Bone Abstracts

Background: Infantile hypercalcemia occurs in ~15% of patients with Williams Syndrome (WS) and is typically mild. Severe hypercalcemia has been reported in infants/toddlers with WS, requiring treatment with intravenous (IV) saline hydration, furosemide, calcitonin, calcium and vitamin D restriction, and in some cases IV bisphosphonates.Presenting problem: Three cases of infants with WS age 9–13 months presented with severe hypercalcemia, failure to ...

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Osteoporotic fractures are very common and represent an enormous unmet medical need. Our group has previously reported that addition of rTNF to the fracture site promotes fracture healing in C57/BL6 mice (Glass et al. PNAS 2011). Using a murine fracture model of endochondral healing, we observed that local addition of rTNF only accelerates fracture repair if administered within the first 24 h following injury. The optimal therapeutic dose is 1 ng. TNF is firs...

Selenium (Se) is an essential trace element active in the catalytic sites of many redox enzymes, including glutathione peroxidase and the iodothyronine deiodinases that metabolize thyroid hormones. In humans 25 genes encode selenoproteins, in which a specific tRNA incorporates the amino acid selenocysteine during protein translation. Although specific roles for many selenoproteins are unclear, studies suggest an important role for Se in skeletal development and bone maintenanc...

Hypothyroidism delays bone development and linear growth, whereas thyrotoxicosis accelerates skeletal development and is an important risk factor for osteoporosis in adulthood. Even sub-clinical thyrotoxicosis is associated with fracture, and treatment with thyroxine at doses that suppress TSH results in increased bone turnover and low BMD in postmenopausal women.To investigate the mechanism of T3 action in the skeleton, we characterized mice ...

Objectives: To identify and determine the extent to which effective/ineffective steps in the implementation of the Kaiser Permanente Southern California Healthy Bones Model of Care were perceived by physician champions and Healthy Bones Care Managers.Methods: The subjects in the study included 20 Physician Champions and 35 Healthy Bones Care Managers employed in the Kaiser Permanente Southern California Healthy Bones Model of Care.25 have been employed i...

Childhood hypothyroidism results in delayed skeletal maturation and impaired growth. Thyroid hormones act via temporo-spatially regulated thyroid hormone receptors α (TRα) and (TRβ).In the skeleton, TRα is the predominant receptor and we hypothesise that the skeletal effects of hypothyroidism are mediated by TRα.To investigate this we assessed the response of wild-type (WT), TRα knockout (TRα...

Thyrotoxicosis results in osteoporosis, and thyroid hormone (T3) stimulates osteoclastic bone resorption by unknown mechanisms. We previously demonstrated that knockout mice lacking thyroid hormone receptor α (TRα0/0) are euthyroid but have high bone mass, whereas mice lacking TRβ (TRβ−/−) are thyrotoxic and osteoporotic. Tartrate resistant acid phosphatase (TRAcP) staining revealed osteoclast numbers were reduced by 13% (<...

Introduction: Benign osteopetrosis type 2 (OPTA2) (Albers-Schönberg disease) is usually associated with a heterozygous mutation in CLCN7. Patients may be asymptomatic and present following an x-ray taken for other reasons or with a low trauma fracture. There may be a family history. Homozygous mutations in CLCN7 usually result in severe disease which presents in the neonatal period or early infancy. We present a case of benign osteopetrosis associated wi...

Objectives: Synovial fluid glutamate concentrations increase in various arthritides. Activation of kainate (KA) and AMPA glutamate receptors (GluRs) increase interleukin 6 (IL6) release and cause arthritic pain respectively. GluR antagonists represent potential peripheral treatments for inflammatory arthritis and inflammatory mechanisms that contribute to osteoarthritis (OA). We hypothesised that AMPA and KA GluRs are expressed in arthritic joint tissues and that peripheral ad...