Evolution of Movement Disorders in Patients With CLN2-Batten Disease Treated With Enzyme Replacement Therapy.

Objectives: Neuronal ceroid lipofuscinosis type 2 (CLN2-disease) is an inherited childhood-onset neurodegenerative condition, with classical early features of speech delay, epilepsy, myoclonus, ataxia, and motor regression. This study aimed to better characterize the spectrum of movement disorders in CLN2-disease in a cohort of children receiving enzyme replacement therapy (ERT).

Methods: A cohort of 18 children attending a single center for treatment with cerliponase alfa ERT was systematically assessed using a standardized structured history and a double-scored, video-recorded examination using the Unified Batten Disease Rating Scale (UBDRS) and Abnormal Involuntary Movement Scale.

Major trauma patients and their outcomes - A retrospective observational study of critical care trauma admissions to a trauma unit with special services.

Introduction: International data describes a changing pattern to trauma over the last decade, with an increasingly comorbid population presenting challenges to trauma management and resources. In Ireland, resource provision and management of trauma is being transformed to deliver a trauma network, in line with international best practice. Our hospital plays a crucial role within this network and is designated a Trauma Unit with Specialist Services (TUSS) to distinguish it from standard trauma units.

The Evolving Role of Electroencephalography in Postarrest Care.

Electroencephalography is an accessible, portable, noninvasive and safe means of evaluating a patient's brain activity. It can aid in diagnosis and management decisions for post-cardiac arrest patients with seizures, myoclonus and other non-epileptic movements. It also plays an important role in a multimodal approach to neuroprognostication predicting both poor and favorable outcomes.

The evolution of vaccine hesitancy through the COVID-19 pandemic: A semi-structured interview study on booster and bivalent doses.

We sought in-depth understanding on the evolution of factors influencing COVID-19 booster dose and bivalent vaccine hesitancy in a longitudinal semi-structured interview-based qualitative study. Serial interviews were conducted between July 25th and September 1, 2022 (Phase I: univalent booster dose availability), and between November 21, 2022 and January 11, 2023 (Phase II: bivalent vaccine availability). Adults (≥18 years) in Canada who had received an initial primary series and had not received a COVID-19 booster dose were eligible for Phase I, and subsequently invited to participate in Phase II.

Protocol and statistical analysis plan for the identification and treatment of hypoxemic respiratory failure and acute respiratory distress syndrome with protection, paralysis, and proning: A type-1 hybrid stepped-wedge cluster randomised effectiveness-implementation study.

Objective: To describe a study protocol and statistical analysis plan (SAP) for the identification and treatment of hypoxemic respiratory failure (HRF) and acute respiratory distress syndrome (ARDS) with protection, paralysis, and proning (TheraPPP) study prior to completion of recruitment, electronic data retrieval, and analysis of any data.

Design: TheraPPP is a stepped-wedge cluster randomised study evaluating a care pathway for HRF and ARDS patients. This is a type-1 hybrid effectiveness-implementation study design evaluating both intervention effectiveness and implementation; however primarily powered for the effectiveness outcome.

Designing a Behaviour Change Wheel guided implementation strategy for a hypoxaemic respiratory failure and ARDS care pathway that targets barriers.

Background: A significant gap exists between ideal evidence-based practice and real-world application of evidence-informed therapies for patients with hypoxaemic respiratory failure (HRF) and acute respiratory distress syndrome (ARDS). Pathways can improve the quality of care provided by helping integrate and organise the use of evidence informed practices, but barriers exist that can influence their adoption and successful implementation. We sought to identify barriers to the implementation of a best practice care pathway for HRF and ARDS and design an implementation science-based strategy targeting these barriers that is tailored to the critical care setting.

Critical illness among patients experiencing homelessness: a retrospective cohort study.

Purpose: To understand the epidemiology and healthcare use of critically ill patients experiencing homelessness compared to critically ill patients with stable housing.

Methods: This retrospective population-based cohort study included adults admitted to any ICU in Alberta, Canada, for a 3-year period. Administrative and clinical data from the hospital, ICU and emergency department were used to examine healthcare resource use (processes of care, ICU and hospital length of stay, hospital readmission and emergency room visits).

Preliminary clinical testing to inform development of the Critical Care Pain Observation Tool for Families (CPOT-Fam).

Introduction: Many patients in the intensive care unit (ICU) cannot communicate. For these patients, family caregivers (family members/close friends) could assist in pain assessment. We previously adapted the Critical Care Pain Observation Tool (CPOT) for family caregiver use (CPOT-Fam).

Histology-Validated Dielectric Characterisation of Lung Carcinoma Tissue for Microwave Thermal Ablation Applications.

