ALS Registry

BACKGROUND: Motor neuron diseases (MND), with amyotrophic lateral sclerosis constituting most cases, are rare conditions of unknown etiology. There have been reports of an increase in incidence during the latter half of the twentieth century in various Western countries, including Sweden. This study provides updated data on the incidence of MND in Sweden during the last 20 years. METHODS: Data was obtained from the Swedish National Patient Register on individuals diagnosed with MND from 2002 to 2021 and analysed in relation to group level data for the entire Swedish population...

OBJECTIVES: Early palliative care improves the quality of life of older patients with cancer. This work aimed to analyse the effect of sociodemographic, geriatric, and tumour-related determinants on hospital-based palliative care (HPC) referral in older patients with cancer, taking into account competing risk of death. METHODS: Older adults with diagnosed cancer from 2014 to 2018 according to the general cancer registry of Gironde (French department) were identified in three population-based cohorts on ageing (PAQUID, 3C - Three City, AMI)...

CONTEXT: In amyotrophic lateral sclerosis (ALS), equal care is important, given that the disease often has complex symptoms at the end of life. OBJECTIVES: The aim was to study the possible associations between demographic and clinical factors, including age, sex, and frailty, with acute healthcare utilization in the last month of life, measured by emergency room (ER) visits, admissions to acute hospitals and, acute hospitals as place of death, among patients with ALS...

OBJECTIVE: One of the difficulties in developing a novel drug for patients with amyotrophic lateral sclerosis (ALS) is the significant variation in the clinical course. To control this variation, a 12-week run-in period is used in some clinical trials. Based on the Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALSFRS-R) change during the run-in period, only moderate progressors are selected in some clinical trials. Some reports showed that the ALSFRS-R progression rate was associated with survival...

BACKGROUND: Patients with advanced cancer who no longer have standard treatment options available may decide to participate in early phase clinical trials (i.e. experimental treatments with uncertain outcomes). Shared decision-making (SDM) models help to understand considerations that influence patients' decision. Discussion of patient values is essential to SDM, but such communication is often limited in this context and may require new interventions. The OnVaCT intervention, consisting of a preparatory online value clarification tool (OnVaCT) for patients and communication training for oncologists, was previously developed to support SDM...

PURPOSE: Mechanical ventilation is a life-supporting intervention but is associated with known risks and complications. To improve the efficacy and safety profile of mechanical ventilation, manufacturers have developed advanced ventilator settings, modes, and alarm strategies to optimize ventilation for patient needs while avoiding complications. However, there is little real-world data published on the deployment of ventilator technology. The main objective of this study was to assess the clinical safety and performance of the Puritan Bennett™ 980 Ventilator System (PB980) using real-world clinical data collected from a diverse, global patient population...

The Advanced Trauma Life Support (ATLS) approach is generally accepted as the standard of care for the initial management of severely injured patients. While whole body computed tomography (WBCT) is still considered a contraindication in haemodynamically unstable trauma patients, there is a growing amount of data indicating the absence of harm from cross sectional imaging in this patient group. Our study aimed to compare the early mortality of unstable trauma patients undergoing a WBCT during the initial workup with those who did not...

OBJECTIVE: The responsibility of caring for patients with leukaemia places a heavy burden on family caregivers (FCs) and negatively impacts their quality of life (QoL). This study aimed to investigate the effects of peer support (PS)-based online education programme on the burden of care (BoC) and QoL of FCs of patients with leukaemia. METHODS: This before-after study involved a total of 80 eligible FCs of patients with leukaemia (40 individuals per group). The participants received the necessary information from a researcher and peers through online sessions and WhatsApp group...

PURPOSE: Many people with cancer (patients) want to know their prognosis (a quantitative estimate of their life expectancy) but this is often not discussed or poorly communicated. The optimal timing of prognostic discussions with people with advanced cancer is highly personalised and complex. We aimed to find, organise, and summarise research regarding the timing of discussions of prognosis with people with advanced cancer. METHODS: We conducted a systematic review of publications from databases, clinical practice guidelines, and grey literature from inception to 2023...

BACKGROUND: Adding immune checkpoint inhibitors (ICIs) to the chemotherapy has shown significant clinical benefits in neoadjuvant treatment of locally advanced esophageal squamous cell carcinoma (ESCC). Sintilimab is one such ICI used for treatment. Herein, we designed a trial to evaluate the safety and efficacy of sintilimab combined with paclitaxel and platinum for locally advanced resectable ESCC. METHODS: Patients with locally advanced resectable (stage II-III) ESCC were enrolled and received at least two cycles of neoadjuvant therapy with sintilimab (200 mg on day 1) plus platinum-based chemotherapy in each 3-week cycle followed by esophagectomy...

