Antibody responses to SARS-CoV-2 spike protein in a cohort of renal transplant recipients following two-dose primary immunization - A Caribbean country's perspective.

This study compares the humoral immune response of a cohort of renal transplant recipients (RTRs), in Trinidad & Tobago following two-dose primary immunization with non-mRNA vaccines amidst the COVID-19 pandemic. RTRs along with healthy, age-and gender-matched controls received either the adenoviral vector vaccine, AstraZeneca-Vaxzevria (AZ) or the inactivated vaccine, Beijing CNBG-BBIBP- CorV/Sinopharm (SP). Samples were taken after completion of a two-dose primary immunization during the period November 2021 to December 2021, at a mean interval of 138 days following immunization. 38/72 RTRs (53 %) failed to generate any protective antibody responses, compared with 7/73 participants, approximately 10 % in the healthy, age and gender-matched control group. In the RTRs, there was no significant correlation of their antibody concentration with either the timing of sample collection or the interval since transplantation. The study provides necessary information about the humoral response after two- doses of non-mRNA vaccines in a group of transplant recipients.

Using the principles of practice development to address challenges in recruitment and data collection when face-to-face methods are unavailable.

BACKGROUND: Researchers conducting studies involving pregnant women often find recruitment challenging. The COVID-19 pandemic added further complexity to studies requiring face-to-face participation. AIM: To demonstrate how to maintain the principles of practice development (PD) when a study must switch from face-to-face to remote methods of collecting data. DISCUSSION: The number of participants in the authors' study increased when they moved from face-to-face to telephone engagement during the COVID-19 pandemic. They continued using PD principles when they changed method and the quality of the data they collected remained constant, even once lockdown restrictions were in place. CONCLUSION: PD principles can offer ways for nurse researchers to engage, collaborate with and reflect with people for research projects, including when constraints compete with participation. They can also assist researchers in optimising and maintaining recruitment and data collection when face-to-face research methods are impossible. IMPLICATIONS FOR PRACTICE: The telephone can be a valuable alternative medium for recruiting participants and collecting data when face-to-face methods are impossible to use. PD principles can be maintained and response rates and participation may even be greater when using it.

Using data from mHealth apps to inform person-centred practice: A discussion paper.

BACKGROUND: mHealth applications (apps) are tools that can enhance research by efficiently collecting and storing large amounts of data. However, data collection alone does not lead to change. Innovation and practice change occur through utilisation of evidence. The volume of data collected raises questions regarding utilisation of data by nurses and midwives, and how data from mHealth apps can be used to improve person-centred practice. There is limited empirical evidence and a lack of direction from global health authorities to guide nurses and midwives in this area. AIM: To describe strategies for nurses and midwives that could enhance the effective use of data generated by mHealth apps to inform person-centred practice. The purpose of this paper is to stimulate reflection and generate actions for data utilisation when using mHealth apps in nursing research and practice. METHODS: This discussion paper has been informed by current evidence, the integrated-Promoting Action on Research Implementation in Health Services (i-PARIHS) framework, and research experience as part of doctoral study. FINDINGS: Before engaging in data collection using mHealth apps, nurses and midwives would benefit from considering the nature of the evidence collected, available technological infrastructure, and staff skill levels. When collecting data and interpreting results, use of a team approach supported by engaged leadership and external facilitation is invaluable. This provides support to operate apps, and more importantly use the data collected to inform person-centred practice. CONCLUSIONS: This paper addresses the limited available evidence to guide nurses and midwives when using mHealth apps to collect and use data to inform practice change. It highlights the need for appropriate technology, external facilitative support, engaged leadership, and a team approach to collect meaningful evidence using mHealth apps. Clinicians, leaders, and researchers can apply the strategies provided to enhance the use of mHealth apps and ensure translation of evidence into practice.

How the expert nursing role was used to facilitate the co-design of a patient interview study.

