Muscle Contractility in Hypokalemic Periodic Paralysis.

Introduction/aims: Primary hypokalemic periodic paralysis (HypoPP) can present with periodic paralysis and/or permanent muscle weakness. Permanent weakness is accompanied by fat replacement of the muscle. It is unknown whether the permanent muscle weakness is solely due to fat replacement or if other factors affect the ability of the remaining muscle fibers to contract.

Surface-Enhanced Raman Spectroscopy on Organic Mixed Ionic-Electronic Conductors: Tracking Dynamic Doping in Light-Emitting Electrochemical Cells.

In the domain of organic mixed ionic-electronic conductors (OMIECs), simultaneous transport and coupling of ionic and electronic charges are crucial for the function of electrochemical devices in organic electronics. Understanding conduction mechanisms and chemical reactions in operational devices is pivotal for performance enhancement and is necessary for the informed and systematic development of more promising materials. Surface-enhanced Raman spectroscopy (SERS) is a potent tool for monitoring electrochemical evolution and dynamic doping in operational devices, offering enhanced sensitivity to subtle spectral changes.

Contractile properties and magnetic resonance imaging-assessed fat replacement of muscles in myotonia congenita.

Background And Purpose: Myotonia congenita (MC) is a muscle channelopathy in which pathogenic variants in a key sarcolemmal chloride channel Gene (CLCN1) cause myotonia. This study used muscle magnetic resonance imaging (MRI) to quantify contractile properties and fat replacement of muscles in a Danish cohort of MC patients.

Methods: Individuals with the Thomsen (dominant) and Becker (recessive) variants of MC were studied.

Impact of Synergy Partner Cel7B on Cel7A Binding Rates: Insights from Single-Molecule Data.

Enzymatic degradation of cellulosic biomass is a well-established route for the sustainable production of biofuels, chemicals, and materials. A strategy employed by nature and industry to achieve an efficient degradation of cellulose is that cellobiohydrolases (or exocellulases), such as Cel7A, work synergistically with endoglucanases, such as Cel7B, to achieve the complete degradation of cellulose. However, a complete mechanistic understanding of this exo-endo synergy is still lacking.

Hypokalemic periodic paralysis: a 3-year follow-up study.

Background And Objectives: Primary hypokalemic periodic paralysis (HypoPP) is an inherited channelopathy most commonly caused by mutations in CACNA1S. HypoPP can present with different phenotypes: periodic paralysis (PP), permanent muscle weakness (PW), and mixed weakness (MW) with both periodic and permanent weakness. Little is known about the natural history of HypoPP.

Reconstitution and post-thaw storage of cryopreserved human mesenchymal stromal cells: Pitfalls and optimizations for clinically compatible formulants.

Introduction: The regenerative and immunomodulatory properties of multipotent mesenchymal stromal cells (MSCs) make them an intriguing asset for therapeutic applications. An off-the-shelf approach, using pre-expanded cryopreserved allogenic MSCs, bypasses many practical difficulties of cellular therapy. Reconstitution of a MSC product away from cytotoxic cryoprotectants towards a preferred administration solution might be favorable for several indications.

Quantifying DNA-mediated liposome fusion kinetics with a fluidic trap.

Self-assembly of synthetic lipid vesicles lipid membrane fusion is a versatile tool for creating biomimetic nano- and micron-sized particles. These so-called liposomes are used in the development of biosensing platforms, design of drug delivery schemes, and for investigating protein-mediated fusion of biological membranes. This work demonstrates DNA-induced liposome fusion in a nanofluidic trap where the reaction occurs in a 15 femtoliter volume at homogeneous mixing.

Muscle fat replacement and contractility in patients with skeletal muscle sodium channel disorders.

Skeletal muscle sodium channel disorders give rise to episodic symptoms such as myotonia and/or periodic paralysis. Chronic symptoms with permanent weakness are not considered characteristic of the phenotypes. Muscle fat replacement represents irreversible damage that inevitably will impact on muscle strength.

Carbon Dioxide-Mediated Desalination.

Conventional desalination membrane technologies, although offer portable drinking water, are still energy-intensive processes. This paper proposes a potentially new approach for performing water desalination and purification by utilizing the reversible interaction of carbon dioxide (CO) with nucleophilic amines─reminiscent of the Solvay process. Based on our model studies with small molecules, CO-responsive amphiphilic insoluble diamines were prepared, characterized, and applied in the formation of soda and ammonium chloride upon exposure to ambient CO (1 atm), thus removing chloride ions from model and real seawater.

β-Galactosidase deficiency in the GLB1 spectrum of lysosomal storage disease can present with severe muscle weakness and atrophy.

Deficiency of the enzyme β-galactosidase due to variants in the -gene is associated with metabolic disorders: Morquio B and GM1-gangliosidosis. Here, we report a case compound heterozygous for variants in the -gene and a severe muscular phenotype. Full body T1-w MRI was conducted for muscular involvement.

Resonant Laser Printing of Optical Metasurfaces.

One of the challenges for metasurface research is upscaling. The conventional methods for fabrication of metasurfaces, such as electron-beam or focused ion beam lithography, are not scalable. The use of ultraviolet steppers or nanoimprinting still requires large-size masks or stamps, which are costly and challenging in further handling.

Objective Outcome Measures Continue to Improve from 6 to 12 Months after Conservatively Treated Distal Radius Fractures in the Elderly-A Prospective Evaluation of 50 Patients.

