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Journal of Clinical Pathology logoLink to Journal of Clinical Pathology
. 1984 Oct;37(10):1081–1089. doi: 10.1136/jcp.37.10.1081

Iliac bone histomorphometry in adults and children with osteogenesis imperfecta.

L G Ste-Marie, S A Charhon, C Edouard, M C Chapuy, P J Meunier
PMCID: PMC498944  PMID: 6490947

Abstract

Histomorphometric analysis was performed on transiliac undecalcified bone biopsies taken from eight adults and four children with osteogenesis imperfecta. Double tetracycline labelling done before the biopsy allowed measurement of the calcification rate in 11 patients. The adults, as well as the children, had thin iliac cortices and considerably decreased trabecular bone volume. In addition, the calcification rate was significantly reduced. Since the thickness index of osteoid seams was low or normal in all subjects, it indicated a reduced bone formation at the cellular level. In the adults, there was no significant increase in the parameters of bone resorption or in the bone formation rates at the basic multicellular unit and the tissue levels. Thus the histomorphometric profile of iliac bone in osteogenesis imperfecta is characterised by cortical and trabecular osteoporosis with decreased activity of the individual osteoblasts. These findings would suggest the use of therapeutic agents stimulating bone formation rather than agents inhibiting bone resorption in osteogenesis imperfecta.