BACKGROUND: Co-design is a research method that seeks to engage service users in research. The approach fosters inclusivity and shared power by having researchers and research participants work together for some or all of a study. AIM: To describe the experience of co-designing a patient interview study from the perspective of an expert stoma nurse, using a case-study approach and reflexive methods. DISCUSSION: Valuing expert patients' experiences when conducting research about them enabled patients to be trained as participant researchers to co-design and undertake a patient interview study. The co-design process enabled the researcher to develop a greater recognition of the fact that experience of looking after people with stomas does not equate to expertise in knowing what it is like to have a stoma. This enriched her research experience and increased the authenticity of the study. CONCLUSION: Co-designing a study with service users creates challenges for nurse researchers. They must pay attention to relational changes, time, planning and organisation to ensure that they conduct their research rigorously and ethically, and safeguard the co-researchers and other participants from potential risks. IMPLICATIONS FOR PRACTICE: Co-designing research is critical for developing effective, patient-centred bodies of evidence. Nurse researchers can play a critical role but must be prepared to shift from directive to participatory methods to identify appropriate, patient-focused improvements.

Enhancing new graduate nurses and midwives person-centredness through clinical supervision during COVID-19; evaluation of a non-randomized intervention study.

AIM: The aim of the study was to evaluate a person-centred model of clinical supervision to enhance person-centredness. DESIGN: Experimental, quantitative. METHODS: One hundred and three New Graduates were supported to reflect through a person-centred lens (July-December 2020). Evaluation was undertaken at 6 months using: the Manchester Clinical Supervision Scale-26 (effectiveness of supervision) and the Person-centred Practice Inventory (measures attributes of the nurse/midwife, the care environment and person-centred processes). Due to participation difficulties, scores were calculated by attendance rates using descriptive and inferential statistics. RESULTS: Regular attendees scored higher on the supervision's effectiveness; however, this did not reach efficacy. 'Finding time' to attend contributed to low scores. Supervision scored well on its supportive function when attended. Many New Graduates perceived a decline in their care environment. Attendance aside, New Graduates averaged an increased in their person-centred attributes and processes. Greater participation was found in those who scored higher at baseline on their person-centred attributes and processes, and this higher scoring continued at 6 months than those who attended less. CONCLUSION: New Graduates who perceive themselves as person-centred and reflective at baseline are more likely to attend a person-centred clinical supervision and score higher at 6 months than those who attended less often. New Graduates found support within supervision during challenging times. IMPLICATIONS FOR PRACTICE FOR PROFESSIONAL AND/OR PATIENT CARE: For successful implementation of Person-centred Clinical Supervision, New Graduates need support to attend, as attendance supports them to begin seeing value in the process. IMPACT: This intervention kept person-centred practice at the forefront of New Graduates reflection, in a time of extreme change. The research has implications for nursing and midwifery management with the imperative to deliver person-centred care and create the person-centred cultures for staff to feel supported and empowered. REPORTING METHOD: Transparent Evaluation of Non-randomized Designs (TREND). PATIENT OF PUBLIC CONTRIBUTION: No patient or public contribution. CONTRIBUTION TO WIDER COMMUNITY: New Graduates grow their person-centredness over their transitioning year; however, this can be enhanced with regular clinical supervision underpinned by person-centred theory. Clinical supervisors can provide support to New Graduates when the environment is challenged.

Career optimism and job-related well-being of Australian nurses during COVID-19: A cross-sectional study.

BACKGROUND: Australia has been confronted with a severe nursing deficit, making it difficult to maintain a strong healthcare workforce capable of meeting the mounting demands of healthcare organizations. AIMS: This study aimed to understand how personal and organizational resources influence career optimism and job-related affective well-being of Australian nurses during a pandemic using the Conservation of Resource Theory. METHODS: A cross-sectional online survey was emailed to 123 Australian nurses from January to February 2021. The survey consisted of self-reported measures, including mindfulness, career optimism, job-related affective well-being, personal and job resources measures, and the Dirty Dozen scale. Correlations, independent sample t-test, and a series of hierarchical regressions were conducted on the cross-sectional data with SPSS Version 27. The STROBE checklist was used to report the results. RESULTS: Findings suggested that mindfulness, perceived supervisor support, and job autonomy were significant predictors of job-related well-being, whereas mindfulness, perceived supervisor support, and opportunities for professional growth contributed more to career optimism of nurses during a health crisis. Male nurses in this study reported significantly higher mindfulness, career optimism, and job-related well-being levels than female nurses. LINKING EVIDENCE TO ACTION: Developing mindfulness among nurses, allocating organizational resources to facilitate more supervisor support, and providing job autonomy may enhance career optimism and job-related well-being of nursing staff who work in disruptive and high-demand work environments such as those experienced during the COVID-19 health crisis. Supervisors should also facilitate and encourage nurses to reflect and be mindful of their behaviors with their peers and patients which can help to reduce exploitative or arrogant behaviors in the workplace.

Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study.

Historically, the majority of patients with complement-mediated atypical hemolytic uremic syndrome (CaHUS) progress to end-stage kidney disease (ESKD). Single-arm trials of eculizumab with a short follow-up suggested efficacy. We prove, for the first time to our knowledge, in a genotype matched CaHUS cohort that the 5-year cumulative estimate of ESKD-free survival improved from 39.5% in a control cohort to 85.5% in the eculizumab-treated cohort (hazard ratio, 4.95; 95% confidence interval [CI], 2.75-8.90; P = .000; number needed to treat, 2.17 [95% CI, 1.81-2.73]). The outcome of eculizumab treatment is associated with the underlying genotype. Lower serum creatinine, lower platelet count, lower blood pressure, and younger age at presentation as well as shorter time between presentation and the first dose of eculizumab were associated with estimated glomerular filtration rate >60 ml/min at 6 months in multivariate analysis. The rate of meningococcal infection in the treated cohort was 550 times greater than the background rate in the general population. The relapse rate upon eculizumab withdrawal was 1 per 9.5 person years for patients with a pathogenic mutation and 1 per 10.8 person years for those with a variant of uncertain significance. No relapses were recorded in 67.3 person years off eculizumab in those with no rare genetic variants. Eculizumab was restarted in 6 individuals with functioning kidneys in whom it had been stopped, with no individual progressing to ESKD. We demonstrated that biallelic pathogenic mutations in RNA-processing genes, including EXOSC3, encoding an essential part of the RNA exosome, cause eculizumab nonresponsive aHUS. Recessive HSD11B2 mutations causing apparent mineralocorticoid excess may also present with thrombotic microangiopathy.

Clinical supervision: A mechanism to support person-centred practice? An integrative review of the literature.

AIMS: To explore whether clinical supervision has an influence on person-centred practice. BACKGROUND: The ability to deliver person-centred care and the ability to engage in reflective practice are two key skills expected of nurses. Person-centred care shifts nurses thinking from the patient's disease state to their personhood. Clinical supervision has been proposed as a place to explore person-centred care. Person-centred practice extends person-centredness to enhance healthful relationships between care providers, services users and their significant others. METHODS: An integrative literature was conducted with the key words 'clinical supervision' and 'person-centredness' within CINAHL, Medline, PsychInfo and Google Scholar. The PRISMA statement was used to report the identification, selection, appraisal and synthesis of articles and PRISMA diagram reports the selection process. The Mixed Methods Appraisal Tool was used to appraise the articles, followed by a thematic analysis. The Template for Intervention Description and Replication was used to explore the concept of clinical supervision. RESULTS: Twelve articles met the inclusion criteria. Person-centredness and clinical supervision are not universally defined concepts. Clinical supervision in this context was mainly an adjunct to educative programmes. Clinical supervision has a positive influence on participants' person-centred attributes, but the influence on others is less clear. The clinical supervisor's qualities are pivotal to supervisions' success. CONCLUSIONS: Clinical supervision is a common reflective strategy used to explore nursing practice, yet as a strategy to enhance person-centred practices, is under-researched. Participants can experience person-centredness when their supervisor has person-centred attributes, an important aspect in delivering person-centred care. RELEVANCE TO CLINICAL PRACTICE: This review identified a gap in the literature between two common nursing concepts 'clinical supervision' and 'person-centred care/practices'. There is a need to conduct further research into the combination of these concepts to seek ways to embed person-centredness into health care and everyday nursing practice.

The impact of ageing simulation education on qualified acute care nurses' empathy towards older people: A mixed-methods study.