Microwave thermal ablation is a promising emerging treatment for early-stage lung cancer. Applicator design optimisation and treatment planning rely on accurate knowledge of dielectric tissue properties. Limited dielectric data are available in the literature for human lung tissue and pulmonary tumours.

Impacts of antipsychotic medication prescribing practices in critically ill adult patients on health resource utilization and new psychoactive medication prescriptions.

Background: Antipsychotic medications are commonly prescribed to critically ill adult patients and initiation of new antipsychotic prescriptions in the intensive care unit (ICU) increases the proportion of patients discharged home on antipsychotics. Critically ill adult patients are also frequently exposed to multiple psychoactive medications during ICU admission and hospitalization including benzodiazepines and opioid medications which may increase the risk of psychoactive polypharmacy following hospital discharge. The associated impact on health resource utilization and risk of new benzodiazepine and opioid prescriptions is unknown.

Codesigning a user-centred digital psychoeducational tool for youth mental well-being with families in Canada: study protocol for a sequential exploratory mixed methods study.

Introduction: On 11 March 2020, WHO declared the novel coronavirus (COVID-19) disease a global pandemic. Governments globally implemented physical distancing measures and closure of public institutions that resulted in varying implications to youth mental well-being (eg, social isolation, reduced extracurricular activities). These impacts may have detrimental short-term and long-term effects on youth mental well-being; care for youth with mental health disorders was already overstretched, underfunded and fragmented before the pandemic and youth are not often considered in mental health initiatives.

Treatment recommendation based on SYNTAX score 2020 derived from coronary computed tomography angiography and invasive coronary angiography.

The diagnostic performance of the SYNTAX score 2020 (SS-2020) when calculated using CCTA remains unknown. This study aimed to compare treatment recommendations based on the SS-2020 derived from coronary computed tomography angiography (CCTA) versus invasive coronary angiography (ICA). This interim analysis included 57 of the planned 114 patients with de-novo three-vessel disease, with or without left main coronary artery disease, enrolled in the ongoing FASTTRACK CABG trial.

Impact of on-pump and off-pump coronary artery bypass grafting on 10-year mortality versus percutaneous coronary intervention.

Objectives: The very long-term mortality of off-pump and on-pump coronary artery bypass grafting (CABG) versus percutaneous coronary intervention (PCI) in a randomized complex coronary artery disease population is unknown. This study aims to investigate the impact of on-pump and off-pump CABG versus PCI on 10-year all-cause mortality.

Methods: The SYNTAX trial randomized 1800 patients with three-vessel and/or left main coronary artery disease to PCI or CABG and assessed their survival at 10 years.

Lung Cancer resection in the absence of pre-operative histology: The Accuracy of Multidisciplinary Team Consensus.

Background: Lung resection remains the gold-standard of treatment for non-small cell lung cancer (NSCLC). British Thoracic Society (BTS) guidelines recommends the pursuit of pre-operative histological diagnosis and staging where possible. In the absence of pre-operative histology, surgical treatment can be offered in conjunction with multidisciplinary team (MDT) and patient consensus.

Family coaching during Spontaneous Awakening Trials and Spontaneous Breathing Trials (FamCAB): pilot study protocol.

Introduction: Many patients in the intensive care unit (ICU) require weaning from deep sedation (Spontaneous Awakening Trials, SATs) and mechanical ventilation (Spontaneous Breathing Trials, SBTs) in their journey to recovery. These procedures can be distressing for patients and their families. The presence of family members as 'coaches' during SATs/SBTs could provide patients with reassurance, reduce stress for patients and families and potentially improve procedural success rates.

Development and preclinical testing of the critical care pain observation tool for family caregiver use (CPOT-Fam).

Background And Aims: Pain assessment in noncommunicative intensive care unit (ICU) patients is challenging. For these patients, family caregivers (i.e.

Occupational therapist-guided cognitive interventions in critically ill patients: a feasibility randomized controlled trial.

Purpose: Intensive care unit (ICU) delirium is a common complication of critical illness requiring a multimodal approach to management. We assessed the feasibility of a novel occupational therapist (OT)-guided cognitive intervention protocol, titrated according to sedation level, in critically ill patients.

Methods: Patients aged ≥ 18 yr admitted to a medical/surgical ICU were randomized to the standard delirium prevention protocol or to the OT-guided cognitive intervention protocol in addition to standard of care.

Expanding the Spectrum of Early Neuroradiologic Findings in β Propeller Protein-Associated Neurodegeneration.

Background And Purpose: β propeller protein-associated neurodegeneration (BPAN) is the most common neurodegeneration with brain iron accumulation disorder. Typical radiologic findings are T2 hypointensity in the substantia nigra and globus pallidus, as well as a T1 halolike substantia nigra hyperintense signal surrounding a hypointense central area. However, these findings are often subtle or absent on initial scans, risking diagnostic delay.