0000000000011625
AUTHOR
B. F. Pontz
Osteogenesis imperfecta: a clinical study of the first ten years of life.
One hundred twenty-seven children with osteogenesis imperfecta (O.I.) were studied during the first 10 years of life. According to Sillence, 40 patients were assigned to type I, 39 to type III, and 48 to type IV O.I. Centiles for height, weight, and the annual number of fractures could be established for the different types of O.I. The development of the skeletal changes could be documented for the different forms of the disease. At birth, the skeletal changes were significantly more severe in type III than in type IV patients. During the first 10 years of life the number of fractures, extent of skeletal deformities, and growth retardation did not differ between types III and IV. Only fract…
Hüftgelenkssonographie bei Skelettdysplasien mit metaphysärer Beteiligung
Skeletal dysplasias with metaphyseal involvement show radiological changes which permit their classification. There are, however, changes in the cartilage which could be demonstrated by hip sonography in two children, one with Stickler's syndrome and the other with metaphyseal chondrodysplasia. The dangers of mis-diagnosis are discussed.
SV40 transformed fibroblasts recognize the same 140 kD fibronectin chemotactic fragment as non-transformed cells
SV40-virus-transformed human embryonal fibroblasts show an enhanced chemotactic response to the glycoprotein fibronectin. However, they recognize the same chemotactic active region as non-transformed fibroblasts. The result suggests that an enhancement of chemotaxis by fibroblasts which have been transformed with Simian Virus 40 is due not to the utilization of further chemotactic domains in the molecule, but to an increased sensitivity of the cells to the chemoattractant.
Spontanfrakturen im Säuglingsalter durch Kupfermangel
A preterm infant of 25 weeks’ gestation developed at 4 months of age an “impressed” fracture of the right femur. In another preterm infant of 27 weeks’ gestation, multiple fractures of the radius and metacarpalia were observed at age 5 months. Also a full term twin showed multiple rib fractures. Additional radiological findings were: generalized skeletal osteopenia, cupping and fraying of the metaphyses of the long bones, and subperiosteal new bone formation. Further symptoms were repeated apnoic attacks, anemia, neutropenia, and low serum levels of both copper and coeruloplasmin. Alimentary copper deficiency was diagnosed and oral copper substitution (CuCl2 1,2–2,0 µmol/kg/d) initiated. Th…