Multifocal osteoclast-rich tumour in Paget bone disease and conventional giant cell tumour, two genetically distinct entities? Sequencing from a single case.

Paget disease of bone is a metabolic disorder with a strong genetic component, characterised by pronounced disorganised bone remodelling. Complications of this disease include an increased risk of developing bone neoplasms. Here, we describe the case of a 60-year-old Italian patient with Paget disease of bone, presenting with an osteoclast-rich tumour.

The natural history of fibrodysplasia ossificans progressiva: A prospective, global 36-month study.

Purpose: We report the first prospective, international, natural history study of the ultra-rare genetic disorder fibrodysplasia ossificans progressiva (FOP). FOP is characterized by painful, recurrent flare-ups, and disabling, cumulative heterotopic ossification (HO) in soft tissues.

Methods: Individuals aged ≤65 years with classical FOP (ACVR1 variant) were assessed at baseline and over 36 months.

Fibrodysplasia Ossificans Progressiva: What Have We Achieved and Where Are We Now? Follow-up to the 2015 Lorentz Workshop.

Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare progressive genetic disease effecting one in a million individuals. During their life, patients with FOP progressively develop bone in the soft tissues resulting in increasing immobility and early death. A mutation in the gene was identified as the causative mutation of FOP in 2006.

Patients with ACVR1 mutations have an increased prevalence of cardiac conduction abnormalities on electrocardiogram in a natural history study of Fibrodysplasia Ossificans Progressiva.

Background: Genetic contributors to cardiac arrhythmias are often found in cardiovascular conduction pathways and ion channel proteins. Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare disease of massive heterotopic ossification caused by a highly recurrent R206H mutation in ACVR1/ALK2. This mutation causes abnormal activation of the bone morphogenetic protein (BMP) pathway in response to Activin A.

Self-reported baseline phenotypes from the International Fibrodysplasia Ossificans Progressiva (FOP) Association Global Registry.

A global, patient-reported registry has been established to characterize the course of disease and track clinical outcomes in patients with fibrodysplasia ossificans progressiva (FOP), an ultra-rare genetic condition of progressive heterotopic ossification (HO) that results in ankylosis of joints and renders most affected individuals immobile by the second decade of life. Here, we present baseline phenotypes on 299 patients (median age 21 years; range 0.1 to 78 years) from 54 countries based on aggregate data from the International FOP Association (IFOPA) Global Registry (the "FOP Registry").

Risk for hip fracture before and after total knee replacement in Sweden.

Unlabelled: We studied the risk for hip fracture before and after total knee replacement (TKR) in the entire population in Sweden. Women and men had a low risk for hip fracture before TKR but an increased risk the first year after TKR.

Purpose: It is known that osteoarthritis is associated with high bone mass.

Increased risk for hip fracture after death of a spouse-further support for bereavement frailty?

Unlabelled: Death of a spouse is associated with poorer physical and mental health. We followed all married individuals, born from 1902 to 1942, during the period from 1987 to 2002, and found that widows and widowers had higher risk for hip fracture, compared with still married women and men.

Introduction: Spousal bereavement can lead to poorer physical and mental health.

Correction to: Natural history of fibrodysplasia ossificans progressiva: cross-sectional analysis of annotated baseline phenotypes.

The original version of this article [1] unfortunately included an error to an author's name. Author Maja Di Rocco was erroneously presented as Maja DiRocco.The correct author name has been included in the author list of this Correction article and is already updated in the original article.

Natural history of fibrodysplasia ossificans progressiva: cross-sectional analysis of annotated baseline phenotypes.

Background: Fibrodysplasia Ossificans Progressiva (FOP; OMIM#135100) is an ultra-rare, severely disabling genetic disease characterized by congenital malformation of the great toes and progressive heterotopic ossification (HO) in muscles, tendons, ligaments, fascia, and aponeuroses often preceded by painful, recurrent soft tissue swelling (flare-ups). The formation of HO leads to progressive disability, severe functional limitations in joint mobility, and to a shortened life-span. In this prospective natural history study, we describe the baseline, cross-sectional disease phenotype of 114 individuals with FOP.

Nutrition and physical activity in the prevention and treatment of sarcopenia: systematic review.

Unlabelled: This systematic review summarizes the effect of combined exercise and nutrition intervention on muscle mass and muscle function. A total of 37 RCTs were identified. Results indicate that physical exercise has a positive impact on muscle mass and muscle function in subjects aged 65 years and older.

Increased risk of hip fracture among spouses-evidence of a homogamy effect.

