Bone Abstracts

Objective: The aim of this study is to compare the bone health index (BHI) for healthy Swiss children born in 1955 with healthy Swiss children born a generation later.Method: BHI is derived from the cortical thickness in the three middle metacarpals. It is determined with the BoneXpert medical device, which automatically analyses a standard bone age hand radiograph. The measurement result is independent of the sharpness of the image. The image data are f...

Objective: The BoneXpert method for automated bone age determination from hand X-rays also determines the cortical thickness T and the bone width W in the three middle metacarpals. From these, the method derives the cortical area A=π W T (1 – T/W), the metacarpal index MCI =A/(WW) and the Bone Health Index. Recently, the method has been extended down to new-borns, and the aim of this study is to report reference curves for these bone measures.M...

Type V OI is characterised by interosseous membrane calcification and hyperplastic callus formation, but the infantile phenotype is less well recognised. In 2012 Arundel et al. described distinctive radiographic changes in an infant with type V OI. We report two further male infants (with genetic confirmation of type V OI) confirming the highly characteristic and consistent radiographic appearances that should aid early diagnosis.Case 1 – P...

Biographical DetailsBonny SpeckerDr Bonny Specker is currently Director and Chair of the E.A. Martin Program in Human Nutrition at South Dakota State University. Prior to moving to SDSU in 1997, she was at the University of Cincinnati and Cincinnati Children’s Hospital Medical Center where she received her PhD in Epidemiology and spent 15 years...

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Despite recent improvements in therapeutic management of osteosarcoma, ongoing challenges in improving the response to chemotherapy warrants new strategies still needed to improve overall patient survival. Among new therapeutic approaches, zoledronic acid represents a promising adjuvant molecule to chemotherapy to limit the osteolytic component of bone tumors. However, zoledronic acid triggers the elevation of heat shock proteins (Hsp), including Hsp27 and clusterin (CLU), whi...

Introduction: Cardiovascular disease and osteoporosis have often been reported to coexist in older people. However, the literature is conflicting regarding size and indeed direction of the association. The aim of the present study was therefore to assess associations between the Framingham general cardiovascular risk score and bone characteristics in a cohort of older adults.Methods: We studied 374 men and 379 women, born 1931–1939, who participated...

: Glucocorticoid-induced osteoporosis (GIO) is the most frequent secondary osteoporosis in patients undergoing steroid therapy.Recently we demonstrated that the inhibition of bone formation in GIO is occurring in part via the suppression of autocrine cytokines by the glucocorticoid receptor (GR) monomer in osteoblasts (Cell Metab 11, 517–531). Since emerging evidences indicate that microRNAs (miRNAs) play a critical role in the differentiat...

Background: Idiopathic infantile hypercalcemia (IIH) is a rare disorder caused by CYP24A1 loss-of-function mutation, resulting in impaired degradation of 1,25-dihydroxyvitamin D3. Typical signs include muscle hypotonia, dehydration, failure to thrive, psychomotor retardation, constipation, nephrocalcinosis. IIH should be distinguished from other causes of hypercalcemia in infancy. Treatment includes low calcium diet, glucocorticoids, furosemide, calcitonin. Pamidron...

Background: Idiopathic infantile hypercalcemia (IIH) is a rare cause of infantile hypercalcemia characterized by failure to thrive, vomiting, dehydration, and nephrocalcinosis. This condition has recently been associated with mutations in the CYP24A1 gene, which encodes 25-hydroxyvitamin D3 24-hydroxylase, the key enzyme of 1,25-dihydroxyvitamin D3 degradation. Until now, only 13 cases genetically tested for IIH have been reported in the literature.Case ...