Searchable abstracts of presentations at key conferences on calcified tissues

ba0004p184 | (1) | ICCBH2015

A subtrochanteric femoral stress fracture following bisphosphonate treatment in an adolescent girl

Boyce Alison , Tosi Laura , Gafni Rachel

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...

ba0007p80 | (1) | ICCBH2019

Characterization of pain in patients with fibrous dysplasia

Spencer Tiahna , Javaid Kassim , Boyce Alison

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...

ba0007p220 | (1) | ICCBH2019

New perspectives in diagnosis and management of optic neuropathy in fibrous dysplasia: utility of optical coherence tomography and computed tomography measurements

Pan Kristen , FitzGibbon Edmond , Lee Janice , Collins Michael , Boyce Alison

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...

ba0004p73 | (1) | ICCBH2015

Long-term outcomes of surgical treatment for craniofacial fibrous dysplasia

Boyce Alison , Burke Andrea , Peck Carolee Cutler , Dufresne Craig , Collins Michael

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 ...

ba0006oc21 | (1) | ICCBH2017

Scoliosis in fibrous dysplasia/McCune-albright syndrome

Berglund Jason , Tella Sri Harsha , Kim Lauren , Stanton Robert , Collins Michael , Boyce Alison

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...

ba0002oc21 | Miscellaneous | ICCBH2013

A randomized, double-blind, placebo-controlled trial of alendronate treatment for fibrous dysplasia of bone

Boyce Alison M , Kelly Marilyn H , Brillante Beth A , Kushner Harvey , Wientroub Shlomo , Riminucci Mara , Bianco Paolo , Robey Pamela G , Collins Michael T

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...

ba0007oc7 | (1) | ICCBH2019

Comparison of zoledronate and pamidronate in children with skeletal disorders: Short term safety experience from a single institution

Tosi Laura , Estrada Andrea , Floor Marianne , Kim Mirini , Weigley Lindsay , Dollar Christina , Gillies Austin , Roberts Mary Scott , Gafni Rachel , Boyce Alison

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...