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. 1992;12:35–41.

The Morphology of the Long Bone Physis in Hypochondroplasia and Comparison with Other Growth Disorders

Frederick R Dietz, Blayne L Fritz, Laura J Trombino, Jerry Maynard
PMCID: PMC2328748

Abstract

The morphology of fibular physes from three growth disorders (achondroplasia, hypochondroplasia, and pseudoachondroplasia) were compared with normal physes. All growth disorders' physes were abnormal with the most consistent abnormality occurring in the hypertrophic zone (HZ). Marked shortening of the HZ was seen in the hypochondroplast and the achondroplast. The shortening of the HZ was more severe in the achondroplast compared to the hypochondroplast. Good column formation was present in most of the physis in both of these disorders. Disorganization of the entire physis was present in pseudoachondroplasia.

These observations are consistent with the hypothesis that physeal elongation is driven by HZ cell enlargement directed longitudinally by the extra cellular matrix. These observations suggest that abnormal HZ function may be the final common pathway of growth disorders.

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Selected References

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  1. Beals R. K. Hypochondroplasia. A report of five kindreds. J Bone Joint Surg Am. 1969 Jun;51(4):728–736. [PubMed] [Google Scholar]
  2. Brighton C. T. Structure and function of the growth plate. Clin Orthop Relat Res. 1978 Oct;(136):22–32. [PubMed] [Google Scholar]
  3. Buckwalter J. A., Mower D., Schafer J., Ungar R., Ginsberg B., Moore K. Growth-plate-chondrocyte profiles and their orientation. J Bone Joint Surg Am. 1985 Jul;67(6):942–955. [PubMed] [Google Scholar]
  4. Buckwalter J. A., Mower D., Ungar R., Schaeffer J., Ginsberg B. Morphometric analysis of chondrocyte hypertrophy. J Bone Joint Surg Am. 1986 Feb;68(2):243–255. [PubMed] [Google Scholar]
  5. Byers P. H. Molecular heterogeneity in chondrodysplasias. Am J Hum Genet. 1989 Jul;45(1):1–4. [PMC free article] [PubMed] [Google Scholar]
  6. Cooper R. R., Ponseti I. V., Maynard J. A. Pseudoachondroplastic dwarfism. A rough-surfaced endoplasmic reticulum storage disorder. J Bone Joint Surg Am. 1973 Apr;55(3):475–484. [PubMed] [Google Scholar]
  7. Godfrey M., Hollister D. W. Type II achondrogenesis-hypochondrogenesis: identification of abnormal type II collagen. Am J Hum Genet. 1988 Dec;43(6):904–913. [PMC free article] [PubMed] [Google Scholar]
  8. Godfrey M., Keene D. R., Blank E., Hori H., Sakai L. Y., Sherwin L. A., Hollister D. W. Type II achondrogenesis-hypochondrogenesis: morphologic and immunohistopathologic studies. Am J Hum Genet. 1988 Dec;43(6):894–903. [PMC free article] [PubMed] [Google Scholar]
  9. Holtrop M. E. The ultrastructure of the epiphyseal plate. I. The flattened chondrocyte. Calcif Tissue Res. 1972;9(2):131–139. doi: 10.1007/BF02061951. [DOI] [PubMed] [Google Scholar]
  10. Horton W. A., Machado M. A., Chou J. W., Campbell D. Achondrogenesis type II, abnormalities of extracellular matrix. Pediatr Res. 1987 Sep;22(3):324–329. doi: 10.1203/00006450-198709000-00017. [DOI] [PubMed] [Google Scholar]
  11. Hunziker E. B., Schenk R. K., Cruz-Orive L. M. Quantitation of chondrocyte performance in growth-plate cartilage during longitudinal bone growth. J Bone Joint Surg Am. 1987 Feb;69(2):162–173. [PubMed] [Google Scholar]
  12. KEMBER N. F. Cell division in endochondral ossification. A study of cell proliferation in rat bones by the method of tritiated thymidine autoradiography. J Bone Joint Surg Br. 1960 Nov;42B:824–839. [PubMed] [Google Scholar]
  13. Maynard J. A., Ippolito E. G., Ponseti I. V., Mickelson M. R. Histochemistry and ultrastructure of the growth plate in achondroplasia. J Bone Joint Surg Am. 1981 Jul;63(6):969–979. [PubMed] [Google Scholar]
