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...

Pain is common in patients with fibrous dysplasia (FD), however the mechanisms and presentation of pain is poorly understood. Retrospective studies have shown that pain in FD presents along a broad spectrum, responds variably to treatment, and does not correlate with FD disease burden. Pain may be generally conceptualized into two categories: nociceptive pain (associated with actual or potentially tissue damaging stimuli) and neuropathic pain (caused by dysfunction of the soma...

Objectives: Optic neuropathy (ON) is a critical complication of fibrous dysplasia (FD). Early surgical decompression can prevent blindness; however, prophylactic intervention may cause blindness from intra-operative nerve damage. There is therefore a critical need to develop diagnostic tests for accurate and early detection of ON in patients with FD. Currently used methodologies, including radiographic evaluation of optic canal size and optic nerve length, are suboptimal and c...

Fibrous dysplasia (FD) is a benign skeletal disease caused by somatic activating mutations of Gsα leading to formation of expansile fibroosseous lesions. These may occur in isolation or in association with McCune-Albright syndrome (MAS), characterized by skin pigmentation and hyperfunctioning endocrinopathies, including growth hormone (GH) excess. FD in the craniofacial skeleton may result in significant morbidity including facial asymmetry, vision and hearing ...

Objectives: Fibrous dysplasia is a rare bone and endocrine disorder resulting from somatic activating mutations in GNAS. In the skeleton, proliferation of undifferentiated stromal cells results in osseous lesions that are prone to deformity, fracture, and pain. Lesions may affect one bone or many, and may occur in isolation or in association with hyperfunctioning endocrinopathies, termed McCune-Albright syndrome (MAS). Scoliosis is a potentially serious, even lethal c...

Fibrous dysplasia (FD) is a benign skeletal disease caused by activating mutations of Gsα. These mutations lead to formation of abnormal and mechanically unsound bone and fibrotic tissue. Clinical sequelae include deformity, fracture, and pain. Studies in bisphosphonates have shown improvement in bone pain and inconsistent effects on FD mineralization; however interpretation has been limited by a lack of controlled trials.Objecti...

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...