Bone Abstracts

Background: Bisphosphonates are increasingly used to treat disorders of low bone mineral density (BMD) in children and adolescents. Long-term bisphosphonate use in adults has been associated with and increased risk of atypical subtrochanteric and diaphyseal femoral fractures (AFFs). To date, bisphosphonate-related AFFs have not been reported in children or adolescents.Presenting problem: A 16-year-old girl presented with a 3-week history of left thigh pa...

Objectives: Two important goals of the Rare Diseases Clinical Research Network Brittle Bone Disorders Consortium (RDCRN BBD) are i) to perform collaborative clinical research in brittle bone disorders including a longitudinal observational study driven by genotypic association and ii) to explore use of the PROMIS tool to provide valid quality of life (QOL) measures in individuals with Osteogenesis Imperfecta (OI). This work has been motivated by the fact that all current outco...

Background: Proteus syndrome (PS) is a rare mosaic disorder comprising asymmetric bony and soft tissue overgrowth leading to significant morbidity. Placement of guided growth hardware with subsequent epiphyseal arrest improves leg length and angular deformities in pediatric patients without PS.Presenting problem: The purpose of this study was to review the surgical approach and present outcomes, complications, and recommendations in eight patients with P...

Objectives: Bisphosphonates are frequently used in children with skeletal disorders, however optimal dosing and regimens are unknown. Early treatment focused on pamidronate (PAM), a second-generation formulation, however use of zoledronate (ZOL), a more potent third-generation bisphosphonate, has recently increased due to shorter and less frequent infusions. The objective of this study is to compare short-term safety of ZOL and PAM in a pediatric population.<p class="abste...

Background: Osteogenesis imperfecta (OI) is a heterogeneous, rare disorder most commonly affecting type I collagen. The OI Adult Natural History Initiative (OI ANHI) was established following a 2010 Ostegenesis Imperfecta Foundation (OIF) national meeting at which patient participants noted that i) there is little information about the natural history and progression of OI beyond childhood, and ii) most of the data available are focused on musculoskeletal issues. Adults with O...