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Bone-related complications of transfusion-dependent beta thalassemia among children and adolescents

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Abstract

Thalassemia and the blood transfusion complications associated with it predispose children to poor bone health. This study was conducted to determine the prevalence of bone-related abnormalities and identify the bone health predictors within this population. One hundred and forty transfusion-dependent beta thalassemic subjects 8–18 years old in Mashhad, Iran, participated in this cross-sectional study. Anthropometric measures, dietary intake, bone-related biomarkers and bone densitometry, were assessed. The incidence of underweight and short stature was 33.6 and 41.4 %, respectively, which were indicators of malnutrition among thalassemic subjects in this study. Low bone density was detected in the lumbar spine and femoral region in 82 and 52 % of subjects, respectively. Hypocalcemia and hypophosphatemia were seen in 22 and 18.2 %, whilst vitamin D deficiency was present in more than 85 % of thalassemic children and adolescents. The relationships between weight, height and other anthropometric indices, serum calcium and bone markers, intake of macronutrients, zinc and vitamin E with bone mineral density (BMD) and bone mineral content (BMC) in the lumbar spine and femoral area were positively related, indicating that better nutritional status were associated with higher BMD and BMC values. Puberty, gender and serum osteocalcin were negative predictors for BMD and BMC values, whereas age, weight and height were the positive predictors. High incidence of low bone density and deficit in other aspects of bone health among thalassemia patients makes routine bone health assessment necessary for this vulnerable group. Considering influencing factors, dietary counseling and preventive supplementation therapy for this high risk group of children and adolescents may be necessary, although this should be assessed by intervention studies.

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References

  1. Kuttikat A, Grant R, Chakravarty K (2004) Management of osteoporosis. J Indian Rheumatol Assoc 12:104–118

    Google Scholar 

  2. Cashman KD (2006) Milk minerals (including trace elements) and bone health. Int Dairy J 16:1389–1398

    Article  CAS  Google Scholar 

  3. Bonjour JP, Ammann P, Chevalley T, Ferrari S, Rizzoli R (2003) Nutrition aspects of bone growth: an overview. In: New S, Bonjour JP (eds) Nutritional aspects of bone health. Royal Society of Chemistry, Cambridge

    Google Scholar 

  4. Burrows M (2007) Exercise and bone mineral accrual in children and adolescents. J Sports Sci Med 6:305–312

    Google Scholar 

  5. Soyka LA, Fairfield WP, Klibanski A (2000) Hormonal determinants and disorders of peak bone mass in children. J Clin Endocrinol Metab 85:3951–3963

    Article  PubMed  CAS  Google Scholar 

  6. Tucker KL (2003) Dietary intake and bone status with aging. Curr Pharm Des 9:2687–2704

    Article  PubMed  CAS  Google Scholar 

  7. Semeao EJ, Jawad AF, Stouffer NO, Zemel BS, Piccoli DA, Stallings VA (1999) Risk factors for low bone mineral density in children and young adults with Crohn’s disease. J Pediatr 135:593–600

    Article  PubMed  CAS  Google Scholar 

  8. Henderson CJ, Specker BL, Sierra RI, Campaigne BN, Lovell DJ (2000) Total body bone mineral content in non-corticosteroid-treated postpubertal females with juvenile rheumatoid arthritis: frequency of osteopenia and contributing factors. Arthritis Rheum 43:531–540

    Article  PubMed  CAS  Google Scholar 

  9. Pirinccioglua AG, Akpolatb V, Koksala O, Haspolata K, Soker M (2011) Bone mineral density in children with beta-thalassemia major in Diyarbakir. Bone 49:819–823

    Article  Google Scholar 

  10. Leung TF, Chu Y, Lee V, Cheng FT, Leung WK, Shing MK, Li CK (2009) Long-term effects of pamidronate in thalassemic patients with severe bone mineral density deficits. J Hemoglobin 33:361–369

    Article  CAS  Google Scholar 

  11. Merchant R, Udani A, Puri V, D’cruz V, Patkar D, Karkera A (2010) Evaluation of osteopathy in thalassemia by bone mineral densitometry and biochemical indices. Indian J Pediatr 77:987–991

    Article  PubMed  Google Scholar 

  12. Bekheirnia R, Shamshirsaz AA, Kamgar M, Bouzari N, Erfanzadeh G, Pourzahedgilani N, Tabatabaie SM, Shamshirsaz AA, Kimiagar M, Ezzati F (2004) Serum zinc and its relation to bone mineral density in β-thalassemic adolescents. J Biol Trace Elem Res 97:215–224

    Article  CAS  Google Scholar 

  13. Shamshirsaz AA, Bekheirnia MR, Kamgar M, Pakbaz Z, Tabatabaei SM, Pourzahedgilani N, Bouzari N, Azarkeivan A, Hashemi SR, Moosavi F, Alebouyeh M, Vosough P, Kimiagar M, Shamshirsaz AA, Moradi M, Habibzadeh MR, Nobakhthaghighi N, Larijani B (2007) Bone mineral density in iranian adolescents and young adults with beta thalassemia major. J Pediatr Hematol Oncol 24:469–479

    Article  CAS  Google Scholar 

  14. Terpos E, Voskaridou E (2010) Treatment options for thalassemia patients with Osteoporosis. Ann N Y Acad Sci 1202:237–243