  14. Maynard J. A., Ippolito E. G., Ponseti I. V., Mickelson M. R. Histochemistry and ultrastructure of the growth plate in metaphyseal dysostosis: further observations on the structure of the cartilage matrix. J Pediatr Orthop. 1981;1(2):161–169. doi: 10.1097/01241398-198110000-00006. [DOI] [PubMed] [Google Scholar]
  15. Murray L. W., Bautista J., James P. L., Rimoin D. L. Type II collagen defects in the chondrodysplasias. I. Spondyloepiphyseal dysplasias. Am J Hum Genet. 1989 Jul;45(1):5–15. [PMC free article] [PubMed] [Google Scholar]
  16. Murray L. W., Rimoin D. L. Abnormal type II collagen in the spondyloepiphyseal dysplasias. Pathol Immunopathol Res. 1988;7(1-2):99–103. doi: 10.1159/000157111. [DOI] [PubMed] [Google Scholar]
  17. Pedrini-Mille A., Maynard J. A., Pedrini V. A. Pseudoachondroplasia: biochemical and histochemical studies of cartilage. J Bone Joint Surg Am. 1984 Dec;66(9):1408–1414. [PubMed] [Google Scholar]
  18. Ponseti I. V. Skeletal growth in achondroplasia. J Bone Joint Surg Am. 1970 Jun;52(4):701–716. [PubMed] [Google Scholar]
  19. Poole A. R., Pidoux I., Reiner A., Rosenberg L., Hollister D., Murray L., Rimoin D. Kniest dysplasia is characterized by an apparent abnormal processing of the C-propeptide of type II cartilage collagen resulting in imperfect fibril assembly. J Clin Invest. 1988 Feb;81(2):579–589. doi: 10.1172/JCI113356. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Poole A. R., Rosenberg L., Murray L., Rimoin D. Kniest dysplasia: a probable type II collagen defect. Pathol Immunopathol Res. 1988;7(1-2):95–98. doi: 10.1159/000157102. [DOI] [PubMed] [Google Scholar]
  21. Rimoin D. L., Hughes G. N., Kaufman R. L., Rosenthal R. E., McAlister W. H., Silberberg R. Endochondral ossification in achondroplastic dwarfism. N Engl J Med. 1970 Oct 1;283(14):728–735. doi: 10.1056/NEJM197010012831404. [DOI] [PubMed] [Google Scholar]
  22. Rimoin D. L., Silberberg R., Hollister D. W. Chondro-osseous pathology in the chondrodystrophies. Clin Orthop Relat Res. 1976 Jan-Feb;(114):137–152. [PubMed] [Google Scholar]
  23. Scott C. I., Jr Achondroplastic and hypochondroplastic dwarfism. Clin Orthop Relat Res. 1976 Jan-Feb;(114):18–30. [PubMed] [Google Scholar]
  24. Seinsheimer F., 3rd, Sledge C. B. Parameters of longitudinal growth rate in rabbit epiphyseal growth plates. J Bone Joint Surg Am. 1981 Apr;63(4):627–630. [PubMed] [Google Scholar]
  25. Sillence D. O., Horton W. A., Rimoin D. L. Morphologic studies in the skeletal dysplasias. Am J Pathol. 1979 Sep;96(3):813–870. [PMC free article] [PubMed] [Google Scholar]
  26. Speer D. P. Collagenous architecture of the growth plate and perichondrial ossification groove. J Bone Joint Surg Am. 1982 Mar;64(3):399–407. [PubMed] [Google Scholar]
  27. Stanescu V., Stanescu R., Maroteaux P. Pathogenic mechanisms in osteochondrodysplasias. J Bone Joint Surg Am. 1984 Jul;66(6):817–836. doi: 10.2106/00004623-198466060-00002. [DOI] [PubMed] [Google Scholar]
  28. Stănescu V., Bona C., Ionescu V. The tibial growing cartilage biopsy in the study of growth disturbances. Acta Endocrinol (Copenh) 1970 Aug;64(4):577–601. doi: 10.1530/acta.0.0640577. [DOI] [PubMed] [Google Scholar]
  29. Walker B. A., Murdoch J. L., McKusick V. A., Langer L. O., Beals R. K. Hypochondroplasia. Am J Dis Child. 1971 Aug;122(2):95–104. doi: 10.1001/archpedi.1971.02110020029001. [DOI] [PubMed] [Google Scholar]
  30. Wynne-Davies R., Walsh W. K., Gormley J. Achondroplasia and hypochondroplasia. Clinical variation and spinal stenosis. J Bone Joint Surg Br. 1981;63B(4):508–515. doi: 10.1302/0301-620X.63B4.7298674. [DOI] [PubMed] [Google Scholar]

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