Publisher Correction: Clinical practice recommendations for the diagnosis and management of X-linked hypophosphataemia
Related Articles
Clinical practice recommendations for the diagnosis and management of X-linked hypophosphataemia
X-linked hypophosphataemia (XLH) is a rare metabolic bone disorder caused by pathogenic variants in the PHEX gene, which is predominantly expressed in osteoblasts, osteocytes and odontoblasts. XLH is characterized by increased synthesis of the bone-derived phosphaturic hormone fibroblast growth factor 23 (FGF23), which results in renal phosphate wasting with consecutive hypophosphataemia, rickets, osteomalacia, disproportionate short stature, oral manifestations, pseudofractures, craniosynostosis, enthesopathies and osteoarthritis. Patients with XLH should be provided with multidisciplinary care organized by a metabolic bone expert. Historically, these patients were treated with frequent doses of oral phosphate supplements and active vitamin D, which was of limited efficiency and associated with adverse effects. However, the management of XLH has evolved in the past few years owing to the availability of burosumab, a fully humanized monoclonal antibody that neutralizes circulating FGF23. Here, we provide updated clinical practice recommendations for the diagnosis and management of XLH to improve outcomes and quality of life in these patients.
Management practices and manufacturing firm responses to a randomized energy audit
Increasing the efficiency of industrial energy use is widely considered important for mitigating climate change. We randomize assignment of an energy audit intervention aimed at improving energy efficiency and reducing energy expenditures of small- and medium-sized metal processing firms in Shandong Province, China, and examine impacts on energy outcomes and interactions with firms’ management practices. We find that the intervention reduced firms’ unit cost of electricity by 8% on average. Firms with more developed structured management practices showed higher rates of recommendation adoption. However, the post-intervention electricity unit cost reduction is larger in firms with less developed practices, primarily driven by a single recommendation that corrected managers’ inaccurate reporting of transformer usage at baseline, lowering their electricity costs. By closing management-associated gaps in awareness of energy expenditures, energy audit programmes may reduce a firm’s unit cost of energy but have an ambiguous impact on energy use and climate change.
Sustainable supply chain management practices and performance: The moderating effect of stakeholder pressure
Currently, sustainable supply chain management practices have become an important strategy for firms to improve performance and gain competitive advantage. However, there is a current debate over the performance outcomes of sustainable supply chain management practices. Additionally, the role of stakeholder pressure is frequently overlooked. Drawing on Natural Resources-Based View and Stakeholder Theory, this study aims to elucidate the ambiguous connection between sustainable supply management, sustainable process management, stakeholder pressure and performance, and investigate the mediation role of sustainable process management and the moderation effect of stakeholder pressure. Our analysis, based on data collected from 235 Chinese manufacturing firms, reveals significant insights. First, stakeholder pressure positively moderates the relationship between sustainable process management and performance, while negatively moderates the relationship between sustainable supply management and performance. Second, sustainable process management has a complete mediation effect on the relationship between sustainable supply management and performance. The conclusion not only explains the inconsistent relationship between sustainable supply chain management practice and performance, but also reveals clearly the relationship between sustainable supply management and sustainable process management. Besides, it also highlights the difference in performance outcomes of sustainable supply management and sustainable process management under stakeholder pressures, and has valuable guidance to the practice of sustainable supply chain management in Chinese manufacturing firms.
A UK population-based case-control study of blood tests before cancer diagnosis in patients with non-specific abdominal symptoms
Abnormal results in commonly used primary care blood tests could be early markers of cancer in patients presenting with non-specific abdominal symptoms.
Autologous haematopoietic stem cell transplantation for treatment of multiple sclerosis and neuromyelitis optica spectrum disorder — recommendations from ECTRIMS and the EBMT
Autologous haematopoietic stem cell transplantation (AHSCT) is a treatment option for relapsing forms of multiple sclerosis (MS) that are refractory to disease-modifying therapy (DMT). AHSCT after failure of high-efficacy DMT in aggressive forms of relapsing–remitting MS is a generally accepted indication, yet the optimal placement of this approach in the treatment sequence is not universally agreed upon. Uncertainties also remain with respect to other indications, such as in rapidly evolving, severe, treatment-naive MS, progressive MS, and neuromyelitis optica spectrum disorder (NMOSD). Furthermore, treatment and monitoring protocols, rehabilitation and other supportive care before and after AHSCT need to be optimized. To address these issues, we convened a European Committee for Treatment and Research in Multiple Sclerosis Focused Workshop in partnership with the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party, in which evidence and key questions were presented and discussed by experts in these diseases and in AHSCT. Based on the workshop output and subsequent written interactions, this Consensus Statement provides practical guidance and recommendations on the use of AHSCT in MS and NMOSD. Recommendations are based on the available evidence, or on consensus when evidence was insufficient. We summarize the key evidence, report the final recommendations, and identify areas for further research.
Responses