AIMS AND OBJECTIVES: To evaluate the influence of an ageing simulation intervention on qualified acute care nurses' empathy towards older people. BACKGROUND: Life expectancy is increasing globally with an increased incidence of older people accessing healthcare services. As such, it is essential for qualified nurses within hospital settings to connect with older people by establishing healthful relationships. Empathy is an essential component of healthful nurse-patient relationships. Fortunately, empathy can be taught through education. DESIGN: A convergent mixed-methods design was utilised. METHODS: Nurses (N = 95) undertook an 8-hour ageing simulation intervention. Data were collected from April 2019 to May 2020 across three time points: before the intervention (T0); immediately after (T1) and at three months follow-up (T2). A mix of convenience (intervention) and purposeful (follow-up) sampling techniques were utilised. Quantitative data were collected via the Interpersonal Reactivity Index survey (n = 86) and analysed using repeat measures ANOVA to compare mean scores across time points. Qualitative data were collected via debriefing discussions (N = 95) and focus groups (n = 38), and analysed using a systematic thematic analysis method. Data convergence occurred during the interpretation phase. Study reported with the TREND checklist. RESULTS: Primarily, when quantitative and qualitative findings were merged they confirmed each others' empathy outcomes. Quantitative results showed a statistically significant increase in affective and cognitive empathy levels among nurses post-intervention. Qualitative findings expanded on quantitative results and revealed an increase in nurses' affective, cognitive and behavioural empathy represented in themes 'enhancing my empathy', 'impact of ageing', 'from self to others' and 'person-centred moments'. CONCLUSIONS: This study adds empirical evidence how a mixed-methods design can be used to evaluate the influence of an ageing simulation intervention on nurses' empathy levels. RELEVANCE TO CLINICAL PRACTICE: Ageing simulation interventions are a suitable experiential educational approach to improve acute care nurses' affective, cognitive and behavioural empathy towards older people.

Assessing the Impact of Prophylactic Eculizumab on Renal Graft Survival in Atypical Hemolytic Uremic Syndrome.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare cause of end-stage kidney disease and associated with poor outcomes after kidney transplantation from early disease recurrence. Prophylactic eculizumab treatment at the time of transplantation is used in selected patients with aHUS. We report a retrospective case note review describing transplant outcomes in patients with aHUS transplanted between 1978 and 2017, including those patients treated with eculizumab. METHODS: The National Renal Complement Therapeutics Centre database identified 118 kidney transplants in 86 recipients who had a confirmed diagnosis of aHUS. Thirty-eight kidney transplants were performed in 38 recipients who received prophylactic eculizumab. The cohort not treated with eculizumab comprised 80 transplants in 60 recipients and was refined to produce a comparable cohort of 33 transplants in 32 medium and high-risk recipients implanted since 2002. Complement pathway genetic screening was performed. Graft survival was censored for graft function at last follow-up or patient death. Graft survival without eculizumab treatment is described by complement defect status and by Kidney Disease: Improving Global Outcomes risk stratification. RESULTS: Prophylactic eculizumab treatment improved renal allograft survival ( P = 0.006) in medium and high-risk recipients with 1-y survival of 97% versus 64% in untreated patients. Our data supports the risk stratification advised by Kidney Disease: Improving Global Outcomes. CONCLUSIONS: Prophylactic eculizumab treatment dramatically improves graft survival making transplantation a viable therapeutic option in aHUS.

A niche for axial stem cells - A cellular perspective in amniotes.

The head-tail axis in birds and mammals develops from a growth zone in the tail-end, which contains the node. This growth zone then forms the tailbud. Labelling experiments have shown that while many cells leave the node and tailbud to contribute to axial (notochord, floorplate) and paraxial (somite) structures, some cells remain resident in the node and tailbud. Could these cells be resident axial stem cells? If so, do the node and tailbud represent an instructive stem cell niche that specifies and maintains these stem cells? Serial transplantation and single cell labelling studies support the existence of self-renewing stem cells and heterotopic transplantations suggest that the node can instruct such self-renewing behaviour. However, only single cell manipulations can reveal whether self-renewing behaviour occurs at the level of a cell population (asymmetric or symmetric cell divisions) or at the level of single cells (asymmetric divisions only). We combine data on resident cells in the node and tailbud and review it in the context of axial development in chick and mouse, summarising our current understanding of axial stem cells and their niche and highlighting future directions of interest.

The experience of staff utilizing data to evaluate and improve person-centred practice: An action research study.

AIM: Explore how nurses and midwives use patient experience data collected from a mobile health app to influence the development of person-centred practice. DESIGN: Participatory action research, underpinned by the Person-Centred Nursing Framework and Practice Development principles. METHODS: Six clinical units in a large health district engaged in three action cycles from 2018 to 2020 using a mobile health app. Nursing/midwifery staff on the units (N = 177) utilized data collected via the app to evaluate and improve person-centred practice. A pre-post survey using the PCPI-S was conducted to evaluate staff perceptions of person-centredness. Data from the surveys (n = 101 in 2018 and n = 102 in 2020) and 17 semi-structured interviews were used to understand the influence working with these data had on person-centred practice. The Guidelines for Best Practices in the Reporting of Participatory Action Research have been used to report this study. RESULTS: Improvements in person-centred practice were noted across both data sets. There was a statistically significant increase in two domains of the PCPI-S in the independent t-test and across all three domains in the paired t-test results. Thematic analysis resulted in the identification of six themes: Getting everyone on board, once we understood, keeping on track, there's a person in the bed, knowing you're doing a good job and improving over time. CONCLUSION: Engaging with the data collected from the app in a facilitated and collaborative way results in increases in person-centredness. IMPACT: This study provides insight into how nurses and midwives used data from a mHealth app to evaluate and improve person-centred practice. Utilizing the data generated by the app resulted in increased person-centredness amongst staff and changes to practice and culture. Nursing and midwifery teams who are supported to engage with patient experience data in an action-oriented way will see person-centred practice improvements, affecting patients and staff.

