Cryoballoon pulmonary vein isolation as first line treatment for typical atrial flutter (CRAFT): study protocol for a randomised controlled trial.

Purpose: Treatment of typical atrial flutter (AFL) with cavo-tricuspid isthmus (CTI) ablation is associated with a high occurrence rate of new onset atrial fibrillation (AF) during follow-up. There are data to support the addition of pulmonary vein isolation (PVI) to CTI ablation in patients with both AF and AFL, but the role of cryoballoon PVI only, with no CTI ablation, in AFL patients with no prior documentation of AF has not been studied.

Methods: CRAFT is an international, prospective, randomised, open with blinded assessment, multicentre superiority study comparing radiofrequency CTI ablation and cryoballoon PVI in patients with typical AFL.

Improved transplant survival and long-term disease outcome in children with MHC class II deficiency.

MHC class II deficiency is a rare, but life-threatening, primary combined immunodeficiency. Hematopoietic cell transplantation (HCT) remains the only curative treatment for this condition, but transplant survival in the previously published result was poor. We analyzed the outcome of 25 patients with MHC class II deficiency undergoing first HCT at Great North Children's Hospital between 1995 and 2018.

Hematopoietic Cell Transplantation for MHC Class II Deficiency.

Major histocompatibility complex (MHC) class II deficiency is a rare and fatal primary combined immunodeficiency. It affects both marrow-derived cells and thymic epithelium, leading to impaired antigen presentation by antigen presenting cells and delayed and incomplete maturation of CD4+ lymphocyte populations. Affected children are susceptible to multiple infections by viruses, , bacteria and fungi.

Differentiating dementia disease subtypes with gait analysis: feasibility of wearable sensors?

Background: There are unique signatures of gait impairments in different dementia disease subtypes, such as Alzheimer's disease (AD), dementia with Lewy bodies (DLB) and Parkinson's disease (PDD). This suggests gait analysis is a useful differential marker for dementia disease subtypes, but this has yet to be assessed using inexpensive wearable technology.

Research Question: This study aimed to assess whether a single accelerometer-based wearable could differentiate dementia disease subtypes through gait analysis.

Conditioning Regimens for Hematopoietic Cell Transplantation in Primary Immunodeficiency.

Purpose Of Review: Hematopoietic cell transplantation (HCT) is an established curative treatment for children with primary immunodeficiencies. This article reviews the latest developments in conditioning regimens for primary immunodeficiency (PID). It focuses on data regarding transplant outcomes according to newer reduced toxicity conditioning regimens used in HCT for PID.

Do Alzheimer's and Lewy body disease have discrete pathological signatures of gait?

Objective: We aimed to refine the hypothesis that dementia has a unique signature of gait impairment reflective of underlying pathology by considering two dementia subtypes, Alzheimer's disease (AD) and Lewy body disease (LBD), and exploring the role of cognition in disease-specific gait impairments.

Background: Accurately differentiating AD and LBD is important for treatment and disease management. Early evidence suggests gait could be a marker of dementia due to associations between discrete gait characteristics and cognitive domains.

Factors That Influence Habitual Activity in Mild Cognitive Impairment and Dementia.

Background: Reduced engagement with habitual activity (HA) is associated with greater risk and progression of cognitive decline and falls in older adults and people with dementia. Understanding external and intrinsic factors that affect HA may provide novel targets for non-pharmacologic interventions.

Objective: This study primarily aims to identify factors that influence HA in normal ageing and cognitive impairment, such as cognitive and motor problems and disease subtype.

Manualised cognitive-behavioural therapy in treating depression in advanced cancer: the CanTalk RCT.

Background: With a prevalence of up to 16.5%, depression is one of the commonest mental disorders in people with advanced cancer. Depression reduces the quality of life (QoL) of patients and those close to them.

The neural correlates of discrete gait characteristics in ageing: A structured review.

Gait is complex, described by diverse characteristics underpinned by widespread central nervous system networks including motor and cognitive functions. Despite this, neural substrates of discrete gait characteristics are poorly understood, limiting understanding of gait impairment in ageing and disease. This structured review aims to map gait characteristics, defined from a pre-specified model reflecting independent gait domains, to brain imaging parameters in older adults.

Adjunctive rifampicin to reduce early mortality from Staphylococcus aureus bacteraemia: the ARREST RCT.

Background: bacteraemia is a common and frequently fatal infection. Adjunctive rifampicin may enhance early killing, sterilise infected foci and blood faster, and thereby reduce the risk of dissemination, metastatic infection and death.

Objectives: To determine whether or not adjunctive rifampicin reduces bacteriological (microbiologically confirmed) failure/recurrence or death through 12 weeks from randomisation.

Systematic Review of Economic Models Used to Compare Techniques for Detecting Peripheral Arterial Disease.

Background And Objective: Peripheral arterial disease (PAD) is a common condition, in which atherosclerotic narrowing in the arteries restricts blood supply to the leg muscles. In order to support future model-based economic evaluations comparing methods of diagnosis in this area, a systematic review of economic modelling studies was conducted.

