Bone Abstracts

Objectives: To elucidate the incidence and risk factors of skeletal toxicity in children with ALL treated with the dexamethasone-based DCOG-ALL9 protocol.Methods: Prospectively, the cumulative incidence of fractures was assessed in 672 patients and compared between different subgroups using the log-rank test. Serial measurements of bone mineral density of the lumbar spine (BMDLS) were performed in 399 ALL patients using dual energy X-ray absor...

Fibrous dysplasia is a mosaic disease resulting from post-zygotic activating mutations of the GNAS locus which codes for the α subunit of the Gs G-coupled protein receptor. In bone, impaired signalling results in impaired differentiation and proliferation of bone marrow stromal cells which are replaced by fibrous tissue resulting in bone fragility and dysplasia. All children diagnosed with Fibrous Dysplasia at The Royal National Orthopaedic Hospital since 2009 (103 childr...

: This study analyses the long-term effect of rhGH on final height (FH) and bone health in renal transplanted patients.Twenty-one young adult patients, aged 17–26 years, were studied. Group A consisted of 15 patients (12 boys) who received rhGH during 3.0 years before transplantation. After transplantation three boys needed rhGH again for 3.5 years. In group B six patients (three boys) didn’t need rhGH before transplantation, three girls receiv...

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...

Objective: To investigate whether patients with secondary osteoporosis or other metabolic bone disease (SECOB) have a higher re-fracture or mortality risk.Method: Patients with a recent non-vertebral fracture who visited the Fracture Liaison Service (FLS) of a hospital were prospectively followed for 2 years. Pearson Chi-square, Fisher’s Exact test, independent samples T-test, and Cox regression models were used.Results: In to...

Background: Osteogenesis imperfecta (OI) is a heterogeneous group of disorders characterized by bone fragility. The current classification comprises five forms (OI types I–V) with autosomal dominant inheritance and seven rarer forms (OI types VI–XII) with recessive inheritance. OI type V (MIM 610967) has distinguishing radiological features, such as propensity to hyperplastic callus formation, calcification of the forearm interosseous membrane, radial-head dislocatio...

In this case report, we present a brother and sister with hereditary vitamin D resistant rickets (HVDRR). Both children presented at the age of 18 months with severe rickets and elevated serum levels of 1,25-(OH)2D3. They differ from each other in that the girl presented with hypophosphatemia instead of hypocalcemia. Besides, she developed alopecia earlier than the boy and needed more 1,25-(OH)2D3 supplementation. Interestingly, the ...

Introduction: Osteogenesis imperfecta (OI) is a genetically heterogeneous disorder characterized by bone fragility. OI type V, with autosomal dominant inheritance, is characterized by ossification of the forearm interosseus membrane, radiodense metaphyseal bands, propensity for hyperplastic callus formation, and mesh-like lamellation on bone histology. Type V OI probands are reported to have white sclerae and normal teeth. Recent reports identified the cause of type V OI as a ...

Objectives: We recently demonstrated that cow’s milk allergic infants who received an amino acid-based formula (AAF) for 16 weeks as oral feeding had adequate mineral status (1). One factor that may negatively affect mineral solubility and bioavailability and hence mineral status, is high gastric pH (2), but data on this in infants are lacking. Therefore, the present study evaluates serum concentrations of phosphorus, calcium and magnesium in a subgroup of infants on AAF ...

Objectives: impairment of bone mineral density (BMD) is a complication of childhood cancer treatment. A comprehensive review on the occurrence of impaired BMD in childhood cancer survivors (CCS), and which disease subgroups are at risk has never been pursued so far. The aim of this study was to summarize all knowledge on BMD status and associated determinants in long-term adult childhood cancer survivors (CCS) based on available literature.Methods: an el...