Bone Abstracts

Objectives: To characterize clinical features and long-term outcome of CRMO, a rare inflammatory bone disease of childhood and adolescence, which forms part of the spectrum of chronic non-bacterial osteomyelitis (CNO).Methods: We studied 33 patients with an established diagnosis of CRMO followed at the Centre for Bone Quality of the Leiden University Medical Center from 1994 to 2018. Demographic and clinical data were collected at presentation and last f...

Peripheral quantitative computed tomography (pQCT) is receiving considerable attention in the diagnosis and monitoring of human bone diseases. It is well accepted that lower image resolution compared to micro-computed tomography (micro-CT) affects bone morphometry. With advances in micro-CT evaluation techniques such as sample-specific remodeling simulations or dynamic bone morphometry, there is the potential to also allow the application of such techniques to clinical pQCT sc...

Interleukin- 34 (IL34) is a new, challenging cytokine discovered in 2008. It promotes the proliferation, survival and differentiation of the monocyte/macrophage lineage with almost the same efficiency as the macrophage-colony stimulating factor (M-CSF). IL34 has already been described to play a key role in various musculoskeletal bone diseases such as bone giant cell tumors or in rheumatoid arthritis. These ‘twin’ cytokines share a lot of functional similarities whic...

Background: Osteogenesis imperfecta (OI) is a rare disease characterized by increased fracture rate and bone deformities. Patients are classified by phenotype and most are affected by mutations in COL1A1/2.Patients with OI type V present with specific clinical symptoms including hyperplastic callus formation, only mildly decreased height, metaphyseal lines and a calcification of the membrane interossea of the forearm. The disease causing mutation for OI ...

Osteoclasts are the major bone resorbing cells, essential for bone turnover and development. Human osteoclasts can be generated in vitro by stimulation of peripheral blood mononuclear cells (PBMCs) with M-CSF and RANK-L. Seeding fully differentiated osteoclasts onto mineralized surfaces facilitates the analysis of molecular interactions between the osteoclast and the mineralized matrix.Currently, a widely used protocol for harvesting osteoclasts...

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Low bone mineral density (BMD) is an important and modifiable risk factor for fracture in postmenopausal women with osteoporosis. Denosumab (DMAb) shows a stronger relationship between BMD increases and antifracture efficacy than oral bisphosphonate (BP) therapies. Subjects who remain at higher risk of fracture despite current BP therapy need treatment. In two studies, DMAb significantly increased BMD and decreased bone turnover markers vs a BP (ibandronate (IBN) or risedronat...

Objectives: Low bone mass in adolescent idiopathic scoliosis (AIS) has been well reported, however the etiologies of the disease and this abnormal phenotype were still unknown. Leptin have profound effects on bone metabolism and skeletal growth, and was speculated to play a role in the etiopathogenesis of AIS. The objective of this study was to investigate the bone quality in AIS and its association with leptin and soluble leptin receptor (sOB-R).Methods...

Objectives: High resolution peripheral quantitative computed tomography (HR-pQCT) was used to evaluate bone quality and volumetric bone mineral density (vBMD). However, manufacturers only provide a standard protocol for adult but not for growing subjects. The aim of this study was to investigate the difference on measurement between two different HR-pQCT scanning protocols in adolescence with unfused growth plate.Methods: 27 boys and 26 girls aged 13&#15...

Bone has recently emerged as a novel endocrine organ regulating glucose metabolism. Ectonucleotide pyrophosphatase/phosphodiesterase-1 (NPP1) controls bone mineralisation by generating the mineralisation inhibitor pyrophosphate. In clinical studies increased activity of NPP1 has been found in patients with insulin resistance, and it has been shown to directly inhibit the insulin receptor. We hypothesised that mice lacking NPP1 (Enpp1−/−) would exhibit im...