuospatial ability, visual and verbal and memory. At multivariable regression analysis, the EVT was independently associated with the 6-month cognitive performance after the adjustment for age, sex, admission National Institutes of Health Stroke Scale score, systolic blood pressure, glucose level, Alberta Stroke Program Early CT score, side of stroke, site of occlusion, and Back Depression Inventory score [Stroop Test Word Reading: adj β = 13.99, 95% confidence interval (CI) 8.47–19.50, p < 0.001; Stroop Test Colour Naming: adj β = 6.63, 95% CI 2.46–10.81, p = 0.002; Trail Making Test-A: adj β = − 92.98, 95% CI − 153.76 to − 32.20, p = 0.003; Trail Making Test-B: adj β = − 181.12, 95% CI − 266.09 to − 96.15; p < 0.001; Digit Span Test Forward: adj β = 1.44, 95% CI 0.77–2.10, p < 0.001; Digit Span Test Backward: adj β = 1.10, 95% CI 0.42–1.77, p = 0.002; Coloured Progressive Matrices: adj β = 5.82, 95% CI 2.71–8.93, p < 0.001; Rey Complex Figure Test-Copy: adj β = 6.02, 95% CI 2.74–9.30, p < 0.001; Rey Complex Figure Test-Immediate recall: adj β = 6.00, 95% CI 2.34–9.66, p = 0.002; Rey Complex Figure Test-Delayed recall: adj β = 5.73, 95% CI 1.95–9.51, p = 0.003; Rey Auditory Verbal Learning Test-Immediate recall: adj β = 12.60, 95% CI 6.69–18.52, p < 0.001; Rey Auditory Verbal Learning Test-Delayed recall: adj β = 1.85, 95% CI 0.24–3.45, p = 0.025]. Patients treated with EVT plus IVT had better cognitive performance than patients treated with IVT alone at 6 months from anterior circulation ischemic stroke.

ObjectiveTo retrospectively evaluate quality of life (QoL) in a large multicenter cohort of adult patients affected by spinal muscular atrophy (SMA) during nusinersen treatment.MethodsWe included adult (≥ 18 years) patients clinically and genetically defined as SMA2, SMA3 and SMA4, who started nusinersen treatment in adulthood. QoL was rated by the Individualized Neuromuscular Quality of Life (INQoL) questionnaire. Concurrent motor function evaluation included the Hammersmith Functional Motor Scale Expanded (HFMSE), the Revised Upper Limb Module (RULM), the 6 min walking test (6MWT).Results189 completed questionnaires were collected during a 14 months’ treatment period. 78 patients were included (7 SMA2 and 69 SMA3 and 2 SMA4) with mean disease duration at first nusinersen administration of 33.2 years (± 12.5 years). All the scores for each INQoL domain (weakness, fatigue, activities, independence, social relationship, emotions, body images) and the derived QoL total score, significantly improved during the observation period, except the muscle locking and pain items. Exploratory analyses suggested that emotions and social relationships were more relevant issues for females compared to males. Social relationships were affected also by a longer disease duration (> 30 years). In SMA3 non-walker patients, activities ameliorate better compared to walkers. The HFMSE and RULM significantly improved from baseline; however, no associations with QoL total score and weakness, activities or independence were demonstrated.ConclusionIn our cohort, adult SMA patients showed a global improvement at the INQoL assessment over 14 months of nusinersen treatment. QoL assessment is relevant to SMA multidisciplinary evaluation.

Among 37 patients with ischemic stroke, the Social Maturity Scale Social Quotient (SMSSQ) score in the Vineland Social Maturity Scale at one month and the change in the SMSSQ score at three months from stroke onset showed a statistically significant correlation with the change in motor function over the same period as assessed by the Modified Barthel Index [6]. In animal models, post-stroke isolation has been associated with a significant increase in infarct size and mortality [7]. [...]in an animal model of ischemia-reperfusion injury, rats in the enriched environment group exhibited smaller infarction volumes and significantly improved neurological functions as compared to rats in standard housing conditions [9].

