Abstract
Over a ten years period 60 children with osteogenesis imperfecta (O.J.) were studied. 15 patients were classified as type I showing a clinically mild disease. 45 patients presenting with O.I.at birth could be subdivided into group A(23) with “broad bone” type femurs and into group B(22) with “thin bone” type femurs on neonatal radiographs. Group A and to a lesser extent group B showed significantly more skeletal abnormalities than type I. Starting from birth the annual incidence of fractures was high, however at a different level in group A and B and significantly declined after the age of 5 years. In contrast type I showed a peak of fractures at the age of 5 years. A considerable reduction in height and weight during the first 5 years was observed in group A(-8 SD) and to a lesser extent in group B(-4 SD). Type I did not develop significant short stature. All patients showed a typical centrifugal body fat distribution. Cardiac malformations and kidney stones were important extraskeletal manifestations of O.I. showing an incidence of 10%. IGFI levels were low, normal or slightly subnormal indicating normal growth hormone status.
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Vetter, U., Ermisch, J., Wörsdörfer, A. et al. 92: OSTEOGENESIS IMPERFECTA. Pediatr Res 24, 276 (1988). https://doi.org/10.1203/00006450-198808000-00118
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DOI: https://doi.org/10.1203/00006450-198808000-00118