The protean presentations of XK disease (McLeod syndrome): a case series with new observations and updates on previously reported families.

XK disease is a very rare, multi-system disease, which can present with a wide spectrum of symptoms. This disorder can also be identified pre-symptomatically with the incidental detection of serological abnormalities when typing erythrocytes in peripheral blood, or on other routine laboratory testing. Increasing awareness of this disorder and improved access to genetic testing are resulting in increasing identification of affected patients and families.

Perceived exertion can be lower when exercising in field versus indoors.

Purpose: Studies indicate that the rated perceived exertion (RPE) during physical exercise can be lower in field environments than indoors. The environmental conditions of those studies are explored. Furthermore, we study if the same phenomenon is valid when cycling indoors versus in cycle commuting environments with high levels of stimuli from both traffic and suburban-urban elements.

Tumour-induced osteomalacia: the long road to diagnosis and recovery.

Tumour-induced osteomalacia is caused by tumorous production of fibroblast growth factor 23 (FGF23) leading to urinary phosphate wasting, hypophosphataemia and decreased vitamin D activation. The resulting osteomalacia presents with muscle weakness and bone pain but progresses to multiple pathological fractures. Patients often remain undiagnosed for years with severe physical, psychological and economic ramifications.

More positive patient-reported outcomes in patients newly diagnosed with atrial fibrillation: a comparative longitudinal study.

Aims: To compare patient-reported outcomes (PROs) in patients newly (<6 months) diagnosed with atrial fibrillation (AF) with those who have had a longer diagnosis (≥6 months) and to investigate whether or not these outcomes change over a 6-month period.

Methods And Results: In this longitudinal survey study, 129 patients with AF completed the Revised Illness Perception Questionnaire, the Arrhythmia-Specific questionnaire in Tachycardia and Arrhythmia, and the Hospital Anxiety and Depression Scale at baseline and after 6 months. At baseline, patients newly diagnosed with AF (n = 53), compared with patients with a previous diagnosis (n = 76), reported AF as more temporary (P = 0.

Increased Rates of Gold and Acrylate Allergy in Individuals with Fibromyalgia Tested with an Extended Dental Patch Test Series.

Fibromyalgia is a common chronic pain condition. Rates of contact allergy in individuals with fibromyalgia have not been widely studied. Systemic contact allergy can present with muscle and joint pain and general malaise.

Enhanced protein synthesis is a defining requirement for neonatal B cell development.

The LIN28B RNA binding protein exhibits an ontogenically restricted expression pattern and is a key molecular regulator of fetal and neonatal B lymphopoiesis. It enhances the positive selection of CD5+ immature B cells early in life through amplifying the CD19/PI3K/c-MYC pathway and is sufficient to reinitiate self-reactive B-1a cell output when ectopically expressed in the adult. In this study, interactome analysis in primary B cell precursors showed direct binding by LIN28B to numerous ribosomal protein transcripts, consistent with a regulatory role in cellular protein synthesis.

Illness Perceptions and Health-Related Quality of Life in Women and Men With Atrial Fibrillation.

Background: Health-related quality of life (HRQoL) is worse in patients with atrial fibrillation (AF) compared with other populations. Factors affecting HRQoL in patients with AF are not fully clarified. Illness perceptions are important determinants of disease management and may affect HRQoL.

Pedestrians' Perceptions of Motorized Traffic Variables in Relation to Appraisals of Urban Route Environments.

It is important to examine how motorized traffic variables affect pedestrians along a gradient from rural to inner urban settings. Relations between pedestrians' perceptions of four traffic variables and appraisals of route environments as hindering-stimulating for walking as well as unsafe-safe for reasons of traffic, were therefore studied in the inner urban area of Stockholm, Sweden ( = 294). The pedestrians rated their perceptions and appraisals with the Active Commuting Route Environment Scale (ACRES).

Perspectives on exercise intensity, volume, step characteristics and health outcomes in walking for transport.

Background: Quantification of movement intensity and energy utilization, together with frequency of trips, duration, distance, step counts and cadence, is essential for interpreting the character of habitual walking for transport, and its potential support of health. The purpose of the study is to illuminate this with valid methods and novel perspectives, and to thereby provide a new basis for characterizing and interpreting walking in relation to health outcomes.

Methods: Habitual middle-aged commuting pedestrians (males = 10, females = 10) were investigated in the laboratory at rest and with maximal treadmill and cycle ergometer tests.

Experiences of living with symptomatic atrial fibrillation.

Aim: To explore the experiences of living with symptomatic atrial fibrillation.

Design: This study, with a descriptive qualitative design, was performed using semi-structured individual interviews.

Method: Six women and nine men with symptomatic atrial fibrillation were included.

Increased rates of fragrance allergy in fibromyalgia individuals tested with the Swedish baseline patch test series.

Background: Contact allergy can manifest in a variety of ways clinically. Systemic contact allergy may occasionally present with pain in muscles and joints. Fibromyalgia is a chronic rheumatic disease characterized by pain and with virtually unknown aetiology.

Invasiveness risks of naked goby, Gobiosoma bosc, to North Sea transitional waters.

In recent decades, gobies have dispersed or introduced from the Ponto-Caspian region of eastern Europe in a westerly direction to North American and western European waters. By contrast, the naked goby, Gobiosoma bosc, is the only known gobiid species to have been introduced in an easterly direction from North American to western Europe. The potential invasiveness of G.

A new theoretical performance landscape for suction feeding reveals adaptive kinematics in a natural population of reef damselfish.

