Abnormal Bone Metabolism in Thalassemia

  • P. J. Giardina
  • R. Schneider
  • M. Lesser
  • B. Simmons
  • A. Rodriguez
  • J. Gertner
  • M. New
  • M. W. Hilgartner


Historically, homozygous beta-thalassemia major has been associated with marked osseous changes, originally described by Cooley et al. in 1927 [1]. The pathogenesis of the abnormal bony changes has been ascribed to the underlying massive ineffective erythropoiesis and erythroid expansion of medullary bone, and the subsequent thinning of cortical bone, as well as metabolic and endocrine dysfunction secondary to hemochromatosis [2, 3]. Concurrent osteopenia results in frequent pathological fractures and premature epiphyseal fusion [4, 5].


Bone Mineral Content Densitometry Bone Mineral Content Chelation Therapy Thalassemic Child Bone Mineral Content Densitometry Study 
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  1. 1.
    Cooley TB, Witwer ER, Lee P (1927) Anemia in children with splenomegaly and peculiar changes in the bones. Am J Dis Child 34: 347–363Google Scholar
  2. 2.
    Caffey I (1975) Cooley’s anemia: a review of the roentgenographic findings in skeleton. Am JR 78: 381–391Google Scholar
  3. 3.
    Canale VC (1974) Beta thalassemia: a clinical review. Pediatr Ann 3: 6–30Google Scholar
  4. 4.
    Currarino G, Erlandson ME (1964) Premature fusion of epiphyses in Cooley’s anemia, Radiology 83: (4): 656–664PubMedGoogle Scholar
  5. 5.
    Dines DM, Canale VC, Arnold WD (1976) Fractures in thalassemia. J Bone Joint Surg [Am] 58: 662–666PubMedGoogle Scholar
  6. 6.
    Piomelli S, Danoff SJ, Becker MH et al. (1969) Prevention of bone malformations and cardiomegaly in Cooley’s anemia by early hypertransfusion regimen. Ann NY Acad Sci 165: 427–436PubMedCrossRefGoogle Scholar
  7. 7.
    Papageorgiou O, Papanastasiou DA, Beratis NG (1991) Scoliosis in β-thalassemia. Pediatrics 88: 341–345PubMedGoogle Scholar
  8. 8.
    Cummings SR (1987) Bone mineral densitometry (Health and Public Policy Committee). Ann Intern Med 107: 932–936Google Scholar
  9. 9.
    Ponder SW, McCormick DP, Fawcett D et al. (1990) Spinal bone mineral density in children aged 5.00 through 11.99 years. Am J Dis Child 144: 1346–1348PubMedGoogle Scholar
  10. 10.
    Glastre C, Braillon P, David L et al. (1990) Measurement of bone mineral content of the lumbar spine by dual energy X–ray absorptiometry in normal children: correlation with growth parameters. J. Clin Endocrinol Metab 70: 1330–1333PubMedCrossRefGoogle Scholar
  11. 11.
    Thomas KA, Cook SD, Bennett JT et al. (1991) Femoral neck and lumbar spine bone mineral densities in a normal population 3–20 years of age. J Pediatr Orthop 11: 48–58PubMedCrossRefGoogle Scholar
  12. 12.
    Mazess RB, Barden HS, Ettinger M et al. (1987) Spine and femur density using dual–photon absorptiometry in US white women. Bone Miner 2: 211–219PubMedGoogle Scholar
  13. 13.
    Mazess RB, Bamca IIS, Drinka PJ (1987) Influence of age and body weight on spine and femur bone mineral density in US white men. J Bone Miner Res 5: 645–651CrossRefGoogle Scholar
  14. 14.
    DeVernejoul MC, Pointillart A, Golenzer CC et al. (1984) Effects of iron overload on bone remodeling in pigs. Am J Pathol 166: 377Google Scholar
  15. 15.
    Diamond T, Stiel D, Posen S (1989) Osteoporosis in hemochromatosis: iron excess, gonadal deficiency of other factors. Ann Intern Med 110: 430–436PubMedGoogle Scholar
  16. 16.
    Aksoy M, Camli N, Dincol K, Erdem S, Dincol G (1973) On the problem of ‘rib-within-a-rib’ appearance in thalassemia intermedia. Radiol Clin Biol 42: 126–133PubMedGoogle Scholar
  17. 17.
    Kattamis C, Touliators N, Haidas S et al. (1970) Growth of children with thalassemia: effect of different transfusion regimens. Arch Dis Child 45: 502PubMedCrossRefGoogle Scholar
  18. 18.
    Giardina PJ, Grady RW, Ehlers KH et al. (1990) Current therapy of Cooley’s anemia: a decade of experience with subcutaneous desferrioxamine. Ann NY Acad Sci 162: 275–285CrossRefGoogle Scholar
  19. 19.
    Gabrielle O (1971) Hypoparathyroidism associated with thalassemia. South Med J 64: 115–116CrossRefGoogle Scholar
  20. 20.
    Flynn DM, Fairney A, Jackson D et al. (1976) Hormonal changes in thalassemia major. Arch Dis Child 51: 828–836PubMedCrossRefGoogle Scholar
  21. 21.
    Saenger P, Schwartz E, Markenson AL et al. (1980) Depressed serum somatomedin activity in beta thalassemia. J Pediatr 96: 214–218PubMedCrossRefGoogle Scholar
  22. 22.
    Modell B, Berdoukas V (eds) (1984) Growth, puberty and endocrinopathy. In: The clinical approach to thalassemia. Grane and Stratton, London PP 175–197Google Scholar
  23. 23.
    DeVirgillis S, Congia M, Frau F et al. (1988) Deferoxamine-induced growth retardation in patients with thalassemia major. J Pediatr 113: 661–669CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1995

Authors and Affiliations

  • P. J. Giardina
  • R. Schneider
  • M. Lesser
  • B. Simmons
  • A. Rodriguez
  • J. Gertner
  • M. New
  • M. W. Hilgartner

There are no affiliations available

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