INTRODUCTION: The widespread incorporation of extracorporeal cardiopulmonary resuscitation (ECPR) for out-of-hospital cardiac arrest requires the delivery of effective and high-quality chest compressions prior to the initiation of ECPR. The aim of this study was to evaluate and compare the effectiveness of mechanical and manual chest compressions until the initiation of ECPR. METHODS: This study was a secondary analysis of the Japanese retrospective multicenter registry "Study of Advanced Life Support for Ventricular Fibrillation by Extracorporeal Circulation II (SAVE-J II)"...

INTRODUCTION: For out-of-hospital cardiac arrests (OHCAs), time is of the essence. While the relationship between EMS response time (ERT) and OHCA outcomes is well studied, a more comprehensive assessment of the effects of other intervention time is needed, which is essential to guide clinical practice. OBJECTIVES: Evaluating how a longer total pre-hospital time (TPT), ERT, advance life support response time (ART) and EMS cardiopulmonary resuscitation time (ECT) increase the mortality rates, unfavorable neurological outcomes, and severe complications at discharge of OHCAs...

BACKGROUND: Pelvic Circumferential Compression Devices (PCCD) are standard in hemorrhage-control of unstable pelvic ring fractures (UPF). Controversial data on their usefulness exists. Aim of the study was to investigate whether prehospital application of PCCD can reduce mortality and transfusion requirements in UPF. METHODS: Retrospective cohort study. From 2016 until 2021, 63,371 adult severely injured patients were included into TraumaRegister DGU® of the German Trauma Society (TR-DGU)...

Chimeric antigen receptor (CAR) T-cells targeting CD19 have been approved for the treatment of relapse/refractory large B-cell lymphoma. Hematotoxicity is the most frequent CAR T-cell-related adverse event. Transfusion support is a surrogate marker of severe cytopenias. Transfusion impacts patients' quality of life, presents specific toxicities and is known to affect immunity through the so-called transfusion-related immunomodulation, that may impact CAR T-cell efficacy. We analyzed data from 671 patients from the French DESCAR-T registry for whom exhaustive transfusion data were available...

Although the near-term benefit of immune tolerance induction (ITI) for the treatment of people with severe hemophilia A with inhibitor is apparent, the magnitude of ITI's longer-term impact on clinical outcomes remains undefined. We examined the association between receiving ITI and the success of ITI on clinical outcomes including 1) clinical events, 2) healthcare utilization, 3) quality of life/function, 4) socioeconomic status (SES), and 5) death, using the Community Counts (CC) registry of US Hemophilia Treatment Centers between 2013-2017...

To evaluate QRS complex features during the first month of life and the association with echocardiographic measurements of left ventricular mass in neonates. Prospective cohort study of neonates with electrocardiography (ECG) and echocardiography performed during the first month of life. Left ventricular mass index (LVMI) was determined by echocardiography and the correlation with electrocardiographic markers of LVMI outliers (≥ 98th percentile) were analyzed. We included 17,450 neonates (52% boys; median age at examination 11 days) and found an increase in median QRS duration and LVMI during the first month of life (54 vs...

BACKGROUND: Obesity is a known cardiovascular risk factor and associated with higher postoperative complication rates in patients undergoing cardiac surgery. In heart failure (HF), conflicting evidence in terms of survival has been reported, whereas sarcopenia is associated with poor prognosis. An increasing number of HF patients require left ventricular assist device (LVAD) implantations. The postoperative mortality has improved in recent years but is still relatively high. The impact of body composition on outcome in this population remains unclear...

OBJECTIVE: To assess the temporal relationship between premorbid lipid levels and long-term amyotrophic lateral sclerosis (ALS) risk. METHODS: From Norwegian cardiovascular health surveys (1974-2003), we collected information on total cholesterol (TC), triglycerides (TG), high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein cholesterol (LDL-C), glucose, and other cardiovascular risk factors. ALS incidence and mortality were identified through validated Norwegian health registries...

The antagonistic pleiotropy hypothesis posits that natural selection for pleiotropic mutations that confer earlier or more reproduction but impair the post-reproductive life causes aging. This hypothesis of the evolutionary origin of aging is supported by case studies but lacks unambiguous genomic evidence. Here, we genomically test this hypothesis using the genotypes, reproductive phenotypes, and death registry of 276,406 U.K. Biobank participants. We observe a strong, negative genetic correlation between reproductive traits and life span...

BACKGROUND AND AIMS: HeartMate 3 (HM3) is a fully magnetically levitated continuous flow left ventricular assist device, which received CE marking in 2015. The ELEVATE Registry was initiated to collect real-world outcomes in patients treated with HM3 post-CE Mark approval. METHODS: A total of 540 subjects implanted at 26 centres between March 2015 and February 2017 were included in this registry. Of these, 463 received the device as a primary implant (primary implant cohort, PIC), 19 as a pump exchange (pump exchange cohort), and in 58 patients, only anonymized survival data were collected (anonymized cohort, AC)...