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: 6.31). 02/2013; 98(3). DOI: 10.1210/jc.2012-4195
Source: PubMed

ABSTRACT Abstract Not Available.

  • [Show abstract] [Hide abstract]
    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; DOI:10.1172/JCI74230 · 13.77 Impact Factor
  • [Show abstract] [Hide abstract]
    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 · 2.59 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Purpose of review Discovery of the Wnt signaling pathway and understanding the central role of osteocyte in skeletal homeostasis have been the major advances in skeletal biology over the past decade. Sclerostin, secreted mainly (but not exclusively) by osteocytes, has emerged as a key player in skeletal homeostasis. This review highlights the most relevant recent advances. Recent findings Sclerostin by inhibiting Wnt signaling pathway decreases bone formation and osteoblast differentiation and promotes osteoblast apoptosis. Ability to measure serum sclerostin levels better clarified the role of sclerostin in various physiologic and pathologic states. Early clinical trials with antibodies to sclerostin have produced robust increases in bone mineral density, and fracture prevention trials are underway. Summary Since the discovery of Wnt signaling pathway and sclerostin's association with high bone mass, there has been a remarkable progress. Clinical trials with fracture endpoints, already underway, should expand osteoanabolic therapeutic horizon in the very near future. Measurement of sclerostin levels in a number of conditions has advanced our knowledge about pathophysiology of skeletal and nonskeletal disorders in an altogether new light.
    Current Opinion in Endocrinology Diabetes and Obesity 10/2014; 21(6). DOI:10.1097/MED.0000000000000114 · 3.77 Impact Factor