Nurse-Led/Involved Home-Based Interventions for Older Adults With Chronic Obstructive Pulmonary Disease (COPD): A Mixed-Methods Systematic Review.

Aims: To determine the effectiveness of nurse-led/involved home-based interventions for older people with COPD and to explore the experiences of older people and nurses with the interventions.

Design: A mixed-methods systematic review following the JBI methodology for mixed-methods systematic reviews.

Data Sources: The search included relevant and peer-reviewed studies published from January 2010 to December 2023 in CINAHL, MEDLINE, Cochrane Central Register of Controlled Trials, PsycINFO, EMBASE, JBI, EMCARE and ProQuest.

Genomic reanalysis of a pan-European rare-disease resource yields new diagnoses.

Genetic diagnosis of rare diseases requires accurate identification and interpretation of genomic variants. Clinical and molecular scientists from 37 expert centers across Europe created the Solve-Rare Diseases Consortium (Solve-RD) resource, encompassing clinical, pedigree and genomic rare-disease data (94.5% exomes, 5.

Bi-allelic KICS2 mutations impair KICSTOR complex-mediated mTORC1 regulation, causing intellectual disability and epilepsy.

Nutrient-dependent mTORC1 regulation upon amino acid deprivation is mediated by the KICSTOR complex, comprising SZT2, KPTN, ITFG2, and KICS2, recruiting GATOR1 to lysosomes. Previously, pathogenic SZT2 and KPTN variants have been associated with autosomal recessive intellectual disability and epileptic encephalopathy. We identified bi-allelic KICS2 variants in eleven affected individuals presenting with intellectual disability and epilepsy.

A Nudge to the Truth: Atom Conservation as a Hard Constraint in Models of Atmospheric Composition Using a Species-Weighted Correction.

Computational models of atmospheric composition are not always physically consistent. For example, not all models respect fundamental conservation laws such as conservation of atoms in an interconnected chemical system. In well performing models, these unphysical deviations are often ignored because they are frequently minor, and thus only need a small nudge to perfectly conserve mass.

Genomic modifications for enhanced antibiotic production in rifamycin derivative-producing S699 strains: focusing on and genes.

Rifamycin and its derivatives are natural products that belong to the class of antibiotic-active polyketides and have significant therapeutic relevance within the therapy scheme of tuberculosis, a worldwide infectious disease caused by . Improving the oral bioavailability of rifamycin B was achieved through semisynthetic modifications, leading to clinically effective derivatives such as rifampicin. Genetic manipulation of the rifamycin polyketide synthase gene cluster responsible for the production of rifamycin B in the strain S699 represents a promising tool to generate new rifamycins.

Epithelial OPA1 links mitochondrial fusion to inflammatory bowel disease.

Dysregulation at the intestinal epithelial barrier is a driver of inflammatory bowel disease (IBD). However, the molecular mechanisms of barrier failure are not well understood. Here, we demonstrate dysregulated mitochondrial fusion in intestinal epithelial cells (IECs) of patients with IBD and show that impaired fusion is sufficient to drive chronic intestinal inflammation.

Prevalence of metal hypersensitivity in pediatric spine surgery.

Purpose: Delayed metal hypersensitivity reactions can cause complications in spine surgery. Currently, there is no information on the prevalence of metal hypersensitivity in pediatric patients undergoing spine surgery. The objective of this study is to determine the prevalence of metal hypersensitivity in pediatric patients undergoing spinal instrumentation.

One is not like the other: health quality of life scores vary in neuromuscular EOS by diagnosis.

Purpose: The etiology of early-onset scoliosis (EOS) has been shown to significantly influence baseline parent-reported health-related quality of life (HrQOL). In combining these etiology groups, we obligatorily lump together many disparate diagnoses, particularly true in the neuromuscular (NM) cohort. We sought to evaluate the influence of underlying neuromuscular diagnosis on the HrQOL at 5 years following surgery for EOS.

Blarcamesine for the treatment of Early Alzheimer's Disease: Results from the ANAVEX2-73-AD-004 Phase IIB/III trial.

Background: There are no approved oral disease-modifying treatments for Alzheimer's disease (AD).

Objectives: The objective of this study was to assess efficacy and safety of blarcamesine (ANAVEX®2-73), an orally available small-molecule activator of the sigma-1 receptor (SIGMAR1) in early AD through restoration of cellular homeostasis including autophagy enhancement.

Design: ANAVEX2-73-AD-004 was a randomized, double-blind, placebo-controlled, 48-week Phase IIb/III trial.

Pharmacist-initiated naloxone discharge prescribing for high-risk hospitalized internal medicine patients.

Pharmacists nationwide may play a critical role in expanding naloxone access after several states enacted legislation to allow pharmacist prescribing of opioid antagonists. This created a unique opportunity for inpatient pharmacists to participate in combating the opioid epidemic by prescribing naloxone at hospital discharge. A multifaceted intervention was developed to identify and educate hospitalized patients eligible for naloxone prescribing.

Practical Considerations for Odevixibat Treatment in Patients with Progressive Familial Intrahepatic Cholestasis: A Single-Center Case Series.

: Patients with progressive familial intrahepatic cholestasis (PFIC) experience cholestasis-associated symptoms, including severe pruritus. Odevixibat is an ileal bile acid transporter inhibitor indicated for treatment of PFIC in the European Union and for the treatment of pruritus in PFIC in the United States. The aim of the current study was to characterize the real-world effectiveness and safety of odevixibat in patients with PFIC.

