Seven decades of clinical experience with Wilson's disease: Report from the national reference centre in Poland.

BACKGROUND AND PURPOSE: Wilson's disease (WD) is a rare autosomal recessive disorder causing excessive copper deposition and a spectrum of manifestations, particularly neurological and hepatic symptoms. We analysed the clinical characteristics of patients with WD admitted to the country's only reference centre, which provided long-term care to most adult patients in Poland over seven decades (pre-1959 to 2019). METHODS: Electronic prospective data collection began in the 2000s and, for prior years, medical records were analysed retrospectively. Demographic and clinical characteristics, treatment and outcomes were analysed by decade of diagnosis. Life-years lost were estimated in patients with WD compared with the general population. Kaplan-Meier curves were used for a time-to-death analysis using 2000-2009 as a reference. RESULTS: In total, 929 patients were analysed. The number of patients increased from 21 before 1959 to 315 for 2000 to 2009 period. Mostly males were diagnosed before the 1990s, but the numbers of female patients diagnosed increased thereafter. Initially, most patients presented with neurological manifestations; however, the incidence of hepatic manifestations and asymptomatic presentations increased over time as patients were diagnosed early and consequently were more independent at diagnosis. Fewer Kayser-Fleischer rings were detected recently. Prior to 1970, patients were treated with D-penicillamine (DP); however, since the introduction of zinc, both therapies have been used as often. Since the 1990s, switches between DP and zinc were recorded in 6%-7% of patients. Consistent improvement in survival has been observed over the years. CONCLUSIONS: This is the largest cohort of patients with WD reported in Poland, with the longest follow-up. Earlier diagnosis and prognosis have improved over seven decades.

Wound Healing Impairment in Type 2 Diabetes Model of Leptin-Deficient Mice-A Mechanistic Systematic Review.

Type II diabetes mellitus (T2DM) is one of the most prevalent diseases in the world, associated with diabetic foot ulcers and impaired wound healing. There is an ongoing need for interventions effective in treating these two problems. Pre-clinical studies in this field rely on adequate animal models. However, producing such a model is near-impossible given the complex and multifactorial pathogenesis of T2DM. A leptin-deficient murine model was developed in 1959 and relies on either dysfunctional leptin (ob/ob) or a leptin receptor (db/db). Though monogenic, this model has been used in hundreds of studies, including diabetic wound healing research. In this study, we systematically summarize data from over one hundred studies, which described the mechanisms underlying wound healing impairment in this model. We briefly review the wound healing dynamics, growth factors' dysregulation, angiogenesis, inflammation, the function of leptin and insulin, the role of advanced glycation end-products, extracellular matrix abnormalities, stem cells' dysregulation, and the role of non-coding RNAs. Some studies investigated novel chronic diabetes wound models, based on a leptin-deficient murine model, which was also described. We also discussed the interventions studied in vivo, which passed into human clinical trials. It is our hope that this review will help plan future research.

Nursing interventions to improve care of people living with dementia in hospital: A mixed methods systematic review.

BACKGROUND: There are growing numbers of people living with dementia being admitted to acute care hospitals. Hospitalization for people living with dementia can be difficult and is often associated with negative outcomes. Nurses play a significant role in shaping the hospital experience of people living with dementia, and there have been efforts to design, implement and evaluate interventions to improve nursing care of people living with dementia. OBJECTIVE: To synthesize evidence on the effectiveness of, and experiences with, nursing interventions to improve care of hospitalized people living with dementia. DESIGN: Mixed methods systematic review following the JBI convergent segregated approach to synthesis and integration of findings. METHODS: The quantitative component considered studies that evaluated nursing interventions to improve the care of people living with dementia in hospital, comparing the intervention to usual care, other therapeutic modalities, or no comparator. The qualitative component considered studies that explored the experiences of nursing interventions from the perspectives of people living with dementia, caregivers, and nurses. A total of 8 databases were used to search for published and unpublished studies. Titles, abstracts, and full text selections were screened by two or more independent reviewers and assessed for methodological quality. RESULTS: A total of 38 studies were included in the review, 24 quantitative, 9 qualitative and 5 mixed method designs. Critical appraisal scores were moderate. All studies regardless of methodological quality were included in the review. Interventions were grouped as principally related to (1) dementia education for nurses, (2) technology, (3) nursing skills, and (4) physical environment. Outcomes are presented related to health outcomes of people living with dementia; nurses' knowledge, confidence, and self-efficacy; and health system outcomes. As the interventions were heterogeneous, a meta-analysis of quantitative findings was not possible. The qualitative analysis incorporated 60 findings from 12 studies and led to nine categories and two synthesized findings recognizing external influences on nurses' practice with people living with dementia and the importance of interventions to humanize nurses' work with people living with dementia. Integration of the quantitative and qualitative results demonstrates the need to recognize the role of organization- and unit-level factors in the design and implementation of effective interventions. CONCLUSIONS: There is limited high-quality evidence to demonstrate the effectiveness of interventions to improve nursing care of people living with dementia in hospital. Using approaches to intervention design and implementation that draw on models of behavior change and learning health systems may support effective change. SYSTEMATIC REVIEW REGISTRATION NUMBER: PROSPERO 2021CRD42021230951.

Interventions to Improve the Nursing Care of People with Dementia in Canadian Hospitals: An Environmental Scan.

As the number of people with dementia admitted to hospitals is expected to grow, now is the time to identify methods to improve nursing care of this population. We conducted an environmental scan to identify and describe interventions in Canadian hospitals to improve the nursing care of people with dementia, how they are being evaluated and what issues influence the success of interventions. Methods included a search of published and unpublished literature and key stakeholder interviews. Interventions are described under three categories: (1) interventions to improve nurses' knowledge, attitudes and skills; (2) interventions to address responsive behaviours; and (3) interventions to help nurses individualize care. The evaluation of interventions rarely included an evaluation of effectiveness and more often included a qualitative evaluation of nurses' experiences with interventions. We summarize the factors affecting the implementation of interventions following the Consolidated Framework for Implementation Research (Damschroder et al. 2009) and suggest strategies for supporting the success of interventions to improve patient care and the experiences of nurses working with people with dementia.