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IntroductionAlthough shortcomings in clinician–family communication and decision making for incapacitated, critically ill patients are common, there are few rigorously tested interventions to improve outcomes. In this manuscript, we present our methodology for the Pairing Re-engineered Intensive Care Unit Teams with Nurse-Driven Emotional support and Relationship Building (PARTNER 2) trial, and discuss design challenges and their resolution.Methods and analysisThis is a pragmatic, stepped-wedge, cluster randomised controlled trial comparing the PARTNER 2 intervention to usual care among 690 incapacitated, critically ill patients and their surrogates in five ICUs in Pennsylvania. Eligible subjects will include critically ill patients at high risk of death and/or severe long-term functional impairment, their main surrogate decision-maker and their clinicians. The PARTNER intervention is delivered by the interprofessional ICU team and overseen by 4–6 nurses from each ICU. It involves: (1) advanced communication skills training for nurses to deliver support to surrogates throughout the ICU stay; (2) deploying a structured family support pathway; (3) enacting strategies to foster collaboration between ICU and palliative care services and (4) providing intensive implementation support to each ICU to incorporate the family support pathway into clinicians’ workflow. The primary outcome is surrogates’ ratings of the quality of communication during the ICU stay as assessed by telephone at 6-month follow-up. Prespecified secondary outcomes include surrogates’ scores on the Hospital Anxiety and Depression Scale, the Impact of Event Scale, the modified Patient Perception of Patient Centredness scale, the Decision Regret Scale, nurses’ scores on the Maslach Burnout Inventory, and length of stay during and costs of the index hospitalisation.We also discuss key methodological challenges, including determining the optimal level of randomisation, using existing staff to deploy the intervention and maximising long-term follow-up of participants.Ethics and disseminationWe obtained ethics approval through the University of Pittsburgh, Human Research Protection Office. The findings will be published in peer-reviewed journals.Trial registration numberNCT02445937

To improve the health self-efficacy of the Lubbock, Texas community, Texas Tech University Health Sciences Center School of Medicine students organized a health fair to promote patient education and improve awareness of health issues, such as diabetes, heart health, hypertension, skin care, hypercholesterolemia, nutrition, stress management, cancer screening and recommendations, physical fitness, and mental health. An evaluation of 57 health fair participants’ self-reported self-efficacy on a scale from 0 to 10 before and after visiting the health fair revealed an average increase in self-efficacy of 0.87 (before: 7.96, after: 8.83, p

Visual data collection methods are gaining momentum in the field of qualitative research because of their ability to document the social world and experiences of participants (Banks, 2001; Rose, 2001. This study employed quilting as a data collection method to capture the experiences of 47 Fetal Alcohol Spectrum Disorder (FASD) prevention workers in the Parent Child Assistance Program (PCAP) across Alberta. Specifically, this article focuses on the process of creating the quilt, the impact that this data collection method has had on participants and researchers, as well as a discussion of our next steps and suggestions for future opportunities to use quilting methods in community-based research. [ABSTRACT FROM AUTHOR]

Idiopathic acquired aplastic anemia (AA) is a rare lymphocyte-mediated bone marrow aplasia. No specific mechanisms or triggers of AA have been identified. We recently observed several patients who developed AA after Hodgkin lymphoma (HL). We hypothesized that the co-occurrence of HL and AA is not random and may be etiologically significant. To test this hypothesis, we determined the incidence of AA in HL patients at our institution. We identified four patients with co-occurring HL and AA, with the incidence of AA in HL patients >20-fold higher compared to the general population. We identified 12 additional patients with AA and HL through a systematic literature review. Of the 16 total patients,15 (93.8%) developed AA after or concurrent with a HL diagnosis. None of the patients had marrow involvement by HL. Five of 15 patients were in complete remission from HL at the time of AA diagnosis, and six had a concurrent presentation with no prior cytotoxic therapy, with diagnostic timeframe information unavailable for four patients. The median interval between HL diagnosis and AA onset was 16 months, ranging from concurrent to 14 years after a HL diagnosis. The median survival after AA diagnosis was 14 months (range: 1 month to 20 years). Our results show that patients with HL have a higher incidence of AA compared to the general population and suggest that HL-related immune dysregulation may be a risk factor for AA. Better recognition and management of AA in HL patients is needed to improve outcomes in this population.

Glucocerebrosidase is a lysosomal hydrolase involved in the breakdown of glucosylceramide. Gaucher disease, a recessive lysosomal storage disorder, is caused by mutations in the gene GBA1. Dysfunctional glucocerebrosidase leads to accumulation of glucosylceramide and glycosylsphingosine in various cell types and organs. Mutations in GBA1 are also a common genetic risk factor for Parkinson disease and related synucleinopathies. In recent years, research on the pathophysiology of Gaucher disease, the molecular link between Gaucher and Parkinson disease, and novel therapeutics, have accelerated the need for relevant cell models with GBA1 mutations. Although induced pluripotent stem cells, primary rodent neurons, and transfected neuroblastoma cell lines have been used to study the effect of glucocerebrosidase deficiency on neuronal function, these models have limitations because of challenges in culturing and propagating the cells, low yield, and the introduction of exogenous mutant GBA1. To address some of these difficulties, we established a high yield, easy-to-culture mouse neuronal cell model with nearly complete glucocerebrosidase deficiency representative of Gaucher disease. We successfully immortalized cortical neurons from embryonic null allele gba−/− mice and the control littermate (gba+/+) by infecting differentiated primary cortical neurons in culture with an EF1α-SV40T lentivirus. Immortalized gba−/− neurons lack glucocerebrosidase protein and enzyme activity, and exhibit a dramatic increase in glucosylceramide and glucosylsphingosine accumulation, enlarged lysosomes, and an impaired ATP-dependent calcium-influx response; these phenotypical characteristics were absent in gba+/+ neurons. This null allele gba−/− mouse neuronal model provides a much-needed tool to study the pathophysiology of Gaucher disease and to evaluate new therapies.

The evolution of a lipid-based cuticle on aerial plant surfaces that protects against dehydration is considered a fundamental innovation in the colonization of the land by the green plants. However, key evolutionary steps in the early regulation of cuticle synthesis are still poorly understood, owing to limited studies in early-diverging land plant lineages. Here, we characterize a land plant specific subgroup 9 R2R3 MYB transcription factor MpSBG9, in the early-diverging land plant model Marchantia polymorpha, that is homologous to MIXTA proteins in vascular plants. The MpSBG9 functions as a key regulator of cuticle biosynthesis by preferentially regulating expression of orthologous genes for cutin formation, but not wax biosynthesis genes. The MpSBG9 also promotes the formation of papillate cells on the adaxial surface of M. polymorpha, which is consisitent with its canonical role in vascular plants. Our observations imply conserved MYB transcriptional regulation in the control of the cutin biosynthesis pathway as a core genetic network in the common ancestor of all land plants, implicating the land plant-specific MIXTA MYB lineage in the early origin and evolution of the cuticle., (© 2020 The Authors New Phytologist © 2020 New Phytologist Foundation.)