Patient's Perception of Primary Health Care Provision With Respect to Access, Continuity and Coordination-InCept: An International Qualitative Perspective.

Health care systems are confronted with an increasing burden of (multi-)morbidity and a shortfall of healthcare providers. Coordination and continuity of care in chronic and multi-morbid patient is especially important. As qualitative patient experience data within care processes is scarce, we aim to increase the understanding of chronically ill patient's perspectives by assessing patient experiences in different health systems while treated in primary care.

EEFSEC deficiency: A selenopathy with early-onset neurodegeneration.

Inborn errors of selenoprotein expression arise from deleterious variants in genes encoding selenoproteins or selenoprotein biosynthetic factors, some of which are associated with neurodegenerative disorders. This study shows that bi-allelic selenocysteine tRNA-specific eukaryotic elongation factor (EEFSEC) variants cause selenoprotein deficiency, leading to progressive neurodegeneration. EEFSEC deficiency, an autosomal recessive disorder, manifests with global developmental delay, progressive spasticity, ataxia, and seizures.

Sex-dependent modulation of T and NK cells and gut microbiome by low sodium diet in patients with primary aldosteronism.

Background: High dietary sodium intake is a major cardiovascular risk factor and adversely affects blood pressure control. Patients with primary aldosteronism (PA) are at increased cardiovascular risk, even after medical treatment, and high dietary sodium intake is common in these patients. Here, we analyze the impact of a moderate dietary sodium restriction on microbiome composition and immunophenotype in patients with PA.

Hierarchical chirality observed from chiral supramolecular assembling of racemic and enantiopure helicene derivatives on silica nanohelix surfaces.

Cast films of racemic helicene derivatives adsorbed onto the surface of nanometric silica helices with controlled handedness exhibited distinct CD signals, whereas no CD signal was observed in the absence of silica nanohelices. These CD signals originate from the helical supramolecular assemblies formed by the racemic mixture of helicenes, with no evidence of enantiospecific adsorption. Interestingly, when enantiomerically pure forms of these helicenes were drop-cast onto the silica helices, different CD spectra were observed depending on the combination of the helicenes' handedness with that of the silica nanohelices.

Polyethyleneimine-Based Cryogels Enabling the Selective and Reversible Adsorption of Chlorine.

Herein hyperbranched polyethyleneimine (hPEI) cryogels are reported for the selective and reversible adsorption of elemental chlorine. The cryogels are prepared in an aqueous solution by crosslinking with glutaraldehyde at subzero temperatures. The final macroporous composites bearing ammonium chloride groups are obtained after freeze-drying.

International consensus on research priorities in hypospadias using a Delphi study approach.

Introduction: Hypospadias is a common congenital anomaly of the male genitalia that poses significant management and treatment challenges. Gaining a comprehensive understanding of priority research questions in hypospadiology will be essential to reach agreement on the optimal approach to assessment, treatment, and outcome prediction for affected patients.

Methods: We employed a consensus-building Delphi method to identify and prioritize research questions in the hypospadias field.

Role of Odor Novelty on Olfactory Issues in Autism Spectrum Disorder.

Sensory processing abnormalities are a hallmark of autism spectrum disorder (ASD) and are included in its diagnostic criteria. Among these challenges, food neophobia has garnered attention due to its prevalence and potential impact on nutritional intake and health outcomes. This review describes the correlation between novel odor perception and feeding difficulties within the context of ASD.

Exploring six successful nurse-led transition clinics: Experiences and outcomes.

Background And Purpose: In the Netherlands, the 2022 Quality Standard 'Youth in transition from paediatric to adult care' underscores the importance of structured transitional care for young adults with chronic health conditions. Despite this emphasis, detailed knowledge about transition programs and their successful elements remains sparse. This study aims to bridge this gap by exploring nurse-led transition clinics that had successfully implemented core interventions such as a transition coordinator, warm handover, and individual transition plans.

Optimizing and Sustaining Clinical Outcomes in 88 US Hospitals Post-Pandemic: A Quality Improvement Initiative.

Background: Optimizing outcomes of hospitalized patients anchors on standardizing processes in medical management, interventions to reduce the risk of decompensation, and prompt intervention when a patient decompensates.

Methods: A quality improvement initiative (optimized sepsis and respiratory compromise management, reducing health care-associated infection and medication risk, swift management of the deteriorating patient, feedback on performance, and accountability) was implemented in a multistate health system. The primary outcome was risk-adjusted in-hospital mortality.

Hymenoptera venom allergy in children.

From a taxonomic point of view, Hymenoptera are subclassified into families: Apidae, including honeybees (Apis mellifera) and bumblebees (Bombus), and Vespidae, which, in turn, are divided into the subfamilies of Vespinae (wasps, including hornets, vespules, dolichovespules) and Polistinae (paper wasp). Hypersensitivity to Hymenoptera venom can be linked to immunological (IgE-mediated or non-IgE-mediated) and non-immunological mechanisms. Reactions are classified into local reactions, large local reactions, systemic reactions, toxic reactions, and unusual reactions.

QTL mapping provides new insights into emamectin benzoate resistance in salmon lice, Lepeophtheirus salmonis.

Background: The salmon louse (Lepeophtheirus salmonis) is a parasite of wild and farmed salmonid fish, causing huge economic damage to the commercial farming of Atlantic salmon (Salmo salar) in the northern hemisphere. The avermectin emamectin benzoate (EMB) is widely used for salmon delousing. While resistance to EMB is widespread in Atlantic populations of L.