Cranial base pathology in pediatric osteogenesis imperfecta patients treated with bisphosphonates

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Femoral neck fractures in osteogenesis imperfecta treated with bisphosphonates

PURPOSE Osteogenesis imperfecta (OI) is a condition characterised by bone fragility and multiple fractures, which cause considerable morbidity in the affected patients. Most cases are associated with mutations in one of the type I collagen genes. Recently, bisphosponates have been used widely to reduce pain and the incidence of fragility fractures in OI in children, even though there have been ...

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Experience with bisphosphonates in osteogenesis imperfecta.

Until recently, medical management of osteogenesis imperfecta, a genetic disorder of reduced bone mass and frequent fractures, was elusive, and treatment was focused on maximizing mobility and function. The introduction of bisphosphonates for the treatment of osteogenesis imperfecta 14 years ago changed this paradigm. Cyclic intravenous pamidronate therapy leads to an increase in bone density a...

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Oral bisphosphonates for paediatric osteogenesis imperfecta?

www.thelancet.com Published online August 6, 2013 http://dx.doi.org/10.1016/S0140-6736(13)61531-7 1 Children and adolescents with osteogenesis imperfecta have numerous fractures, often after what would otherwise seem trivial trauma. Even in mild disease, the risk of breaking a long bone (femur, tibia, fi bula, humerus, radius, or ulna) is about 100 times higher than in the general population. A...

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Hypercalcaemia in osteogenesis imperfecta treated with pamidronate.

The response to the bisphosphonate, pamidronate, is reported in a child with osteogenesis imperfecta who had recurrent symptomatic hypercalcaemia after immobilisation following fractures. Oral clodronate was effective in the prevention of immobilisation hypercalcaemia in the same child. The bisphosphonates may have other roles in osteogenesis imperfecta by decreasing bone turnover.

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Changes in cranial base and craniocervical junction during growth in healthy individuals and in patients with Osteogenesis imperfecta

4 Contents 6 List of original publications 8 Abbreviations 9 1. Introduction 11 2. Review of the literature 12 2.1. Bone metabolism and growth 12 2.2. Neurocranium and craniocervical junction 15 2.2.1. Anatomy 15 2.2.2. Prenatal development 16 2.2.3. Postnatal development 20 2.3. Osteogenesis imperfecta 23 2.3.1. Molecular basis 23 2.3.2. Clinical feature...

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ژورنال

عنوان ژورنال: Journal of Neurosurgery: Pediatrics

سال: 2015

ISSN: 1933-0707,1933-0715

DOI: 10.3171/2014.11.peds14113