Background and Objectives: The prognostic impact of ventricular fibrillation (VF) recurrences after a successful shock in out-of-hospital cardiac arrest (OOHCA) is still poorly understood, and some evidence suggests a potential pro-arrhythmic effect of chest compressions in this setting. In the present analysis, we looked at the short-term and long-term prognosis of VF recurrences in OOHCA. And their potential association with chest compressions. Materials and Methods: The Progetto Vita, prospectively collecting data on all resuscitation efforts in the Piacenza province (Italy), was used for the present analysis. From the 461 OOHCAs found in a shockable rhythm, only those with optimal ECG tracings and good audio recordings (160) were assessed. Rhythms other than VF post-shock were analyzed five seconds after shock delivery and survival to hospital admission, hospital discharge, and long-term survival data over a 14-year follow-up were collected. Results: Population mean age was 64.4 ± 16.9 years, and 31.9% of all patients were female. Mean time to EMS arrival was 5.9 ± 4.5 min. Short- and long-term survival without neurological impairment were higher in patients without VF recurrence when compared to patients with VF recurrence, independently from the pre-induction rhythm (p < 0.001). After shock delivery, VF recurrence was higher when chest compressions were resumed early after discharge and more vigorously. Conclusions: VF recurrences after a shock could worsen short and long-term survival. The potential pro-arrhythmic effect of chest compressions should be factored in when considering the real risks and benefits of this procedure.

Despite guideline-directed medical therapy for heart failure with reduced ejection fraction (HFrEF), a residual risk of adverse outcomes persists, particularly after worsening heart failure (WHF). The VICTORIA trial demonstrated the benefit of adding vericiguat in high-risk patients. However, its real-world adoption in Italy remains unclear. The aim of this study was to assess the use, safety, and prescription patterns of vericiguat in Italian patients with recent WHF. The multicenter VeriChange survey was conducted across 28 hospitals in Northern Italy. A total of 399 anonymized clinical records of HFrEF patients with recent WHF were collected. The survey included demographic, clinical, therapeutic data and safety outcomes. Overall, 68% of patients were classified as NYHA III-IV and 77% had a left ventricular ejection fraction ≤35%. Vericiguat was initiated after the first WHF episode in 54% of cases, and during hospitalization in 50%. The target dose of 10 mg/day was reached in 56% of patients. Tolerability was high, with only 3% treatment discontinuation. Prescription occurred in a context of strong adherence to guideline-based therapy. Vericiguat was introduced early and safely in Italian real-world practice, especially in tertiary and referral centers. Broader implementation and earlier WHF recognition are still needed to reduce residual risk in advanced heart failure patients.

Hypercoagulability is a common complication of the systemic inflammation related to coronavirus disease 2019 creating debate within the critical care community on the therapeutic utility of Low Molecular Weight Heparin (LMWH). We collected data on consecutive patients with COVID-19 admitted to the Emergency Department of Castel San Giovanni Hospital, between February 29th and April 7th, 2020. Exclusion criteria were age <18 years, hospital stay <7 days, patients on dialysis and patients who had been transferred to other centers for which we could not collect data. Of the 257 patients included in the study, 49 (19.1%) died during hospitalization. We considered a wide set of variables as independent variables (age, sex, comorbidities and in-hospital treatments). We used a multivariate logistic regression model and, being heparin the only one therapy affecting survival rate, we compared prophylactic LMWH (p-LMWH) and Therapeutic LMWH (T-LMWH) groups. Kaplan Meier curve showed a higher survival probability in the T-LMWH and the difference between the two groups was statistically significant according to the log-rank or Mantel- Haenszel test (p< 0.0001). In a stratified analysis by ventilation type, the subgroup of patients who benefited from therapeutic LMWH was that in non-invasive mechanical ventilation. Using a multivariate analysis and adjusting for the drugs intake, TLMWH was the only therapy impacting on survival (HR 0.293, p <0.001). No fatal bleeding was observed. Therapeutic dose of LMWH in patients admitted to hospital with COVID-19 pneumonia was 70 associated with a decrease risk of intra-hospital mortality.

