Vertebral bone mineral content in osteogenesis imperfecta

1985 ◽  
Vol 37 (1) ◽  
pp. 14-18 ◽  
Author(s):  
David Kurtz ◽  
Kathleen Morrish ◽  
Jay Shapiro
1979 ◽  
Vol 50 (6) ◽  
pp. 639-643 ◽  
Author(s):  
H. E. Nielsen ◽  
U. Pedersen ◽  
H. Hvid Hansen ◽  
O. Elbrønd

Blood ◽  
2001 ◽  
Vol 97 (5) ◽  
pp. 1227-1231 ◽  
Author(s):  
Edwin M. Horwitz ◽  
Darwin J. Prockop ◽  
Patricia L. Gordon ◽  
Winston W. K. Koo ◽  
Lorraine A. Fitzpatrick ◽  
...  

Preclinical models have shown that transplantation of marrow mesenchymal cells has the potential to correct inherited disorders of bone, cartilage, and muscle. The report describes clinical responses of the first children to undergo allogeneic bone marrow transplantation (BMT) for severe osteogenesis imperfecta (OI), a genetic disorder characterized by defective type I collagen, osteopenia, bone fragility, severe bony deformities, and growth retardation. Five children with severe OI were enrolled in a study of BMT from human leukocyte antigen (HLA)–compatible sibling donors. Linear growth, bone mineralization, and fracture rate were taken as measures of treatment response. The 3 children with documented donor osteoblast engraftment had a median 7.5-cm increase in body length (range, 6.5-8.0 cm) 6 months after transplantation compared with 1.25 cm (range, 1.0-1.5 cm) for age-matched control patients. These patients gained 21.0 to 65.3 g total body bone mineral content by 3 months after treatment or 45% to 77% of their baseline values. With extended follow-up, the patients' growth rates either slowed or reached a plateau phase. Bone mineral content continued to increase at a rate similar to that for weight-matched healthy children, even as growth rates declined. These results suggest that BMT from HLA-compatible donors may benefit children with severe OI. Further studies are needed to determine the full potential of this strategy.


1993 ◽  
Vol 6 (4) ◽  
pp. 314-323 ◽  
Author(s):  
Pavel Neumann ◽  
Anna-Lisa Osvalder ◽  
Anders Nordwall ◽  
Per Lövsund ◽  
Tommy Hansson

1983 ◽  
Vol 18 (4) ◽  
pp. S26
Author(s):  
Daniel Baran ◽  
Grigorios Arsenis ◽  
Daniel Prosser

1993 ◽  
Vol 52 (1) ◽  
pp. 5-9 ◽  
Author(s):  
R. F. J. M. Laan ◽  
W. C. A. M. Buijs ◽  
L. J. Th. O. van Erning ◽  
J. A. M. Lemmens ◽  
F. H. M. Corstens ◽  
...  

1983 ◽  
Vol 64 (5) ◽  
pp. 537-540 ◽  
Author(s):  
Bjørn Krølner ◽  
Birte Toft

1. The skeletal effects of simple bed rest and re-ambulation were studied in a consecutive series of 34 patients (aged 18–60 years) hospitalized with low backache due to protrusion of a lumbar intervertebral disc. The bone mineral content of the second, third and fourth lumbar vertebrae was determined by dual-photon (153Gd) absorptiometry immediately after admission to the hospital, at the end of the bed-rest period (mean 27 days, range 11–61 days) and approximately 15 weeks later (range 11–24 weeks). 2. During recumbency a mean decrease in lumbar spine bone mineral content of 0.9% per week was observed. 3. Re-ambulation resulted in bone mineral gain, and restoration of lumbar spine bone mineral content was nearly complete after 4 months. 4. The findings suggest that the simple therapeutic bed-rest regimen leads to excessive vertebral bone loss. Recurrent bed-rest periods may predispose to spinal osteoporosis.


1986 ◽  
Vol 13 (3) ◽  
pp. 340-343 ◽  
Author(s):  
J. R. Vetter ◽  
W. H. Perman ◽  
W. A. Kalender ◽  
R. B. Mazess ◽  
J. E. Holden

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