Clinical spectrum and uncommon features of McCune-Albright syndrome in children: a cohort study from a National Referral Center.

Introduction: McCune-Albright syndrome (MAS) is a rare disease caused by somatic gain-of-function variants in the gene that lead to constitutive activation of the G protein alpha subunit (Gsα). Pathologic consequences can involve several tissues. Fibrous dysplasia (FD), café-au-lait skin macules and hyperfunctioning endocrinopathies are classic manifestations.

CADOR (Capsaicin in neuropathic-like pain in digital osteoarthritis) study protocol: a multicentre randomised parallel-group trial.

Introduction: It is common for finger pain in hand osteoarthritis (HOA) to display a neuropathic component. Non-steroidal anti-inflammatory drugs (NSAIDs) and conventional analgesics are not very effective in relieving this neuropathic-like pain. Capsaicin, a compound extracted from chilli peppers, is approved for the management of localised neuropathic pain.

Daily or intermittent vitamin D supplementation in patients with or at risk of osteoporosis: Position statement from the GRIO.

Advantages and disadvantages of intermittent versus daily vitamin D supplementation especially in adults with or at risk of osteoporosis are discussed by the Osteoporosis Research and Information Group (GRIO). The analysis of the literature suggests that intermittent long-term high doses vitamin D supplementation (such as 60,000IU/month or more), may increase the risk of falls, fracture and premature death in certain populations, while daily doses of 800-1000IU with calcium decrease falls and non-vertebral fractures in the elderly with vitamin D deficiency. In patients with or at risk of osteoporosis we hence recommend measuring the 25(OH)D concentration prior to supplementation and to provide vitamin D supplementation (with optimization of calcium intake if needed) to obtain a concentration between 30 and 60ng/mL.

Rheumatoid arthritis and subsequent fracture risk: an individual person meta-analysis to update FRAX.

Unlabelled: The relationship between rheumatoid arthritis (RA) and fracture risk was estimated in an international meta-analysis of individual-level data from 29 prospective cohorts. RA was associated with an increased fracture risk in men and women, and these data will be used to update FRAX®.

Introduction: RA is a well-documented risk factor for subsequent fracture that is incorporated into the FRAX algorithm.

Expert consensus recommendations for the diagnosis and treatment of chronic non-bacterial osteitis (CNO) in adults.

Background: There is considerable practice variation in labelling, diagnosis and treatment of adults with sterile bone inflammation. We developed a expert consensus recommendations on the disease definition, diagnosis and treatment of this rare condition.

Methods: Systematic literature review and Grading of Recommendations, Assessment, Development and Evaluations-based appraisal of evidence, two Delphi surveys and three digital and in-person consensus meetings with a multidisciplinary expert panel and patient representatives.

Exploring the general practitioners' perception of the inter-professional care of rheumatoid arthritis patients (GEPRA-II): a qualitative interview study.

Background: A lack of medication adherence among patients with rheumatoid arthritis (RA) has been reported. Inter-professional collaborations seem essential for an optimal therapeutic management of patients. The aim of this study was to analyse the barriers and facilitators of general practitioners (GPs) for the implementation of collaborative support programmes in RA.

Improvement of bone properties in children with osteogenesis imperfecta after pamidronate: a bone biopsy study.

OI, or bone brittle disease, is characterized by increased mineralization of bone matrix independently of clinical severity. So, a beneficial effect of antiresorptive treatments such as bisphosphonates (BP) is questionable. We aim to compare the bone matrix characteristics before and after BP pamidronate (PAM).

Interstitial Lung Disease Associated with Anti-Ku Antibodies: A Case Series of 19 Patients.

Antibodies against Ku have been described in patients with various connective tissue diseases. The objective of this study was to describe the clinical, functional, and imaging characteristics of interstitial lung disease in patients with anti-Ku antibodies. : This single-center, retrospective observational study was conducted at a tertiary referral institution.

A Qualitative Approach to Quality of Life in Fibrous Bone Dysplasia /McCune Albright Syndrome: Looking Beyond Quantitative Analysis.

This study explores FD/MAS patient's perceptions about their disease and its impact on their quality of life. We have evaluated quality of life (QoL) in French Fibrous Dysplasia/MacCune-Albright Syndrome (FD/MAS) patients using a qualitative approach with focus groups to explore perceptions, symptoms and limitations associated with FD/MAS and a quantitative method with the Short Form-36 (SF36) to quantify QoL. Focus groups revealed the heterogeneity of FD forms and allowed for understanding the reasons of reduced QoL.

