The Indian Journal of Pediatrics

, Volume 77, Issue 9, pp 987–991

Evaluation of Osteopathy in Thalassemia by Bone Mineral Densitometry and Biochemical Indices

  • Rashid Merchant
  • Amish Udani
  • Vipla Puri
  • Valentina D’cruz
  • Deepak Patkar
  • Aarti Karkera
Special Article

Abstract

Objective

To evaluate osteopathy in thalassemia by bone mineral densitometry (BMD) and biochemical indices.

Methods

Prospective review analysis with no follow up from 2006 to 2007 of 42 regularly transfused thalassemics aged 10–25 years (27 boys, 15 girls) was done. Anthropometry, pubertal stage and symptomatology were noted. Urinary C–terminal cross–linked telopeptide of type–1 collagen (Crosslaps) by ELISA; serum 25–OH vitamin D and osteocalcin by RIA; parathyroid hormone (PTH) and ferritin by chemiluminescence and IGF–1 by Enzyme immunoassay were evaluated. Dual Energy X-ray Absorptiometry (DEXA) of lumbar spine and femur was done on Lunar prodigy system. Data was entered and analyzed using the SPSS for Windows software. Mean comparisons were done by ANOVA 1 and data was compared using Chi–square test and p value < 0.05 was taken as significant.

Results

Of 42 patients, 81% had osteoporosis by Z–score of DEXA. Urinary crosslaps was high in 55%; 36% had increased osteocalcin; 62% had low vitamin D levels; 38% had high parathyroid levels and IGF–1 was low in 52%. Mean serum ferritin level was 5344 ± 2855 ng/dl. There was statistical significance (p = 0.046) between chronological age and BMD. All 42 cases were divided into two groups: Group–1 (Normal DEXA), Group–2 (Abnormal DEXA) and analysis of biochemical indices between two groups showed no significant difference in any of the biochemical parameters.

Conclusion

This study revealed majority of thalassemics with inadequate chelation have bone resorption with advancing chronological age and BMD should be evaluated regularly for early diagnosis to prevent morbidity.

