Bone Abstracts

The oim/oim mouse resembles type III OI. Our study mimics clinical questions: is RANK-Fc as efficacious as ALN in reducing fracture number? Is RANK-Fc safe to administer to children? Is there any advantage to receiving RANK-Fc treatment post adolescence, following no treatment or ALN treatment from infancy through adolescence?All animal work was done under an IACUC-approval. Mice – n=200, WT or oim/oim. Treatment spanned a...

Radiographs of the second metacarpal are used to assess bone strength development in paediatric populations. Children with achondroplasia and osteogenesis imperfecta (OI) have known differences in bone strength. Details of how bone strength develops and compares within these populations to unaffected children are lacking. A data set for patients with achondroplasia and OI was established.A retrospective IRB-approved review of bone-age films (n=6...

Objectives: Respiratory insufficiency is the leading cause of mortality for individuals diagnosed with Osteogenesis Imperfecta (OI), a skeletal dysplasia clinically characterized by fractures, bone fragility, and scoliosis. The objective of this study is to explore respiratory function and its relation to quality of life, type of OI, presence of scoliosis, stature, and other factors such as age or co-existing co-morbidities.Methods: Adults with OI comple...

Objectives: Osteogenesis imperfecta (OI) is a group of rare genetic disorders characterized by osteoporosis, predisposition to fracture, and scoliosis. Recently, however, there has been increased focus on pulmonary insufficiency, as it is the leading cause of mortality in individuals with OI. The primary objective of this study is to determine if reduced pulmonary function in individuals with OI is intrinsic to the underlying connective tissue disorder. Another goal of this st...

Objective: The purpose of this study was to determine whether osteogenesis imperfecta (OI) patients entering adulthood should continue with bisphosphonate therapy or would benefit from switching to a RANKL blockade therapy. To address this question, we used a mouse model of type III OI.Methods: Animal studies were performed under IACUC approval. OI (oim/oim) and wild-type (WT) mice were treated from 2–26 weeks with i) saline; ii) alendronat...

Objective: The purpose of this ongoing study was to determine whether osteogenesis imperfecta (OI) patients entering adulthood should continue with bisphosphonate therapy or would benefit from switching to a RANKL blockade therapy. To address this question, we used a mouse model of type-III OI.Methods: Under IACUC-approval, +/+ and oim/oim mice were treated from 2–26 weeks (n=20/treatment/genotype) with either 1) saline 24 weeks; 2...

Objectives: The objective of this study is to characterize patterns of bone morphology in children with osteogenesis imperfecta (OI), using measurements of the second metacarpal. A secondary objective is to look for impact of bisphosphonate treatment on bone morphology in this population.Methods: This is an IRB-approved retrospective review of 82 de-identified bone age films (AP hand/wrist) for 42 children with OI (17M, 25F). Measurements (Sectra IDS 7 P...

Objective: Existing case series examining outcomes following spine surgery for patients with skeletal dysplasias demonstrate high rates of complications. Yet, these earlier studies are from a single institution, contain surgeries performed almost 50 years ago, and only include patients with a diagnosis of achondroplasia. The objective is to determine contemporary outcomes after spinal surgery in patients with skeletal dypslasias, focusing on complications and revisions. We pre...