Bone Abstracts

Biographical DetailsDr A Linglart is a Paediatric Endocrinologist working at the Hôpital St Vincent de Paul in Parism, France. She has a special interest in rare diseases....

Objectives and method: We retrospectively reviewed records of current patients with osteogenesis imperfecta (OI) to identify those on risedronate, doses used and effects of treatment. BMD changes over time were calculated as difference between measurement before risedronate and at one year (including measurements −3–0 m and 10–14 m after starting risedronate respectively).Results: 219/296 (74%) patients with OI had received bisphosphonate...

Osteoclasts are highly specialized cell types, responsible for the resorption of bone matrix. Coordinated with osteoblasts they contribute to a proper bone turnover. An impaired or reduced function of the osteoclast leads to a pathogenic increase of the bone mass and finally osteopetrosis. Lysosomal hydrolases, as exemplied by CTSK and Acp5 (TRAP), are known to play an important role in the function of osteoclasts, and knockout mouse models of these proteins develop a bone res...

Background: Magnetic resonance imaging (MRI) is used clinically to assess bone marrow, muscle and joints. The assessment of cortical and trabecular structure using MRI may provide further insight into the muscle–bone–bone marrow unit. Previous studies using MRI to evaluate microarchitecture are confined to research due to the need for specially adapted coils and navigator software to limit motion artifact. We present a novel statistical method using HRpQCT to determi...

Objectives: The clinical assessment of bone mass in children with osteogenesis imperfecta (OI) is normally assessed by DXA. Further information about bone microarchitecture is obtained from bone biopsy which is invasive and requires anaesthetic. High-resolution peripheral quantitative computer tomography (HRpQCT) is an in-vivo imaging modality capable of assessing skeletal microstructure and integrity to a resolution of 80 μm. To date, no HRpQCT studies have incl...

Mineralized tissues like bone, articular calcified cartilage or mineralized turkey leg tendon (MTLT) are build by a composite of hydroxyapatite nano-particles and organic matrix. In bone and MTLT the matrix is formed by collagen type-I, but in contrast to bone in MTLT the collagen is uniaxial orientated, while in cartilage the matrix consists of collagen type-II and proteoglycans.Composition/orientation differences were investigated by a new scanning aco...

Vitamin D deficiency is common in older persons and non-western immigrants. Vitamin D is often started in loading doses of 50 000 IU/ml solution. Though, generally considered safe, this highly concentrated solution carries some risks as is illustrated by the following cases. A woman of >80 years old was admitted with hypercalcemia, calcium 3.27 mmol/l. She complained of nausea, thirst and polyuria for 3 months. History included cholecystitis, atrial fibrillation, myocardia...

The co-chaperone protein, Bcl-2-associated athanogene 1 (BAG-1) is expressed ubiquitously in bone including cells of the osteoblast-lineage and, plays an important role in cell proliferation, apoptosis and differentiation by regulating signalling and transcription. The study aims to elucidate the function of BAG-1 in osteoblast development by examining differences in osteogenic differentiation of bone marrow stromal cells (BMSCs) from Bag-1+/−</sup...

Objectives: Guidelines for treatment of vitamin D deficiency (VDD) vary. We aimed to review the range of treatment regimens for VDD used locally and variation in responses.Methods: We conducted a retrospective review of the records of consecutive patients referred to a Children’s Bone Disease Service with a putative diagnosis of VDD over a 14-month period. Data collected includes vitamin D type used, dose and duration of treatment, and pre- and post...

Background: Measurement and interpretation of metaphyseal shape in children is difficult. We aim to develop relevant assessment methods using commonly available spiral computed tomography (CT) scanning.Methods: We analysed 12 pairs of age and pubertal stage matched subjects from a larger group of children recruited into a non-interventional case–control study in which all had suffered trauma; half had sustained a fracture. Each subject underwent ant...