Bone strain index for fracture risk assessment in older men-the prospective STRAMBO study.

Objective: Bone strain index (BSI) is a noninvasive index of bone strength assessed on lumbar spine and femur dual energy X-ray absorptiometry scans using the finite-element method. In this study, we assess the link of the lumbar spine and hip BSI with fracture risk in older men.

Design: Prospective cohort study.

The Fracture Phenotypes in Women and Men of 50 Years and Older with a Recent Clinical Fracture.

Purpose Of Review: We review the literature about patients 50 years and older with a recent clinical fracture for the presence of skeletal and extra-skeletal risks, their perspectives of imminent subsequent fracture, falls, mortality, and other risks, and on the role of the fracture liaison service (FLS) for timely secondary fracture prevention.

Recent Findings: Patients with a recent clinical fracture present with heterogeneous patterns of bone-, fall-, and comorbidity-related risks. Short-term perspectives include bone loss, increased risk of fractures, falls, and mortality, and a decrease in physical performance and quality of life.

Deciphering the circulating microRNA signature of hemophilic arthropathy.

Background: Haemophilic arthropathy (HArt) is a serious complication in patients with hemophilia. Early diagnosis and treatment are essential to minimise the development of HArt. The use of biomarkers may improve early diagnosis of HArt.

A quick access to information on influenza burden and prevention in Lyon university hospital: A prospective QR code-based information campaign in 2022-2023.

Background: Influenza vaccines are effective in decreasing hospitalizations and mortality related to influenza and its complications. However, the Vaccine Coverage Rate of influenza remains low and multifaceted efforts are required to improve it. The aim of this study was to assess the impact on influenza vaccine perception using a digital tool among outpatients and health care workers (HCWs).

Evaluation of romosozumab's effects on bone marrow adiposity in postmenopausal osteoporotic women: results from the FRAME bone biopsy sub-study.

Romosozumab, a humanized monoclonal antibody that binds and inhibits sclerostin, produces a marked increase in bone formation with a concomitant decreased bone resorption. This transient rise in bone formation in the first 2 months of treatment is mainly due to an increased modeling-based bone formation. This requires the recruitment and differentiation of osteoblasts, one possibility being a preferential switch in commitment of precursors to osteoblasts over adipocytes.

Evaluation of circulating microRNA signature in patients with erosive hand osteoarthritis: The HOAmiR study.

Objectives: To identify circulating micro-RNAs differentially expressed in patients with erosive hand osteoarthritis (HOA) compared to patients with non-erosive HOA and patients without HOA.

Methods: In the screening phase, 768 well-characterized micro-RNAs using Taqman low-density array cards were measured in 30 sera from 10 patients with erosive HOA, 10 patients with non-erosive HOA, and 10 controls without HOA, matched for age and body mass index (BMI). In a second step, we validated the micro-RNAs identified at the screening phase (adjusted p value < 0.

Data collection on rare bone and mineral conditions in Europe: The landscape of registries and databases.

Background: knowledge on the natural history of rare diseases is necessary to improve outcomes. Disease registries may play a key role in covering these unmet needs in the rare bone and mineral community.

Objective: to map existing bone and mineral conditions registries in Europe and their characteristics.

Reconstruction of remodeling units reveals positive effects after 2 and 12 months of romosozumab treatment.

Romosozumab treatment results in a transient early increase in bone formation and sustained decrease in bone resorption. Histomorphometric analyses revealed that the primary bone-forming effect of romosozumab is a transient early stimulation of modeling-based bone formation on cancellous and endocortical surfaces. Furthermore, preclinical studies have demonstrated that romosozumab may affect changes in the remodeling unit, resulting in positive bone balance.

Rapid bone microarchitecture decline in older men with high bone turnover-the prospective STRAMBO study.

Older men with high bone turnover have faster bone loss. We assessed the link between the baseline levels of bone turnover markers (BTMs) and the prospectively assessed bone microarchitecture decline in men. In 825 men aged 60-87 yr, we measured the serum osteocalcin (OC), bone alkaline phosphatase (BAP), N-terminal propeptide of type I procollagen (PINP), and C-terminal telopeptide of type I collagen (CTX-I), and urinary total deoxypyridinoline (tDPD).