    Article  PubMed  Google Scholar 

  15. International Society for the Advancement of Kinanthropometry (2001) International Standards for Anthropometric Assessment (ISAK), 1st edn. National Library of Australia

  16. Misra M, Pacaud D, Petryk A, Collett-Solberg PF, Kappy M, on behalf of the Drug and Therapeutics Committee of the Lawson, Wilkins Pediatric Endocrine Society (2008) Vitamin D deficiency in children and its management: review of current knowledge and recommendations. Pediatrics 122:398–417

    Article  PubMed  Google Scholar 

  17. Khadilkar AV, Sanwalka NJ, Chiplonkar SA, Khadilkar VV, Mughal MZ (2011) Normative data and percentile curves for Dual Energy X-ray Absorptiometry in healthy Indian girls and boys aged 5–17 years. Bone 48:810–819

    Article  PubMed  Google Scholar 

  18. Najafipour F (2008) Evaluation of endocrine disorders in patients with thalassemia major. Int J Endocrinol Metab 2:104–113

    Google Scholar 

  19. Salama OS, Al-Tonbary YA, Shahin RA, Eldeen OA (2006) Unbalanced bone turnover in children with beta-thalassemia. J Hematol 11:197–202

    Article  CAS  Google Scholar 

  20. Wood JC, Claster S, Carson S, Menteer JD, Hofstra T, Khanna R, Coates T (2008) Vitamin D deficiency, cardiac Iron and cardiac function in thalassaemia major. Br J Haematol 141:891–894

    Article  PubMed  CAS  Google Scholar 

  21. Fung EB, Aguilar C, Micaily I, Haines D, Lal A (2011) Treatment of vitamin D deficiency in transfusion-dependent thalassemia. Am J Hematol 86:871–873

    Article  PubMed  Google Scholar 

  22. Yazigi A, Maalouf G, Inati-Khoriati A, Tamim H, Saab C (2002) Bone mineral density in beta—thalassemic Lebanese children. J Musculoskelet Neuron Interact 2:463–468

    CAS  Google Scholar 

  23. Eren E, Yilmaz N (2005) Biochemical markers of bone turnover and bone mineral density in patients with beta-thalassaemia major. Int J Clin Pract 59:46–51

    Article  PubMed  CAS  Google Scholar 

  24. Rashidi MR, Aboomardani M, Rafraf M, Arefhosseini SR, Keshtkar N, Joshanghani H (2011) Effects of Vitamin E and zinc supplementation on antioxidants in beta thalassemia major patients. Iranian J Pediatr 21:8–14

    Google Scholar 

  25. Kardinaal AFM, Hoorneman G, Vananen K, Charles P, Ando S, Maggiolini M, Charzewska J, Rotily M, Deloraine A, Heikkinen J, Juvin R, Schaafsma G (2000) Determinants of bone mass and bone geometry in adolescent and young adult women. Calcif Tissue Int 66:81–89

    Article  PubMed  CAS  Google Scholar 

  26. Palasuwan A, Soogarun S, Wiwanitkit V, Luechapudiporn R, Pradniwat P, Lertlum T (2006) Preliminary study of the effect of vitamin e supplementation on the antioxidant status of hemoglobin-e carriers. Southeast Asian J Trop Med Public Health 37:184–189

    PubMed  CAS  Google Scholar 

  27. Malina RM, Bouchard C (2004) Growth, maturation, and physical activity. Human Kinetics, Champaign

    Google Scholar 

  28. Bounds W, Skinner J, Carruth BR, Ziegler P (2005) The relationship of dietary and lifestyle factors to Bone Mineral Indexes in children. J Am Diet Assoc 105:735–741

    Article  PubMed  Google Scholar 

  29. Tantawy AA, El Kholy M, Moustafa T, Elsedfy H (2008) Bone mineral density and calcium metabolism in adolescents with Beta-Thalassemia Major. Pediatr Endocrinol Rev 6:132–135

    PubMed  Google Scholar 

  30. Winzenberg T, Powell S, Shaw KA, Jones G (2011) Effects of Vitamin D supplementation on bone density in healthy children: systematic review and meta-analysis. Br Med J 342:c7254

    Article  Google Scholar 

  31. Burtis CA, Ashwood ER, Teitz NW (1999) Teitz Textbook of Clinical Chemistry, 3rd edn. Saunders, Philadelphia

Download references

Acknowledgments

This research project was financially supported by the Mashhad University of Medical Science Research Council and Iran National Science Foundation. The contribution of the staff of the Avicenna (Bu-Ali) Research Institute, Biochemistry and Nutrition Department of the Mashhad University of Medical Science, Iran National Science Foundation (INSF), Pharmacy faculty, Noor bone densitometry center, Sarvar clinic and lab, Sadra lab and Nutrition and Dietetics Department of the Universiti Kebangsaan Malaysia is gratefully acknowledged. Scientific editing of Prof Gordon Fern is gratefully appreciated.

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All authors have no conflict of interest.

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Correspondence to Majid Ghayour-Mobarhan.

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Mirhosseini, N.Z., Shahar, S., Ghayour-Mobarhan, M. et al. Bone-related complications of transfusion-dependent beta thalassemia among children and adolescents. J Bone Miner Metab 31, 468–476 (2013). https://doi.org/10.1007/s00774-013-0433-1

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  • DOI: https://doi.org/10.1007/s00774-013-0433-1

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