Distal radius fractures (DRF) in the elderly population above 65 years represent 18% of all fractures and are thereby the second most frequent fracture in the elderly. Fracture dislocation and comminution are often used to determine whether non-operative or operative treatment is indicated. The purpose of this prospective case series of minimally displaced DRF treated with a dorsal cast was to assess the complication rate and patient-reported outcome measures.

Label-Free Sensing of Biorecognition on Liposomes.

Nanometer-sized liposomes decorated with macromolecules are increasingly used as drug delivery vehicles due to their long lifetimes and target cell specificity, but surface characterization methods often change their properties, which leads to incorrect results. Ligand binding is commonly applied for characterizing these surface modifications. Here, we use a nanofluidic-based label-free sensor for real-time sensing of ligands binding to liposomes.

Pulmonary vein isolation using Ablation Index improves outcome in patients with atrial fibrillation.

Aims: To evaluate the clinical outcome of pulmonary vein isolation (PVI) in radiofrequency ablation of atrial fibrillation (AF) comparing a strategy using Ablation Index (AI) and lesion contiguity with Contact Force (CF) only.

Methods: In a single-center retrospective design, we included 479 patients with AF (n=341 (71.2%) paroxysmal AF (PAF) and n=138 (28.

Reconstruction of corrupted datasets from ammonium-ISE sensors at WRRFs through merging with daily composite samples.

Long-term, continuous datasets of high quality are important for instrumentation, control, and automation efforts of wastewater resources recovery facility (WRRFs). This study presents a methodology to increase the reliability of measurements from ammonium ion-selective electrodes (ISEs). This is done by correcting corrupted ISE data with a data source that often is available at WRRFs (volume-proportional composite samples).

Size and surface charge characterization of nanoparticles with a salt gradient.

Exosomes are nanometer-sized lipid vesicles present in liquid biopsies and used as biomarkers for several diseases including cancer, Alzheimer's, and central nervous system diseases. Purification and subsequent size and surface characterization are essential to exosome-based diagnostics. Sample purification is, however, time consuming and potentially damaging, and no current method gives the size and zeta potential from a single measurement.

Evaluating the performance of a simple phenomenological model for online forecasting of ammonium concentrations at WWTP inlets.

A simple model for online forecasting of ammonium (NH ) concentrations in sewer systems is proposed. The forecast model utilizes a simple representation of daily NH profiles and the dilution approach combined with information from online NH and flow sensors. The method utilizes an ensemble approach based on past observations to create model prediction bounds.

A protocol for a single-center, single-blinded randomized-controlled trial investigating volar plating versus conservative treatment of unstable distal radius fractures in patients older than 65 years.

Background: Distal radius fractures (DRF) are very common in elderly patients, who present at the Emergency Department. Surgical treatment with open reduction and internal fixation using volar locking plates is widely prevalent despite the lack of evidence proving its superiority to conservative treatment with closed reduction and plaster immobilization. The purpose of this study is to investigate whether conservative treatment is superior to volar plating in terms of number of complications and results in a comparable or superior functional outcome in patients ≥65 years.

Predictive validity of the CriSTAL tool for short-term mortality in older people presenting at Emergency Departments: a prospective study.

Abstract: To determine the validity of the Australian clinical prediction tool Criteria for Screening and Triaging to Appropriate aLternative care (CRISTAL) based on objective clinical criteria to accurately identify risk of death within 3 months of admission among older patients.

Methods: Prospective study of ≥ 65 year-olds presenting at emergency departments in five Australian (Aus) and four Danish (DK) hospitals. Logistic regression analysis was used to model factors for death prediction; Sensitivity, specificity, area under the ROC curve and calibration with bootstrapping techniques were used to describe predictive accuracy.

Single-molecule DNA-mapping and whole-genome sequencing of individual cells.

To elucidate cellular diversity and clonal evolution in tissues and tumors, one must resolve genomic heterogeneity in single cells. To this end, we have developed low-cost, mass-producible micro-/nanofluidic chips for DNA extraction from individual cells. These chips have modules that collect genomic DNA for sequencing or map genomic structure directly, on-chip, with denaturation-renaturation (D-R) optical mapping [Marie R, et al.

Current and Future Treatments for Persistent Pulmonary Hypertension in the Newborn.

Persistent pulmonary hypertension in newborn (PPHN) is a serious and possibly fatal syndrome characterized by sustained foetal elevation of pulmonary vascular resistance at birth. PPHN may manifest secondary to other conditions as meconium aspiration syndrome, infection and congenital diaphragmatic hernia. This MiniReview provides the reader with an overview of current and future treatment options for patients with PPHN without congenital diaphragmatic hernia.

Efficacy of a tool to predict short-term mortality in older people presenting at emergency departments: Protocol for a multi-centre cohort study.

Background: Prognostic uncertainty inhibits clinicians from initiating timely end-of-life discussions and advance care planning. This study evaluates the efficacy of the CriSTAL (Criteria for Screening and Triaging to Appropriate aLternative care) checklist in emergency departments.

Methods: Prospective cohort study of patients aged ≥65 years with any diagnosis admitted via emergency departments in ten hospitals in Australia, Denmark and Ireland.

Designing in situ simulation in the emergency department: evaluating safety attitudes amongst physicians and nurses.

Background: This intervention study aimed to enhance patient safety attitudes through the design of an in situ simulation program based on a needs analysis involving thematic analysis of patient safety data and short-term ethnography. The study took place at an Emergency Department (ED) in the Central Region of Denmark. Research suggests that poor handover communication can increase the likelihood of critical incidents and adverse events in the ED.