Bone Abstracts

Objectives: Current guidelines recommend annual assessments of bone densitometry in boys with Duchenne muscular dystrophy (DMD). However, this recommendation is based on the assumption that bone density is a predictor of fractures in this patient group. The aim of this study was to evaluate the relationships between long-term changes in bone density, corticosteroid exposure and mobility with vertebral and long bone fractures.Methods: Twenty-four DMD boys...

Introduction: Giant cell tumour of the bone (GCTB) is a benign, locally aggressive tumour whose neoplastic stromal cells express receptor activator of nuclear factor kappa-B ligand (RANKL) and activate its receptor RANK on osteoclast-like giant cells. Denosumab (RANKL inhibitor) is an FDA/EMA approved treatment for GCTB in adults and ‘skeletally mature’ adolescents. Safety concerns include oversuppression of bone remodelling, with risk of osteonecrosis of the jaw [ON...

Introduction: In 1979 Sillence described Type II OI as perinatal lethal. We report two children whose features were consistent with Type II OI who survived beyond infancy. Both have mutations previously reported in cases of lethal OI.Case 1: This girl was born full term, small for gestational age (SGA) following antenatal detection of short bowed femora. Skeletal survey showed multiple long bone fractures and a small chest with beaded ribs. A COL1A2 gene...

Hypophosphatasia (HPP) is a rare genetic disease characterised by low tissue-nonspecific alkaline phosphatase activity, causing defective mineralisation of bone and teeth. There is limited data on the measurement of bone mineral density (BMD) and body composition in these children.Objectives: To assess whether BMD and lean body mass (LBM) in treatment naïve children with HPP correlate with functional outcomes using the 6-minute walk test (6MWT)....

Osteoporosis-related hip fractures represent a substantial cause of mortality and morbidity in industrialized countries; nonetheless, past studies in Austria lack mortality figures save during hospitalization (in-hospital mortality).We therefore retrospectively retrieved pseudonymized invoice data from Austrian social insurance authorities covering roughly 98% of the entire population including 31 548 subjects over 51 years of age who sustained first hip...

Osteoporosis is commonly known as the prime risk factor for hip fracture in the elderly. We thus evaluated status and effect of osteoporosis treatment amongst hip fracture patients in a large Austrian cohort.Retrospectively retrieved pseudonymized invoice data from Austrian social insurance authorities covering roughly 98% of the entire population included 31 548 subjects over 50 years with first hip fractures between July 2008 and December 2010, with fo...

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In type 2 diabetes mellitus (T2DM) patients, an increased fracture risk is observed, although the bone mineral density is even higher than in non-diabetic patients. This raises the question of the quality of the organic and inorganic matrix in bone1–3. T2DM is also known to forward dysfunctions in the development of soft tissues such as brittle skin due to cross-linking of the collagen or inflammation of the gingiva. For the latter, a possible influence of diab...

Bisphosphonates (BPs) including alendronate (ALN) are the most widely prescribed therapy for post-menopausal osteoporosis. Despite their overall excellent safety record and efficacy in reducing fractures, concerns have been expressed lately regarding potential detrimental effects due to prolonged bone turnover reduction, although no definite cause–effect relationship has been established to date. The purpose of the present study was to determine bone material quality by R...

Background: DMD is a progressive neuromuscular disease often treated with oral glucocorticoids (GC) to prolong ambulation and maintain cardiovascular function. However, the osteotoxic GC effects often result in a skeleton prone to fractures. DMD boys presenting with fractures are treated with bisphosphonates (BP) but evidence of beneficial effects is limited. The aim of this work was to review the use of intravenous BPs in a group of severely affected boys with fractures.<...