Unlabelled: Spouses tend to share habits and therefore have an increased risk of same diseases. We followed all married couples in Sweden, born 1902 to 1942, in hospital records from 1987 to 2002, and found that individuals whose spouse had a hip fracture had an increased risk of hip fracture.

Introduction: The purpose of this study was to determine whether spouses of hip fracture patients have an elevated risk of hip fracture.

The effect of prior bisphosphonate therapy on the subsequent therapeutic effects of strontium ranelate over 2 years.

Unlabelled: Many osteoporotic women prescribed strontium ranelate have previously received bisphosphonates. Prior bisphosphonate use blunted the spinal bone mineral density (BMD) response for 6 months. Hip BMD was blunted to a degree for 2 years, although there was an overall increase in hip BMD in contrast to the heel where BMD did not increase.

The effect of prior bisphosphonate therapy on the subsequent BMD and bone turnover response to strontium ranelate.

Strontium ranelate is an effective treatment for osteoporosis in treatment-naive women. In the United Kingdom, bisphosphonates are often used first line. Prior bisphosphonate use may blunt the bone mineral density (BMD) response to strontium ranelate by reducing strontium uptake into the bone.

Which women should be selected for vertebral fracture assessment? Comparing different methods of targeting VFA.

Vertebral fracture assessment (VFA) is a potential screening tool for vertebral fractures, but it is uncertain how to optimize the selection of women for VFA. We investigate the use of a probability score (VFscore) to select women for VFA screening and compare this to other means of targeting VFA. We identified 1,572 treatment-naive women over age 65 who had undergone routine VFA screening.

Reprint of The nature and utility of the phytoestrogens: a review of the evidence.

Non-prescription remedies are becoming increasingly popular particularly amongst postmenopausal who in this market are the largest consumers. Phytoestrogens are a large family of plant derived molecules possessing various degrees oestrogen like activity. Food or food supplements containing phytoestrogen are often been advocated as an alternative to hormonal replacement therapy (HRT) in women with contraindications to the use of conventional oestrogen replacement, or simply wanting a more 'natural' alternatives.

Osteoporosis and osteoporotic fracture occurrence and prevention in the elderly: a geriatric perspective.

Age is a major determinant of osteoporosis, but the elderly are rarely assessed and often remain untreated for this condition. Falls, co-morbidities and co-medications compound the risk of fracture in senile osteoporosis. The prevalence of osteoporosis is expected to increase with increasing life expectancy, and the associated fractures - particularly hip fractures - will lead to significant demands on health resources.

The Alara Metriscan phalangeal densitometer: evaluation and triage thresholds.

The Metriscan (Alara Inc, CA) is a compact digital radiographic absorptiometry device capable of determining phalangeal bone mineral density in arbitrary units (BMD(au)) from the second phalanges of the middle three digits. We have examined the utility and the in vitro and in vivo performances of the Metriscan, and established triage thresholds based on the UK's National Osteoporosis Society guidelines on peripheral densitometry. 170 white female participants (70 osteoporotic and 100 non-osteoporotic at the hip or spine) aged between 55 years and 70 years were recruited from patients attending for routine dual X-ray absorptiometry (DXA) examination.

Generation of a 3D proximal femur shape from a single projection 2D radiographic image.

Unlabelled: Generalized Procrustes analysis and thin plate splines were employed to create an average 3D shape template of the proximal femur that was warped to the size and shape of a single 2D radiographic image of a subject. Mean absolute depth errors are comparable with previous approaches utilising multiple 2D input projections.

Introduction: Several approaches have been adopted to derive volumetric density (g cm(-3)) from a conventional 2D representation of areal bone mineral density (BMD, g cm(-2)).

The safety and efficacy of vertebroplasty using Cortoss cement in a newly established vertebroplasty service.

Vertebroplasty provides an effective means of treating painful vertebral lesions although the majority of the literature relates to vertebroplasty using PMMA cement. The purpose of this study is to assess the safety and efficacy of vertebroplasty using Cortoss, a recently developed bis-GMA resin. Our newly established vertebroplasty service exclusively uses Cortoss cement and has a patient database which is updated on a regular basis using the medical records.

Routine versus targeted vertebral fracture assessment for the detection of vertebral fractures.

Unlabelled: Fracture risk is underestimated in women with unknown vertebral fractures. Using VFA, we compared two screening methods: targeted (6,388 women) and routine (2,176 women). Routine screening detected fractures in 20%.

The effect of prior bisphosphonate exposure on the treatment response to teriparatide in clinical practice.

Our objective was to determine the effect of prior bisphosphonate exposure on the treatment response to teriparatide. All patients started on teriparatide in our hospital are entered into a database. All patients who had at least 12 months' treatment were identified.