Professional values in student nurse education: An integrative literature review.

AIM: The aim is to understand current research into the impact of undergraduate nursing education on the development of professional values. BACKGROUND: Values are evident in the professional standards for nurses and the guidelines and healthcare policies of many countries. These professional values guide decisions and behaviour and are recognised as an essential component in the professions ability to provide safe and professional care. This literature review presents the current research on the impact of education on professional values in undergraduate nurse education. DESIGN: An integrative review of the findings was conducted to provide insight into the current research on the professional value development in undergraduate nurses. DATA SOURCES: CINAHL, PubMed and Scopus. REVIEW METHODS: A literature search was undertaken within defined date parameters 2010-2021 using a systematic approach. The Preferred Reporting Items for Systematic Reviews and Meta-analysis guide PRISMA was used to guide and illustrate the process. Papers were assessed for quality using the Mixed Methods Appraisal Tool. RESULTS: Two distinct areas of inquiry were identified. (a) Changes in professional values as an outcome of undergraduate nursing education or (b) changes in professional values as an outcome of specifically designed educational content. These areas were further explored to better understand the influences of undergraduate education on students' professional values. CONCLUSION: There is a lack of evidence in the literature to support the premise that professional values develop in line with academic year progression; however, there is strong evidence to support the inclusion of explicit learning in undergraduate education that engages students in education specifically designed to explore and develop professional values.

Beyond the technology: Applying the NLN Jeffries Simulation Theory in the context of aging simulation.

BACKGROUND: Caring for older people has become a focus for simulation-based education. Specifically, aging simulation has emerged as an innovative and effective educational approach that utilizes aging-suits for nurses to gain insight into the aging process and the everyday difficulties faced by older people. There is limited evidence within the literature about how researchers and educators support decision-making processes in the design, implementation, and evaluation of aging simulation programs. AIM: This is a theoretical development paper and its purpose is to explore the practical application of the Jeffries Simulation Theory in the design, implementation, and evaluation of an aging simulation program to contribute to knowledge development and guide educational practices for nurse educators and researchers. RESULTS: The authors describe a practical application of the theory to an aging simulation program using the five key theory components: context, background, design, simulation experience, and outcomes. Specific theory strengths are highlighted in practical examples generated from the authors aging simulation interventional study example: engaging stakeholders, consideration of additional observer simulation roles, reflective debrief discussions, influence of facilitator and participant personal attributes, and outcomes beyond the participants. CONCLUSION: The Jeffries Simulation Theory is well suited for underpinning the design, implementation, and evaluation of aging simulation programs.

Factors that positively influence in-hospital exclusive breastfeeding among women with gestational diabetes: An integrative review.

PROBLEM: Women with gestational diabetes have higher rates of introducing infant formula before leaving hospital. BACKGROUND: Despite health professional support, less women with gestational diabetes exclusively breastfeed in hospital. AIM: To find factors that positively influence in-hospital exclusive breastfeeding practices among women with gestational diabetes. METHODS: An online search was performed in Medline, Scopus, Pubmed, CINAHL and Cochrane databases. Studies containing the keywords gestational diabetes and breastfeeding were retrieved. FINDINGS: Authors identified 1935 papers from search criteria. Twenty-six papers with no restrictions on research design met inclusion criteria and were included in the review. Factors were divided into personal, antenatal, intrapartum and postnatal factors. The main modifiable factors that were associated with improved in-hospital exclusive breastfeeding rates were having a strong intention to breastfeed, being confident, feeling supported and having continuity of education and support. Women's main reasons to introduce formula were related to baby's hypoglycaemia, delayed lactogenesis II and perceived low milk supply. Skin-to-skin contact after birth combined with frequent breastfeeds were effective ways to improve in-hospital exclusive breastfeeding rates. CONCLUSION: Influencing factors such as women's breastfeeding intention, confidence and ongoing support are no different to the general population of women. However, promoting skin-to-skin contact after birth combined with frequent feeds are crucial for women with gestational diabetes who are more likely to introduce formula due to delayed lactogenesis II and fear of neonatal hypoglycaemia. There is a need for developing educational and supportive interventions that are tailored specifically for women who have gestational diabetes.