Methods: A systematic literature review was performed in June 2017 to identify model-based economic evaluations of diagnostic tests to detect PAD, with six individual databases searched.

Disease Evolution and Response to Rapamycin in Activated Phosphoinositide 3-Kinase δ Syndrome: The European Society for Immunodeficiencies-Activated Phosphoinositide 3-Kinase δ Syndrome Registry.

Activated phosphoinositide 3-kinase (PI3K) δ Syndrome (APDS), caused by autosomal dominant mutations in (APDS1) or (APDS2), is a heterogeneous primary immunodeficiency. While initial cohort-descriptions summarized the spectrum of clinical and immunological manifestations, questions about long-term disease evolution and response to therapy remain. The prospective European Society for Immunodeficiencies (ESID)-APDS registry aims to characterize the disease course, identify outcome predictors, and evaluate treatment responses.

Extending the phenotype associated with the CSNK2A1-related Okur-Chung syndrome-A clinical study of 11 individuals.

Variants in the Protein Kinase CK2 alpha subunit, encoding the CSNK2A1 gene, have previously been reported in children with an intellectual disability and dysmorphic facial features syndrome: now termed the Okur-Chung neurodevelopmental syndrome. More recently, through trio-based exome sequencing undertaken by the Deciphering Developmental Disorders Study (DDD study), a further 11 children with de novo CSNK2A1 variants have been identified. We have undertaken detailed phenotyping of these patients.

Adjunctive rifampicin for Staphylococcus aureus bacteraemia (ARREST): a multicentre, randomised, double-blind, placebo-controlled trial.

Background: Staphylococcus aureus bacteraemia is a common cause of severe community-acquired and hospital-acquired infection worldwide. We tested the hypothesis that adjunctive rifampicin would reduce bacteriologically confirmed treatment failure or disease recurrence, or death, by enhancing early S aureus killing, sterilising infected foci and blood faster, and reducing risks of dissemination and metastatic infection.

Methods: In this multicentre, randomised, double-blind, placebo-controlled trial, adults (≥18 years) with S aureus bacteraemia who had received ≤96 h of active antibiotic therapy were recruited from 29 UK hospitals.

Do perioperative antibiotics reduce the risk of surgical-site infections following excision of ulcerated skin cancers? A Critically Appraised Topic.

Aim: To review the efficacy of perioperative antibiotics in reducing the risk of surgical-site infections (SSIs) following excision of ulcerated skin cancers.

Setting And Design: Study selection, data extraction and analysis were carried out independently by four authors. Only randomized controlled trials (RCTs) reported in the English language were included.

Treosulfan and Fludarabine Conditioning for Hematopoietic Stem Cell Transplantation in Children with Primary Immunodeficiency: UK Experience.

We previously published results for 70 children who received conditioning with treosulfan and cyclophosphamide (n = 30) or fludarabine (n = 40) before undergoing hematopoietic stem cell transplantation (HSCT) for primary immunodeficiency (PID). Toxicity was lower and T cell chimerism was better in the patients receiving fludarabine, but cohort numbers were relatively small and follow-up was short. Here we report outcomes of 160 children who received homogeneous conditioning with treosulfan, fludarabine, and, in most cases, alemtuzumab (n = 124).

What Can Quantitative Gait Analysis Tell Us about Dementia and Its Subtypes? A Structured Review.

Distinguishing dementia subtypes can be difficult due to similarities in clinical presentation. There is increasing interest in discrete gait characteristics as markers to aid diagnostic algorithms in dementia. This structured review explores the differences in quantitative gait characteristics between dementia and healthy controls, and between four dementia subtypes under single-task conditions: Alzheimer's disease (AD), dementia with Lewy bodies and Parkinson's disease dementia, and vascular dementia.

The prevalence of lumbar spondylolysis in young children: a retrospective analysis using CT.

Purpose: Although lumbar spondylolysis is encountered in general population with an incidence estimated to be 3-10%, limited information is available for children. The aim of the study is to determine the prevalence of spondylolysis according to associated vertebral bony malformation and spinopelvic parameters in children under eight requiring CT evaluation for unrelated lumbar conditions.

Methods: Seven hundred and seventeen abdominal and pelvic multi-detector CT scans were obtained in patients under 8 years of age were reviewed.

The clinical and cost effectiveness of cognitive behavioural therapy plus treatment as usual for the treatment of depression in advanced cancer (CanTalk): study protocol for a randomised controlled trial.

Background: The prevalence of depressive disorder in adults with advanced cancer is around 20 %. Although cognitive behavioural therapy (CBT) is recommended for depression and may be beneficial in depressed people with cancer, its use for depression in those with advanced disease for whom cure is not likely has not been explored.

Methods: People aged 18 years and above with advanced cancer attending General Practitioner (GP), oncology or hospice outpatients from centres across England will be screened to establish a DSM-IV diagnosis of depression.