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- W206483575 endingPage "842" @default.
- W206483575 startingPage "831" @default.
- W206483575 abstract "Osteogenesis imperfecta (OI) is a heritable disorder of bone formation that may affect more than 1:10,000 individuals. It is characterized by bone fragility due to low bone mass giving an increased fracture incidence. The bone fragility has led to the adoption of the lay name of “brittle bone disease.” The heritable nature of the disorder distinguishes it from idiopathic juvenile osteoporosis, although clinical osteoporosis is also a consequence of OI. It has long been a topic of interest for basic scientists and a source of frustration to clinicians. Much progress has been made concerning the molecular, cellular, and material basis of the disorder, but up till now these insights have contributed little to alleviate the disease burden on affected individuals. Only the recent advent of pamidronate therapy has brought clear improvements to the lives of children and adolescents suffering from moderate to severe OI. Nevertheless, this form of treatment does not constitute a cure, but rather is an adjunct to physiotherapy, rehabilitation, and orthopedic care. Gene-based therapy offers the hope for a curative treatment of OI, but remains at present in the early stages of preclinical research. New therapeutic avenues in severe OI will have to be evaluated against the results obtained with bisphosphonates." @default.
- W206483575 created "2016-06-24" @default.
- W206483575 creator A5004489635 @default.
- W206483575 creator A5013272432 @default.
- W206483575 date "2006-01-01" @default.
- W206483575 modified "2023-09-24" @default.
- W206483575 title "Osteogenesis Imperfecta" @default.
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