Effect of intensive blood pressure control on subtypes of mild cognitive impairment and risk of progression from SPRINT study.

BACKGROUND: To examine the effect of intensive blood pressure control on the occurrence of subtypes of mild cognitive impairment (MCI) and determine the risk of progression to dementia or death. METHODS: Secondary analysis of a randomized trial of community-dwelling adults (≥50 years) with hypertension. Participants were randomized to a systolic blood pressure (SBP) goal of <120 mm Hg (intensive treatment; n = 4678) or <140 mm Hg (Standard treatment; n = 4683). Outcomes included adjudicated MCI, MCI subtype (amnestic, non-amnestic, multi-domain, single domain), and probable dementia. Multistate survival models were used to examine transitions in cognitive status accounting for the competing risk of death. RESULTS: Among 9361 randomized participants (mean age, 67.9 years; 3332 women [35.6%]), 640 participants met the protocol definition for MCI, with intensive treatment reducing the risk of MCI overall (hazard ratio [HR], 0.81 [95% confidence interval {CI}, 0.69-0.94]), as previously reported. This effect was largely reflected in amnestic subtypes (HR, 0.78 [95% CI, 0.66-0.92]) and multi-domain subtypes (HR, 0.78 [95% CI, 0.65-0.93]). An adjudication of MCI, as compared with normal cognitive function, substantially increased the probability of progressing to probable dementia (5.9% [95% CI: 4.5%-7.7%] vs. 0.6% [95% CI: 0.3%-0.9%]) and to death (10.0% [95% CI: 8.3%-11.9%] vs. 2.3% [95% CI: 2.0%-2.7%]) within 2 years. CONCLUSIONS: Intensive treatment reduced the risk for amnestic and multi-domain subtypes of MCI. An adjudication of MCI was associated with increased risk of progression to dementia and death, highlighting the relevance of MCI as a primary outcome in clinical and research settings.

How nurses and midwives engage with patient experience data to inform person-centred quality and safety improvements.

AIMS AND OBJECTIVES: To explore how nurses and midwives engage with patient experience data collected via a mobile health app to inform person-centred practice improvements. BACKGROUND: A large amount of data is collected in healthcare, yet there is limited evidence outlining how nursing and midwifery staff utilise patient experience data to inform person-centred quality and safety improvements. METHODS: This study utilised action research, underpinned by Practice Development methodology and has been reported using the SQUIRE 2.0 checklist. Six clinical units (medical short stay, acute medical, surgical, oncology/haematology, day surgery and maternity) in a large health district in Australia engaged in three cycles of data collection using a mobile health app. The app captured patient experience data relating to the person-centred KPIs developed and tested by McCance et al. (2012). Staff used the data to develop and evaluate person-centred practice. RESULTS: A number of improvements in scores and practice occurred through engaging with the data in a cyclical way. All six clinical units saw an improvement in four or more of the KPIs in the patient survey results from cycle one to cycle three, with two clinical units improving in all eight. On average across the six units, there was also an increase in time nurses/midwives were visible to their patients, an increase in clinical documentation reflecting the patients' needs and what was important to them, an increase in positive comments and a decrease in negative comments in patient stories. CONCLUSION: This study shows that collecting and utilising data from the person-centred KPIs in a collaborative and cyclical way lead to enhanced patient experience and the development and implementation of person-centred quality and safety improvements. RELEVANCE TO CLINICAL PRACTICE: Capturing and utilising data that are meaningful to nursing/midwifery teams in a cyclical, action-orientated approach result in person-centred practice improvements that enhance the experience of those that are receiving and delivering patient care.

Further delineation of phenotypic spectrum of SCN2A-related disorder.