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Selected References

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  1. Albright J. P., Albright J. A., Crelin E. S. Osteogenesis imperfecta tarda. The morphology of rib biopsies. Clin Orthop Relat Res. 1975 May;(108):204–213. [PubMed] [Google Scholar]
  2. August G. P., Shapiro J., Hung W. Calcitonin therapy of children with osteogenesis imperfecta. J Pediatr. 1977 Dec;91(6):1001–1005. doi: 10.1016/s0022-3476(77)80916-5. [DOI] [PubMed] [Google Scholar]
  3. BORDIER P., MATRAJT H., MIRAVET L., HIOCO D. MESURE HISTOLOGIQUE DE LA MASSE ET DE LA R'ESORPTION DES TRAV'EES OSSEUSES. Pathol Biol. 1964 Dec;12:1238–1243. [PubMed] [Google Scholar]
  4. Baron R., Gertner J. M., Lang R., Vignery A. Increased bone turnover with decreased bone formation by osteoblasts in children with osteogenesis imperfecta tarda. Pediatr Res. 1983 Mar;17(3):204–207. doi: 10.1203/00006450-198303000-00007. [DOI] [PubMed] [Google Scholar]
  5. Bauze R. J., Smith R., Francis M. J. A new look at osteogenesis imperfecta. A clinical, radiological and biochemical study of forty-two patients. J Bone Joint Surg Br. 1975 Feb;57(1):2–12. [PubMed] [Google Scholar]
  6. Briancon D., Meunier P. J. Treatment of osteoporosis with fluoride, calcium, and vitamin D. Orthop Clin North Am. 1981 Jul;12(3):629–648. [PubMed] [Google Scholar]
  7. Byers P. H., Bonadio J. F., Steinmann B. Osteogenesis imperfecta: update and perspective. Am J Med Genet. 1984 Feb;17(2):429–435. doi: 10.1002/ajmg.1320170206. [DOI] [PubMed] [Google Scholar]
  8. Castells S., Colbert C., Chakrabarti C., Bachtell R. S., Kassner E. G., Yasumura S. Therapy of osteogenesis imperfecta with synthetic salmon calcitonin. J Pediatr. 1979 Nov;95(5 Pt 1):807–811. doi: 10.1016/s0022-3476(79)80741-6. [DOI] [PubMed] [Google Scholar]
  9. Castells S., Lu C., Baker R. K., Wallach S. Effects of synthetic salmon calcitonin in osteogenesis imperfecta. Curr Ther Res Clin Exp. 1974 Jan;16(1):1–14. [PubMed] [Google Scholar]
  10. Clark S., Zorab P. A. Hydroxyproline centiles for normal adolescent boys and girls. Clin Orthop Relat Res. 1978 Nov-Dec;(137):217–226. [PubMed] [Google Scholar]
  11. Delmas P., Meunier P. J. L'ostéoporose au cours du syndrome de Klinefelter. Données histologiques osseuses quantitatives dans cinq cas. Relation avec la carence hormonale. Nouv Presse Med. 1981 Feb 28;10(9):687–690. [PubMed] [Google Scholar]
  12. Dickson I. R., Millar E. A., Veis A. Evidence for abnormality of bone-matrix proteins in osteogenesis imperfecta. Lancet. 1975 Sep 27;2(7935):586–587. doi: 10.1016/s0140-6736(75)90173-7. [DOI] [PubMed] [Google Scholar]
  13. Doty S. B., Mathews R. S. Electron microscopic and histochemical investigation of osteogenesis imperfecta tarda. Clin Orthop Relat Res. 1971 Oct;80:191–201. doi: 10.1097/00003086-197110000-00027. [DOI] [PubMed] [Google Scholar]
  14. Epker B. N., Frost H. M. A histological study of remodeling at the periosteal, haversian canal, cortical endosteal, and trabecular endosteal surfaces in human rib. Anat Rec. 1965 Jun;152(2):129–135. doi: 10.1002/ar.1091520203. [DOI] [PubMed] [Google Scholar]
  15. Falvo K. A., Bullough P. G. Osteogenesis imperfecta: a histometric analysis. J Bone Joint Surg Am. 1973 Mar;55(2):275–286. [PubMed] [Google Scholar]
  16. Falvo K. A., Root L., Bullough P. G. Osteogenesis imperfecta: clinical evaluation and management. J Bone Joint Surg Am. 1974 Jun;56(4):783–793. [PubMed] [Google Scholar]
  17. Firschein H. E., Shill J. P. The determination of total hydroxyproline in urine and bone extracts. Anal Biochem. 1966 Feb;14(2):296–304. doi: 10.1016/0003-2697(66)90140-0. [DOI] [PubMed] [Google Scholar]
  18. Frost H. M. Tetracycline-based histological analysis of bone remodeling. Calcif Tissue Res. 1969;3(3):211–237. doi: 10.1007/BF02058664. [DOI] [PubMed] [Google Scholar]
  19. Goldfield E. B., Braiker B. M., Prendergast J. J., Kolb F. O. Synthetic salmon calcitonin. Treatment of Paget's disease and osteogenesis imperfecta. JAMA. 1972 Sep 4;221(10):1127–1129. doi: 10.1001/jama.221.10.1127. [DOI] [PubMed] [Google Scholar]