ObjectiveTo retrospectively investigate safety and efficacy of nusinersen in a large cohort of adult Italian patients with spinal muscular atrophy (SMA).MethodsInclusion criteria were: (1) clinical and molecular diagnosis of SMA2 or SMA3; (2) nusinersen treatment started in adult age (>18 years); (3) clinical data available at least at baseline (T0-beginning of treatment) and 6 months (T6).ResultsWe included 116 patients (13 SMA2 and 103 SMA3) with median age at first administration of 34 years (range 18–72). The Hammersmith Functional Rating Scale Expanded (HFMSE) in patients with SMA3 increased significantly from baseline to T6 (median change +1 point, p<0.0001), T10 (+2, p<0.0001) and T14 (+3, p<0.0001). HFMSE changes were independently significant in SMA3 sitter and walker subgroups. The Revised Upper Limb Module (RULM) in SMA3 significantly improved between T0 and T14 (median +0.5, p=0.012), with most of the benefit observed in sitters (+2, p=0.018). Conversely, patients with SMA2 had no significant changes of median HFMSE and RULM between T0 and the following time points, although a trend for improvement of RULM was observed in those with some residual baseline function. The rate of patients showing clinically meaningful improvements (as defined during clinical trials) increased from 53% to 69% from T6 to T14.ConclusionsOur data provide further evidence of nusinersen safety and efficacy in adult SMA2 and SMA3, with the latter appearing to be cumulative over time. In patients with extremely advanced disease, effects on residual motor function are less clear.

Razionale. Nonostante la terapia consigliata dalle attuali linee guida della Società Europea di Cardiologia sullo scompenso cardiaco con frazione di eiezione ridotta (HFrEF), persiste un importante rischio residuo di eventi avversi, specialmente dopo un episodio di aggravamento dello scompenso cardiaco (worsening heart failure, WHF). Lo studio VICTORIA ha dimostrato il beneficio clinico dell’aggiunta di vericiguat in questa specifica popolazione di pazienti ad alto rischio di eventi. Tuttavia, l’adozione di tale strategia nella pratica clinica reale italiana non è stata fino ad ora descritta. Obiettivo della survey VeriChange è stato quello di analizzare l’uso di vericiguat nella realtà italiana dopo la pubblicazione dello studio VICTORIA e le nuove indicazioni delle linee guida. Materiali e metodi. La survey multicentrica VeriChange ha coinvolto 28 Centri del Nord Italia che si interessano di insufficienza cardiaca. Sono stati raccolti dati clinici anonimi aggregati su 389 pazienti con HFrEF e recente WHF. Il questionario includeva variabili demografiche, cliniche, terapeutiche ed eventi avversi.Risultati. Il 68% dei pazienti era in classe funzionale NYHA III-IV e il 77% presentava una frazione di eiezione ventricolare sinistra ≤35%. Vericiguat è stato prescritto nel 54% dei casi dopo il primo episodio di WHF e nel 50% durante l’ospedalizzazione. Il 56% dei pazienti ha raggiunto la dose target di 10 mg/die, solo il 3% dei pazienti ha sospeso il farmaco per mancata tollerabilità. La terapia è stata implementata in un contesto di alta aderenza alle linee guida. Conclusioni. Vericiguat è stato introdotto in modo precoce e sicuro nella pratica clinica italiana per pazienti con WHF, specialmente nei Centri di secondo e terzo livello. Rimane necessario promuovere una maggiore diffusione del farmaco e una precoce identificazione del WHF per ridurre il rischio residuo dei pazienti più fragili affetti da HFrEF.

Background In Amyotrophic Lateral Sclerosis (ALS) patients with SOD1 mutation the intrathecal administration of tofersen slowed down the progression of disease in a controlled clinical study, but results were not statistically significant. Methods In this multicentre, observational study, we evaluated a cohort of 27 ALS- SOD1 patients who were treated with tofersen, focussing on 17 patients who were followed for at least 48 weeks (median period of 84 weeks, range 48–108). We compared the clinical slopes, as measured by ALSFRS-R, MRC scale and Forced Vital Capacity, during tofersen treatment with retrospective data at 1 year prior to therapy. Cerebrospinal fluid (CSF) and serum neurofilament light chains (NFL) were measured in all patients. Results Cumulative evaluation of the ALSFRS-R and MRC progression rates showed a statistically significant change during treatment with respect to the period prior to therapy ( p  = 0.023 and p  = 0.007, respectively). The analysis of individual patients showed that nine of the seventeen patients substantially stabilized or slightly improved. Four patients deteriorated during treatment, while in the remaining patients the very slow course did not allow to identify significant changes. CSF and serum NFL concentration markedly decreased in the near totality of patients. Increased levels of white blood cells and proteins in the CSF were found in 60% of patients. Such alterations were clinically asymptomatic in all but two patients who showed an acute pure motor radiculitis, which responded to steroid therapy. Conclusions Clinical findings and NFL analysis strongly suggest that tofersen may have a disease-modifying effect in a subset of SOD1 -ALS patients.