Bone Abstracts

: Congenital limb abnormalities represent a prevalence of 0.79/1000 of live births in Massachusetts1. A better understanding of their physiopathology could improve the management of the patients. We report on a 23 weeks female fetus affected by an isolated bilateral terminal transverse defect of the lower limbs with nubbins. Both familial history and chromosomal analyses were irrelevant. We performed a deep morphological examination of the fetus in comparison with a...

Background: Progressive osseous heteroplasia (POH) (OMIM 166350) is a rare autosomal dominant condition, characterized by heterotopic ossification of the skin, subcutaneous fat and deep connective tissue. This condition is distinct from Albright’s hereditary osteodystrophy or Mccune–Albright syndrome (AHO) (OMIM 103580) and fibrodysplasia ossificans progressiva (FOP) (OMIM 135100).Presenting problem: We present an unu...

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Osteogenesis imperfecta (OI) type V is caused by a unique dominant mutation (c.−14C>T) in IFITM5, which encodes BRIL, a transmembrane ifitm-like protein most strongly expressed in osteoblasts, while type VI OI is caused by recessive null mutations in SERPINF1, encoding pigment epithelium-derived factor (PEDF). We identified a 25-year-old woman with severe OI, whose dermal fibroblasts and cultured osteoblasts displayed minimal secretion of PEDF, but ...

Bone strength is influenced by cortical thickness, area, mass and porosity, all of which contribute to nonvertebral fracture risk. Cortical porosity is one parameter of structural decay associated with bone fragility. This is caused by unbalanced and accelerated remodelling of Haversian units which enlarge, coalesce and fragment the cortex. Antiresorptive therapies will limit progression of cortical porosity; reducing existing porosity would be a goal for those already at incr...

Objective: To identify predictors of inadequate response to oral bisphosphonate therapy, defined as the incidence of ≧2 fractures while on treatment among incident oral bisphosphonate users with high refill compliance (≧80%).Material and methods: Data from computerized records and pharmacy invoices were obtained from SIDIAP (Catalonia, E) and Danish Health Registries (DK) for all incident users of oral bisphosphonates in 2006–2007 and 2000&#15...

Adolescent idiopathic scoliosis (AIS) is associated with low bone mineral density (BMD) which has been reported to be an important prognostic factor for curve progression. In addition to BMD, high-resolution peripheral quantitative computed tomography (HR-pQCT) allows measurement of volumetric BMD, bone morphology, trabecular micro-architecture, and evaluation with structure model index (SMI) and finite element analysis (FEA) for mechanical property assessment. The aim of this...

Objectives: This study aimed to evaluate detailed bone profiles during puberty for Chinese adolescents in Hong Kong using high-resolution peripheral quantitative computed tomography (HR-pQCT) and dual-energy x-ray absorptiometry (DXA).Methods: 333 healthy girls and 231 healthy boys (12–16-years-old) were recruited from secondary schools. Areal bone mineral density (aBMD) of bilateral femoral necks were measured by DXA while bone quality parameters w...

C5aR is a membrane-associated receptor for C5a, a potent immune mediator generated after complement activation. C5aR expressed in tumor infiltrating immune cells creates a favorable microenvironment for tumor progression. However, the expression of C5aR by cancer cells and its contribution to their malignant phenotype is poorly understood. Immunohistochemical analysis revealed that high levels of C5aR in human lung tumors were associated with poor survival (P=0.005) a...