  20. Jett S., Ramser J. R., Frost H. M., Villanueva A. R. Bone turnover and osteogenesis imperfecta. Arch Pathol. 1966 Feb;81(2):112–116. [PubMed] [Google Scholar]
  21. Kocher P. Etude de l'hydroxyprolinurie dans les ostéopathies. Pathol Biol. 1966 Nov;14(21):1020–1039. [PubMed] [Google Scholar]
  22. Langness U., Behnke H. Collagen metabolites in plasma and urine in osteogenesis imperfecta. Metabolism. 1971 May;20(5):456–463. doi: 10.1016/0026-0495(71)90121-1. [DOI] [PubMed] [Google Scholar]
  23. MITOMA C., SMITH T. E., DAVIDSON J. D., UDENFRIEND S., DACOSTA F. M., SJOERDSMA A. Improvements in methods for measuring hydroxyproline: application to human urine. J Lab Clin Med. 1959 Jun;53(6):970–976. [PubMed] [Google Scholar]
  24. Melsen F., Mosekilde L. Tetracycline double-labeling of iliac trabecular bone in 41 normal adults. Calcif Tissue Res. 1978 Dec 8;26(2):99–102. doi: 10.1007/BF02013242. [DOI] [PubMed] [Google Scholar]
  25. Meunier P., Courpron P., Edouard C., Bernard J., Bringuier J., Vignon G. Physiological senile involution and pathological rarefaction of bone. Quantitative and comparative histological data. Clin Endocrinol Metab. 1973 Jul;2(2):239–256. doi: 10.1016/s0300-595x(73)80042-8. [DOI] [PubMed] [Google Scholar]
  26. Minaire P., Neunier P., Edouard C., Bernard J., Courpron P., Bourret J. Quantitative histological data on disuse osteoporosis: comparison with biological data. Calcif Tissue Res. 1974;17(1):57–73. doi: 10.1007/BF02547214. [DOI] [PubMed] [Google Scholar]
  27. Ramser J. R., Villanueva A. R., Pirok D., Frost H. M. Tetracycline-based measurement of bone dynamics in 3 women with osteogenesis imperfecta. Clin Orthop Relat Res. 1966 Nov-Dec;49:151–162. [PubMed] [Google Scholar]
  28. Rico Lenza H., Del Rio Vazquez A., Espinos Perez D. L'hydroxyprolinurie, indice de gravité dans l'osteogenesis imperfecta tarda. Nouv Presse Med. 1978 Dec 2;7(43):3941–3941. [PubMed] [Google Scholar]
  29. Riley F. C., Jowsey J., Brown D. M. Osteogenesis imperfecta: morphologic and biochemical studies of connective tissue. Pediatr Res. 1973 Sep;7(9):757–768. doi: 10.1203/00006450-197309000-00005. [DOI] [PubMed] [Google Scholar]
  30. Robichon J., Germain J. P. Pathogenesis of osteogenesis imperfecta. Can Med Assoc J. 1968 Nov 23;99(20):975–979. [PMC free article] [PubMed] [Google Scholar]
  31. Rosenberg E., Lang R., Boisseau V., Rojanasathit S., Avioli L. V. Effect of long-term calcitonin therapy on the clinical course of osteogenesis imperfecta. J Clin Endocrinol Metab. 1977 Feb;44(2):346–355. doi: 10.1210/jcem-44-2-346. [DOI] [PubMed] [Google Scholar]
  32. Shapiro J. R., Triche T., Rowe D. W., Munabi A., Cattell H. S., Schlesinger S. Osteogenesis imperfecta and Paget's disease of bone. Biochemical and morphologic studies. Arch Intern Med. 1983 Dec;143(12):2250–2257. [PubMed] [Google Scholar]
  33. Shoenfeld Y. Osteogenesis imperfecta. Review of the literature with presentation of 29 cases. Am J Dis Child. 1975 Jun;129(6):679–687. doi: 10.1001/archpedi.1975.02120430019007. [DOI] [PubMed] [Google Scholar]
  34. Sillence D. Osteogenesis imperfecta: an expanding panorama of variants. Clin Orthop Relat Res. 1981 Sep;(159):11–25. [PubMed] [Google Scholar]
  35. Teitelbaum S. L., Kraft W. J., Lang R., Avioli L. V. Bone collagen aggregation abnormalities in osteogenesis imperfecta. Calcif Tissue Res. 1974;17(1):75–79. doi: 10.1007/BF02547215. [DOI] [PubMed] [Google Scholar]
  36. Vedi S., Compston J. E., Webb A., Tighe J. R. Histomorphometric analysis of dynamic parameters of trabecular bone formation in the iliac crest of normal British subjects. Metab Bone Dis Relat Res. 1983;5(2):69–74. doi: 10.1016/0221-8747(83)90004-8. [DOI] [PubMed] [Google Scholar]
  37. Villanueva A. R., Frost H. M. Bone formation in human osteogenesis imperfecta, measured by tetracycline bone labeling. Acta Orthop Scand. 1970;41(5):531–538. doi: 10.3109/17453677008991541. [DOI] [PubMed] [Google Scholar]

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