Understanding how organismal traits determine performance and, ultimately, fitness is a fundamental goal of evolutionary eco-morphology. However, multiple traits can interact in non-linear and context-dependent ways to affect performance, hindering efforts to place natural populations with respect to performance peaks or valleys. Here, we used an established mechanistic model of suction-feeding performance (SIFF) derived from hydrodynamic principles to estimate a theoretical performance landscape for zooplankton prey capture.

Interchangeability and optimization of heart rate methods for estimating oxygen uptake in ergometer cycling, level treadmill walking and running.

Background: The heart rate (HR) method enables estimating oxygen uptake (V̇O) in physical activities. However, there is a paucity in knowledge about the interchangeability of this method when applied to cycling, walking and running. Furthermore, with the aim of optimization, there is a need to compare different models for establishing HR-V̇O relationships.

Trophic guilds of suction-feeding fishes are distinguished by their characteristic hydrodynamics of swimming and feeding.

Suction-feeding in fishes is a ubiquitous form of prey capture whose outcome depends both on the movements of the predator and the prey, and on the dynamics of the surrounding fluid, which exerts forces on the two organisms. The inherent complexity of suction-feeding has challenged previous efforts to understand how the feeding strikes are modified when species evolve to feed on different prey types. Here, we use the concept of dynamic similarity, commonly applied to understanding the mechanisms of swimming, flying, walking and aquatic feeding.

Heart Rate Methods Can Be Valid for Estimating Intensity Spectrums of Oxygen Uptake in Field Exercise.

Purpose: Quantifying intensities of physical activities through measuring oxygen uptake (V̇O) is of importance for understanding the relation between human movement, health and performance. This can in principle be estimated by the heart rate (HR) method, based on the linear relationship between HR and V̇O established in the laboratory. It needs, however, to be explored whether HR methods, based on HR-V̇O relationships determined in the laboratory, are valid for estimating spectrums of V̇O in field exercise.

A global-scale screening of non-native aquatic organisms to identify potentially invasive species under current and future climate conditions.

The threat posed by invasive non-native species worldwide requires a global approach to identify which introduced species are likely to pose an elevated risk of impact to native species and ecosystems. To inform policy, stakeholders and management decisions on global threats to aquatic ecosystems, 195 assessors representing 120 risk assessment areas across all six inhabited continents screened 819 non-native species from 15 groups of aquatic organisms (freshwater, brackish, marine plants and animals) using the Aquatic Species Invasiveness Screening Kit. This multi-lingual decision-support tool for the risk screening of aquatic organisms provides assessors with risk scores for a species under current and future climate change conditions that, following a statistically based calibration, permits the accurate classification of species into high-, medium- and low-risk categories under current and predicted climate conditions.

Treated with preventive anticoagulation therapy in atrial fibrillation: The patients' perspective.

Aim: The aim of this study was to explore patients' experiences of preventive anticoagulation therapy in atrial fibrillation.

Design: This was a descriptive qualitative study based on interviews.

Methods: Individual interviews with 15 patients, 6 women and 9 men, treated with preventive oral anticoagulant due to atrial fibrillation, were conducted.

Successful tyrosine kinase inhibitor discontinuation outside clinical trials - data from the population-based Swedish chronic myeloid leukaemia registry.

Clinical trials show that tyrosine kinase inhibitor (TKI) treatment can be discontinued in selected patients with chronic myeloid leukaemia (CML). Although updated CML guidelines support such procedure in clinical routine, data on TKI stopping outside clinical trials are limited. In this retrospective study utilising the Swedish CML registry, we examined TKI discontinuation in a population-based setting.

A Pilot Study of the Feasibility and Effects of Table Tennis Training in Parkinson Disease.

Objective: To investigate feasibility and effects of table tennis training on balance control and physical function in individuals with Parkinson disease.

Design: Single group, observational, before-after trial.

Setting: Table tennis training in a gymnasium.

Are heart rate methods based on ergometer cycling and level treadmill walking interchangeable?

Introduction: The heart rate (HR) method is a promising approach for evaluating oxygen uptake ([Formula: see text]), energy demands and exercise intensities in different forms of physical activities. It would be valuable if the HR method, established on ergometer cycling, is interchangeable with other regular activities, such as level walking. This study therefore aimed to examine the interchangeability of the HR method when estimating [Formula: see text] for ergometer cycling and level treadmill walking in submaximal conditions.

Hydrodynamic Simulations of the Performance Landscape for Suction-Feeding Fishes Reveal Multiple Peaks for Different Prey Types.

The complex interplay between form and function forms the basis for generating and maintaining organismal diversity. Fishes that rely on suction-feeding for prey capture exhibit remarkable phenotypic and trophic diversity. Yet the relationships between fish phenotypes and feeding performance on different prey types are unclear, partly because the morphological, biomechanical, and hydrodynamic mechanisms that underlie suction-feeding are complex.

Lin28b controls a neonatal to adult switch in B cell positive selection.

The ability of B-1 cells to become positively selected into the mature B cell pool, despite being weakly self-reactive, has puzzled the field since its initial discovery. Here, we explore changes in B cell positive selection as a function of developmental time by exploiting a link between CD5 surface levels and the natural occurrence of self-reactive B cell receptors (BCRs) in BCR wild-type mice. We show that the heterochronic RNA binding protein Lin28b potentiates a neonatal mode of B cell selection characterized by enhanced overall positive selection in general and the developmental progression of CD5 immature B cells in particular.