Keywords

DEXA Osteoporosis Thalassemia Vitamin D 

References

  1. 1.
    Soliman AT, El Banna N, Fattah MA, et al. Bone mineral density in prepubertal children with beta thalassemia: correlation with growth and hormonal data. Metabolism. 1998;47:541–8.CrossRefPubMedGoogle Scholar
  2. 2.
    Benigno V, Bertelloni S, Giampiero I, et al. Effects of thalassemia major on bone mineral density in late adolescence. J Pediatr Endocrinol Metab. 2003;16:337–42.PubMedGoogle Scholar
  3. 3.
    Karimi M, Ghiam AF, Hashemi A, et al. Bone mineral density in beta thalassemia major and intermedia. Indian Pediatr. 2007;44:29–32.PubMedGoogle Scholar
  4. 4.
    Getrim/BMI calculator. Available from http://www.getrim.in. Accessed 21 Aug 2009.
  5. 5.
    Jensen CE, Tuck SM, Agnew JE, et al. High prevalence of low bone mass in thalassemia major. Br J Hematol. 1998;103(4):911–5.CrossRefGoogle Scholar
  6. 6.
    Perrotta S, Cappellini M, Bertoldo F, et al. Osteoporosis in β–thalassemia major patients: analysis of the genetic background. Br J Hematol. 2000;111:461–6.CrossRefGoogle Scholar
  7. 7.
    Bekherinia MR, Abdollah Shamshiraz A, Kamgar M, et al. Serum Zinc and its relation to bone mineral density in β–thalassemic adolescents. Trace Elem Res. 2004;9:215–24.CrossRefGoogle Scholar
  8. 8.
    Angastiniotis M, Pavlides N, Aristidou K, et al. Bone pain in thalassemia: assessment of DEXA and MRI findings. J Pediatr Endocrinol Metab. 1998;11(3):779–84.PubMedGoogle Scholar
  9. 9.
    Origa R, Fiumana E, Gamberini MR, et al. Osteoporosis in β–thalassemia: clinical and genetic aspects. Ann NY Acad Sci. 2005;1054:451–6.CrossRefPubMedGoogle Scholar
  10. 10.
    Rund D, Rachmilewitz E. Thalassemia major–older patients, new therapy. Blood Rev. 1995;9:25–32.CrossRefPubMedGoogle Scholar
  11. 11.
    Voskaridou E, Terpos E. New insights into the pathophysiology and management of osteoporosis in patients with β–thalassemia. J Hematol. 2004;127(2):127–39.CrossRefGoogle Scholar
  12. 12.
    Morabito N, Gaudio A, Lasco A, et al. Osteoprotegerin and RANKL in the pathogenesis of thalassemia–induced osteoporosis. J Bone Miner Res. 2004;19(5):722–7.CrossRefPubMedGoogle Scholar
  13. 13.
    George A, Bhaduri A, Sen S, Choudhry VP. Physical growth parameters in Thalassemic children. Indian J Pediatr. 1997;64:861–71.CrossRefPubMedGoogle Scholar
  14. 14.
    Ruggerio L, De Sanctis V. Multicentre study on prevalence of fractures in transfusion dependent thalassemic patients. J Pediatr Endocrinol Metab. 1998;11:773–8.Google Scholar
  15. 15.
    Scuteralli PN, Orzincolo C, Calzolari F, Tilotta F. The ribs in β–thalassemia. Course of the changes in relation to transfusion therapy. Radiol Med (Torino). 1983;69:654–60.Google Scholar
  16. 16.
    Katz K, Horev G, Goshen J, Tamary H. The pattern of bone disease in transfusion–dependent thalassemia major. Isr J Med Sci. 1994;30:577–80.PubMedGoogle Scholar
  17. 17.
    Mahachoklertwattana P, Sirikulchayanonta V, Chuansumrit A, et al. Bone histomorphometry in children and adolescents with β–Thalassemia disease: iron associated focal osteomalacia. J Clin Endocrinol Metab. 2003;88(8):3966–72.CrossRefPubMedGoogle Scholar
  18. 18.
    Somneuk D, Vorachai S, Pantep A, et al. Abnormalities in bone mineral density and bone histology in thalassemia. J Bone Miner Res. 2003;18:1682–8.CrossRefGoogle Scholar
  19. 19.
    Angelucci E, Brittenham GM, McLaren CE, et al. Hepatic iron concentration and total body iron stores in thalassemia major. N Engl J Med. 2000;343:327–31.CrossRefPubMedGoogle Scholar
  20. 20.
    Yazigi A, Maalouf G, Inati-Khoriati A, et al. Bone mineral density in β–thalassemic Lebanese children. J Musculoskel Neuron Interact. 2002;2(5):463–8.Google Scholar
  21. 21.
    Mc Donagh K, Nienhuis A. The thalassemias. In: Nathan, Oski, editors. Hematology of infancy and childhood. 4th ed. Philadelphia: WB Saunders; 1993. p. 841.Google Scholar
  22. 22.
    Pollak RD, Rachmilewitz E, Blumenfeld A, et al. Bone mineral metabolism in adults with β–thalassemia major and intermedia. Br J Hematol. 2000;111:902–7.CrossRefGoogle Scholar
  23. 23.
    Chow LH, Frci JV, Hodsman AB, Valberg LS. Low serum 25–hydroxy vitamin D in iron status. Gastroenterology. 1985;88:865–9.PubMedGoogle Scholar
  24. 24.
    Rioja L, Girot R, Garabedian M, et al. Bone disease in children with homozygous β–thalassemia. Bone Miner. 1990;8:69–86.CrossRefPubMedGoogle Scholar
  25. 25.
    Voskaridou E, Kyrtsonis MC, Terpos E, et al. Bone resorption is increased in young adults with thalassemia major. Br J Hematol. 2001;112:36–41.CrossRefGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2010

Authors and Affiliations

  • Rashid Merchant
    • 1
  • Amish Udani
    • 1
  • Vipla Puri
    • 2
  • Valentina D’cruz
    • 2
  • Deepak Patkar
    • 3
  • Aarti Karkera
    • 3
  1. 1.Department of PediatricsDr Balabhai Nanavati hospital MumbaiMumbaiIndia
  2. 2.Department of RIAP. D. Hinduja National Hospital and Medical Research Centre MumbaiMumbaiIndia
  3. 3.Department of RadiologyDr. Balabhai Nanavati Hospital MumbaiMumbaiIndia

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