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.
International consensus guidelines on the implementation and monitoring of vosoritide therapy in individuals with achondroplasia
Achondroplasia is the most common genetic form of short-limbed skeletal dysplasia (dwarfism). Clinical manifestations and complications can affect individuals across the lifespan, including the need for adaptations for activities of daily living, which can affect quality of life. Current international guidelines focus on symptomatic management, with little discussion regarding potential medication, as therapeutic options were limited at the time of their publication. Vosoritide is the first pharmacological, precision treatment for achondroplasia; it was approved for use in 2021, creating a need for vosoritide treatment guidelines to support clinicians. An international collaborative of leading experts and patient advocates was formed to develop this Consensus Statement. The group developed the guideline scope and topics during a hybrid meeting in November 2023; guideline statements were subsequently ratified via Delphi methodology using a predefined consensus threshold. These statements provide recommendations across the treatment pathway, from starting treatment with vosoritide through ongoing monitoring and evaluation, to stopping vosoritide and ongoing monitoring following cessation. These guidelines recommend a minimum set of requirements and a practical framework for professionals and health services worldwide regarding the use of vosoritide to treat infants, children and young people with achondroplasia. This Consensus Statement is a supplement to already established consensus guidelines for management and care of individuals with achondroplasia.
Fasting appetite-related gut hormone responses after weight loss induced by calorie restriction, exercise, or both in people with overweight or obesity: a meta‐analysis
Altered appetite-related gut hormone concentrations may reflect a physiological adaptation facilitating weight regain after weight loss. This review investigates hormonal changes after weight loss achieved through calorie restriction (CR), exercise (EX), or both combined (CREX).
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.
Who drives weight stigma? A multinational exploration of clustering characteristics behind weight bias against preconception, pregnant, and postpartum women
Weight bias is a global health challenge and community members are endorsed as the most common source of weight bias. The nature of weight biases specifically against preconception, pregnant, and postpartum (PPP) women from the perspective of community members is not known, especially in terms of cross-cultural trends. We investigated the magnitude of explicit and implicit weight bias and profiles of characteristics associated with harbouring weight bias.
Responses