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osteogenesis imperfecta
Non-accidental injury: confusion with temporary brittle bone disease and mild osteogenesis imperfecta
      
Osteogenesis imperfecta-like syndrome with severe mental retardation and extrapyramidal tract signs
      
Generalised osteoporosis with overtubulation of long bones was similar to that of osteogenesis imperfecta (OI).
      
Osteogenesis imperfecta with joint contractures: Bruck syndrome
      
We describe an Egyptian boy with osteogenesis imperfecta who was born with thumb contractures and bilateral antecubital pterygia.
      
This condition is probably a hitherto undescribed form of osteogenesis imperfecta type III or a new bone disease.
      
Objective: To determine the effect of intravenous pamidronate on the bone mineral density of children with osteogenesis imperfecta and spastic quadriplegic cerebral palsy.
      
Materials and methods: Charts of 38 children with osteogenesis imperfecta (n=20) and spastic quadriplegic cerebral palsy (n=18) treated with pamidronate were retrospectively reviewed.
      
The area of greatest lateral distal femur bone mineral density improvement was in the metaphysis adjacent to the growth plate, with a 96±87.8% improvement in the osteogenesis imperfecta group and 65.7±55.2% improvement in the cerebral palsy group.
      
No children with spastic quadriplegic cerebral palsy experienced fractures after the first week of treatment, whereas patients with osteogenesis imperfecta continued to have fractures but at a decreased rate.
      
Conclusions: Intravenous pamidronate given at 3- to 4-month intervals proved to be effective in increasing bone mineral density in patients with osteogenesis imperfecta and spastic quadriplegic cerebral palsy.
      
The greatest gains in bone mineral density were observed in the children with osteogenesis imperfecta, but they did continue to fracture, albeit at a decreased rate.
      
In contrast to osteogenesis imperfecta, which is the main differential diagnosis, a familial history is absent in these patients.
      
Wormian bones in osteogenesis imperfecta and other disorders
      
When are Wormian bones significant is not an easy question to answer, but its relevance is important in relation to bone dysplasias such as osteogenesis imperfecta.
      
In order to attempt an answer, the skull radiographs of 81 cases of osteogenesis imperfecta of varying ages were examined for the presence of wormian bones.
      
They were found in all the cases of osteogenesis imperfecta but not in the normal skulls.
      
Other incidental findings in the skulls of the cases of osteogenesis imperfecta were also appraised.
      
Radiological manifestations of bisphosphonate treatment with APD in a child suffering from osteogenesis imperfecta
      
In one case of osteogenesis imperfecta without pneumonia, we believe normal muscle forces bent ribs weakened by deficiency of normal cortical architecture.
      
 

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