SCN2A-related disorders include intellectual disability, autism spectrum disorder, seizures, episodic ataxia, and schizophrenia. In this study, the phenotype-genotype association in SCN2A-related disorders was further delineated by collecting detailed clinical and molecular characteristics. Using previously proposed genotype-phenotype hypotheses based on variant function and position, the potential of phenotype prediction from the variants found was examined. Patients were identified through the Deciphering Developmental Disorders study and gene matching strategies. Phenotypic information and variant interpretation evidence were collated. Seventeen previously unreported patients and five patients who had been previously reported (but with minimal phenotypic and segregation data) were included (10 males, 12 females; median age 10.5 years). All patients had developmental delays and the majority had intellectual disabilities. Seizures were reported in 15 of 22 (68.2%), four of 22 (18.2%) had autism spectrum disorder and no patients were reported with episodic ataxia. The majority of variants were de novo. One family had presumed gonadal mosaicism. The correlation of the use of sodium channel-blocking antiepileptic drugs with phenotype or genotype was variable. These data suggest that variant type and position alone can provide some predictive information about the phenotype in a proportion of cases, but more precise assessment of variant function is needed for meaningful phenotype prediction.

C3 Glomerulopathy and Related Disorders in Children: Etiology-Phenotype Correlation and Outcomes.

BACKGROUND AND OBJECTIVES: Membranoproliferative GN and C3 glomerulopathy are rare and overlapping disorders associated with dysregulation of the alternative complement pathway. Specific etiologic data for pediatric membranoproliferative GN/C3 glomerulopathy are lacking, and outcome data are based on retrospective studies without etiologic data. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: A total of 80 prevalent pediatric patients with membranoproliferative GN/C3 glomerulopathy underwent detailed phenotyping and long-term follow-up within the National Registry of Rare Kidney Diseases (RaDaR). Risk factors for kidney survival were determined using a Cox proportional hazards model. Kidney and transplant graft survival was determined using the Kaplan-Meier method. RESULTS: Central histology review determined 39 patients with C3 glomerulopathy, 31 with immune-complex membranoproliferative GN, and ten with immune-complex GN. Patients were aged 2-15 (median, 9; interquartile range, 7-11) years. Median complement C3 and C4 levels were 0.31 g/L and 0.14 g/L, respectively; acquired (anticomplement autoantibodies) or genetic alternative pathway abnormalities were detected in 46% and 9% of patients, respectively, across all groups, including those with immune-complex GN. Median follow-up was 5.18 (interquartile range, 2.13-8.08) years. Eleven patients (14%) progressed to kidney failure, with nine transplants performed in eight patients, two of which failed due to recurrent disease. Presence of >50% crescents on the initial biopsy specimen was the sole variable associated with kidney failure in multivariable analysis (hazard ratio, 6.2; 95% confidence interval, 1.05 to 36.6; P<0.05). Three distinct C3 glomerulopathy prognostic groups were identified according to presenting eGFR and >50% crescents on the initial biopsy specimen. CONCLUSIONS: Crescentic disease was a key risk factor associated with kidney failure in a national cohort of pediatric patients with membranoproliferative GN/C3 glomerulopathy and immune-complex GN. Presenting eGFR and crescentic disease help define prognostic groups in pediatric C3 glomerulopathy. Acquired abnormalities of the alternative pathway were commonly identified but not a risk factor for kidney failure.

Maintaining momentum in action research.

BACKGROUND: Action research (AR) provides a robust platform for collaboration to develop and evaluate nursing practice. It results in several outcomes, including changes in evidence-based practice, the development of research capacity, and the evaluation and sustainability of interventions, all of which can be seen as benefits compared to other approaches. However, the methodology involves cycles of action, reflection, theory and practice, so it can be challenging to maintain momentum when engaging with teams over long periods of time. AIM: To offer strategies for maintaining momentum when using AR in nursing research. DISCUSSION: Three strategies for maintaining momentum when undertaking AR are covered. Theory, literature and experience of using AR in which the strategies of 'connecting as people', 'working with the context' and 'understanding the influence of the leadership team' made a considerable difference in maintaining momentum and are drawn on. CONCLUSION: Maintaining momentum in studies that use AR can be arduous, but critical reflection enables researchers to identify and overcome the challenges that arise. Researchers undertaking AR can apply the three strategies provided or other approaches to maintain momentum during all phases of a study. IMPLICATIONS FOR PRACTICE: Maintaining momentum in AR studies is more successful when researchers connect with those with whom they are undertaking research. It is advantageous for nurse researchers to reflect on and understand the influence of the leadership team and context rather than try to adapt them to the study's or their own needs.