The Paradox of Prevention-Bilateral Atypical Subtrochanteric Fractures due to Bisphosphonates in Osteogenesis Imperfecta

Departments of Endocrinology (K.N.M., N.G.) and Imaging (A.W.), Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham B15 2TH, United Kingdom
The Journal of Clinical Endocrinology and Metabolism (Impact Factor: 6.21). 02/2013; 98(3). DOI: 10.1210/jc.2012-4195
Source: PubMed


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    ABSTRACT: Background/aims: Intravenous bisphosphonate therapy is the mainstay of medical treatment in osteogenesis imperfecta (OI) and has been shown to increase bone mass, decrease bone pain, improve mobility, and reduce the incidence of fractures. Sclerotic metaphyseal lines parallel to the growth plate are seen on long bone radiographs following cyclical intravenous therapy. These areas create stress risers within the bone that may act as foci for subsequent fractures as exemplified in this clinical case. Methods: An 8-year-old girl with OI sustained a distal radial fracture following 3 years of treatment with 6-monthly intravenous zoledronate. Her diagnosis, response to treatment, and subsequent fracture at a sclerotic metaphyseal line is described. Results: Peripheral quantitative computer tomography was used to characterise the presence of multiple stress risers at the distal forearm. Trabecular bone mineral density fluctuated from 34 to 126% compared to neighbouring 2-mm regions. Conclusion: There remain many unanswered questions about optimal bisphosphonate treatment regimens in children with OI. The formation of stress risers following intravenous bisphosphonate treatment raises the hypothesis that a more frequent and low-dose bisphosphonate regimen would provide more uniform dosing of bone in the growing child and reduce the likelihood of fractures compared to current treatment practices.
    Hormone Research in Paediatrics 12/2013; 81(3). DOI:10.1159/000355111 · 1.57 Impact Factor
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    ABSTRACT: A 42-year-old premenopausal woman with osteogenesis imperfecta presents to the metabolic bone clinic. She has a daughter with osteogenesis imperfecta who is seen regularly in a specialist pediatric clinic, but the patient herself hasn't had a clinical consultation in years. She has pain and stiffness in her back and is worried for her future bone health. The patient asks, "Am I going to fall apart?" She had numerous fractures in childhood, including fractures of her femur and wrist; fractured her ankles several times in her late teens; and had occasional fractures in adulthood. Her last fracture was a comminuted fracture of her humerus three years ago, when she stumbled and fell forward onto her hands and knees. The woman is hyperextensible and thinks her ankles feel weak. Her bone mineral density T scores are -2.6 at the lumbar spine and -1.9 at the total hip, and spine imaging shows several vertebral endplate deformities, but overall preservation of vertebral height. What are the available pharmacological and nonpharmacological strategies to preserve her skeletal health and function?
    The Journal of clinical investigation 01/2014; 124(2):1-2. DOI:10.1172/JCI74230 · 13.22 Impact Factor
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    ABSTRACT: We report a case of a successfully healed atypical femoral fracture (AFF) following treatment with teriparatide in a patient with osteogenesis imperfecta (OI). To our knowledge no successful treatment of AFF's with teriparatide in this subpopulation has ever been described.METHODS: This is a case report of an AFF treated with teriparatide.RESULTS: The patient was treated with hormone replacement therapy for 18 years and bisphosphonates for nine years before suffering a spontaneous AFF in the form of a dislocated non-comminute transverse fracture of the right femoral shaft and an open reduction and internal fixation (ORIF) with a T2 Femoral Nail was done. Due to nonunion and another fracture distal to the nail she was re-operated with exchange ORIF and off label treatment with teriparatide 20 microgram/day was started. An x-ray one month later showed early signs of fracture healing. A subsequent x-ray 6 months after the last operation showed a solid healing of both right femoral fractures.CONCLUSION: This is a rare case that highly suggests a potential fracture healing effect of teriparatide treatment and highlights a potential significant practical therapeutic consideration in relation to the management of AFF with delayed healing.
    Endocrine Practice 06/2014; 20(10):1-7. DOI:10.4158/EP14141.CR · 2.81 Impact Factor
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