Bone Abstracts

Low bone mineral density (BMD) and an increased rate of both peripheral and vertebral fractures have been observed in patients with Duchenne muscular dystrophy (DMD), but studies on bone metabolic alterations in this disease are still very few.We are now presenting the preliminary findings of an ongoing multicenter, prospective study aimed to identify the characteristics of DMD boys carrying a higher risk of bone loss and fractures, through the evaluatio...

We analyzed body composition (DXA, Hologic) in 334 young patients (aged 3–24 years) with chronic diseases, most of them on long-term glucocorticoid (GC) treatment, and monitored its changes over 3–14 years (6.9±6.2 years).Bone mineral content (BMC), fat mass (FM) and fat-free mass (FFM) were measured on total body (TB), trunk, upper limbs, lower limbs. BMC, FM, FFM were expressed as percentages and compared with age- and sex-matched health...

Introduction: The clinical diagnosis of mild forms of hypophosphatasia [HPP], a rare genetic bone disease, is often made in adulthood, on the basis of persistently low serum levels of alkaline phosphatase [ALP], often coupled with signs of poor bone/teeth mineralization.Case report: A 50-year-old male on treatment with vitamin D supplementation because of osteoporosis of lumbar spine (T-score –3.2) and femoral neck (T-score –2.4), was referred ...

Objectives: Low bone mineral density (BMD) and increased frequency of peripheral and vertebral fractures have been reported in boys with Duchenne muscular dystrophy (DMD), but studies on the determinants of low BMD are still very few. We are currently carrying out a multicenter, prospective study aimed to identify the characteristics of DMD boys with a higher risk of bone loss and fractures.Methods: Forty-two DMD boys (mean age 9.9±3.3 years) underw...

Introduction: Reduced bone mineral density [BMD] and increased fracture risk are common complications in all conditions characterized by severely reduced physical activity and/or requiring long-term glucocorticoid [GC] treatment, including Duchenne Muscular Dystrophy [DMD].Objectives: The RisBo-DMD study (EudraCT 2011-005745-12) is a 24-month prospective multicenter study, aimed at identifying DMD patients at higher risk of fractures and improving the bo...

Nacre, or mother of pearl, is a promising natural biocompatible biomaterial consisting of aragonite (97%) and of organics (3%) and capable to increase the cell osteogenic activity. It has been established that osteoarthritic osteoblasts present a mineralization defect and, to date, only a few molecules (vitamin D3 and bone morphogenetic protein2) could improve the mineralization potential of this cell type. In this context, we evaluated the impact of nacreous molecules on the ...

Nacre is able to induce bone-forming cells mineralization, and gains widespread interest in bone regeneration. While, the osteoinductive compounds are not identified yet. The nacre extract, ethanol soluble matrix (ESM), was proven having the capacity to induce the mineralization of mouse MC3T3-E1 cells and to restore mineralization defect in human osteoarthritic osteoblasts. Being tested on the two cells, ESM could induce the formation of mineralization nodules, identified as ...

Background: Bisphosphonates are increasingly used to treat disorders of low bone mineral density (BMD) in children and adolescents. Long-term bisphosphonate use in adults has been associated with and increased risk of atypical subtrochanteric and diaphyseal femoral fractures (AFFs). To date, bisphosphonate-related AFFs have not been reported in children or adolescents.Presenting problem: A 16-year-old girl presented with a 3-week history of left thigh pa...

Screening gene function in vivo is a powerful approach to discover novel drug targets in the human genome (Nat Rev Drug Discov 2 38–51, 2003). We present data for 3776 distinct gene knockout (KO) mouse lines with viable adult homozygous mice generated using both gene-trapping and homologous recombination technologies. Bone mass was determined from PIXImus DEXA scans of male and female mice at 14 weeks of age and by microCT analyses of bones from ...

Primary hyperparathyroidism is extremely rare in childhood and adolescence. Here we report a clinical case of a 15-year-3-month-old boy who began 2 years before with pain in his knees, genu varum, and fatigue. Physical examination showed severe genu valgum with an inter-malleolar distance of more than 30 cm. Biochemical tests showed hypercalcemia (12.2 mg/dl), hypophosphatemia (2.3 mg/ dl), hypercalciuria (6.4 mg/kg per day), high alkaline phosphatase (2812 IU/l), low 25-hydro...