Brittle bone disease (osteogenesis imperfecta)

2003 ◽  
Vol 5 (1) ◽  
pp. 39-45 ◽  
Author(s):  
c. r. paterson

this paper describes some radiological features of osteogenesis imperfecta and temporary brittle bone disease. both conditions cause fractures in early childhood that the parents cannot explain. they can underlie the finding of unsuspected fractures when x-rays are done for other reasons, including fractures of different ages. both can readily be confused with non-accidental injury.in these disorders any type of fracture can occur; no fracture pattern makes bone disease more or less likely. metaphyseal fractures, often regarded as specific for non-accidental injury, also have a wide range of other causes including several bone diseases. osteopenia cannot be reliably used in diagnosis, not least because it can be absent in some bone diseases such as osteogenesis imperfecta. wormian bones, if present in excess, are a valuable pointer to osteogenesis imperfecta but their absence does not eliminate this diagnosis.there is growing evidence for the identification of temporary brittle bone disease as a distinctive disorder with its own characteristic clinical and radiological findings.


2000 ◽  
Vol 15 (9) ◽  
pp. 1650-1658 ◽  
Author(s):  
Francis H. Glorieux ◽  
Frank Rauch ◽  
Horacio Plotkin ◽  
Leanne Ward ◽  
Rose Travers ◽  
...  

New Medicine ◽  
2020 ◽  
Vol 24 (2) ◽  
Author(s):  
Agnieszka Kozubska ◽  
Danuta Chlebna-Sokół ◽  
Elżbieta Jakubowska-Pietkiewicz ◽  
Izabela Michałus ◽  
Karolina Beska-Bartecka ◽  
...  

Introduction. Osteogenesis imperfecta is an inherited disorder of the connective tissue. Which in most cases, is caused by mutations in the genes encoding collagen type I. Apart from clinical features, there are characteristic dental aberrations. Aim. The purpose of this research was the assessment of the condition of teeth and therapeutic needs of children with the congenital brittle bone disease. Material and methods. The questionnaires with patient’s parents, consisting medical history and dental history were performed. The intra-oral examination included the condition of the dentition, the presence of dentinogenesis imperfecta, malocclusion, the assessment of the attrition index and dmft/DMFT index. Results. 62 patients with osteogenesis imperfecta were examined – 32 boys and 30 girls. There were normal eruption times of deciduous (48 patients – 77.42%) and permanent teeth (34 patients – 82.93%) reported in the majority of the patients with OI. In most cases bad eating and hygienic habits were observed. Dentinogenesis imperfecta in deciduous and permanent teeth was reported mostly in type III of OI. dmft/DMFT index among children with OI were low in comparison to the population studies of corresponding age groups. Conclusions. Despite bad eating and hygienic habits as well as pathological structure of dentition of patients with the congenital brittle bone disease, caries index among these children were low in comparison to the population studies of corresponding age groups.


Bone ◽  
2002 ◽  
Vol 31 (1) ◽  
pp. 12-18 ◽  
Author(s):  
L.M Ward ◽  
F Rauch ◽  
R Travers ◽  
G Chabot ◽  
E.M Azouz ◽  
...  

2002 ◽  
Vol 17 (1) ◽  
pp. 30-38 ◽  
Author(s):  
Francis H. Glorieux ◽  
Leanne M. Ward ◽  
Frank Rauch ◽  
Ljiljana Lalic ◽  
Peter J. Roughley ◽  
...  

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