Mehmet Nevzat ÇİZMECİ, Arzu AKÇAY, Teoman AKÇAY, Zafer SALCIOĞLU, Deniz TUĞCU, Ferhan AKICI, Gönül AYDOĞAN

Beta talasemi major'lu çocuk hastalarda kemik mineral yoğunluğunun değerlendirilmesi

Evaluation of bone mineral density in patients with beta thalassemia major

Osteopathies are a major cause of morbidity in patients with ǃ WKDODVHPLD PDMRU %70 7KHUH LV QR GHILQLWH protocol for scanning and imaging osteopathies in thalassemia patients although Dual-Energy X-ray Absorptiometry (DEXA) is considered to be 'gold- standard' for diagnosing osteopenia and osteoporosis. Objective : The aim of this study is to evaluate bone mineral density in thalassemi a patients of pediatric age group and to consider possible ethiological factors in patients found to suffer from osteopathies. Material and Method : This study was conducted in patients under regular follow-up at the hematology outpatient clinic of Istanbul %DNÕUN|\0DWHUQLW\DQG Childrens Hospital. Patients were selected according to the following criteria: 50 patients between the ages of 4 and 18 whose diagnosis for BTM was confirmed with hemoglobin electrophoresis and gene analysis, who receieved at least one red blood cell transfusion per month, who used at least one of three chelation therapies (desferrioxamine, deferiprone or deferasirox) for at least one year and who did not have any skeletal malformations were included in the study. Findings : Although there were no statistically significant differences between the effects of age, sex, bone minerals, ferritin values and chelation modalities, the bone mineral densities of patients in the study were found to be significantly low with 50% of patients being osteopenic and 16% of patients being osteoporotic.

PDF

___

1. DeBaun MR, Vichinsky E. Hemoglobinopathies. in: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, editors. 17th ed. Nelson Textbook of Pediatrics. Philadelphia: Saunders; 2007. p.2025-37.
2. Sanctis V, Angastiniotis M, Eleftheriou A. Thalassemic Bone Disease, An Overview. Pediatr Endocrinol Rev 2008;6 Suppl 1:71-80.
3. Dresner Pollak R, Rachmilewitz E, Blumenfeld A, Idelson A, Goldfarb AW. Bone mineral metabolism in adults with β-thalassaemia major and intermedia. Br J Haematol 2000;111(3):902-7.
4. Bielinski BK, Darbyshire PJ, Mathers L, Crabtree NJ, Kirk JM, Stirling HF, et al. Impact of disordered puberty on bone density in beta-thallasemia major. Br J Haematol 2003;120(2):353-8.
5. Danesi L, Cherubini R, Ciceri L, Graziadei G, Cappellini MD, Cavagnini F, et al. Evaluation of spine and hip bone density by DXA and QCT in thalassemic patients. J Pediatr Endocrinol Metab 1998;11(Suppl 3): 961-2.
6. Mylona M, Leotsinides M, Alexansrides T, Zoumbos N, Dimopoulos PA. Comparison of DXA, QCT and trabecular structure in beta thalassemia. Eur J Haematol 2005;74(5):430-7.
7. Angelopoulos NG, Katounda E, Rombopoulos G, Goula A, Kaltzidou V, Kaltsas D, et al. Evaluation of bone mineral density of the lumbar spines in patients with beta thalassemia major DEXA and QCT: a comparison study. J Pediatr Hematol Oncol 2006;28(2):73-8.
8. Kyriakou A, Savva SC, Savvides I, Pangalou E, Ioannou YS, Christou S, et al. Gender differences in the prevalence and severity of bone disease in thalassemia. Pediatr Endocrinol Rev 2008 Oct;6 Suppl 1:116-22.
9. Wonke B, Jensen C, Hanslip JJ, Prescott E, Lalloz M, Layton M, et al. Genetic and acquired predisposing factors and treatment of osteoporosis in thalassemia major. J Pediatr Endocrinol Metab 1998;11 Suppl 3:795-801.
10. Garofalo F, Piga A, Lala R, Chiabotto S, Di Stefano M, Isaia GC. Bone metabolism in thalassemia. Ann NY Acad Sci 1998;850:475-478.
11. Mahachoklertwattana P, Chuansumrit A, Sirisriro R, Choubtum L, Sriphrapradang A, Rajatanavin R. Bone mineral density, biochemical and hormonal profiles in suboptimally treated children and adolescents with beta thalassemia disease. Clin Endocrinol (Oxf) 2003;58(3):273-9.
12. Specker BL, Schoenau E. Quantitative bone analysis in children: current methods and recommendations. J Pediatr 2005;146(6):726-31.
13. Jensen CE, Tuck SM, Agnew JE, Koneru S, Morris RW, Yardumian A, et al. High prevalence of low bone mass in thalassaemia major. Br J Haematol 1998;103(4):911-5.
14. Lasco A, Morabito N, Gaudio A, Buemi M, Wasniewska M, Frisina N. Effects of hormonal replacement therapy on bone metabolism in young adults with beta thalassemia major. Osteoporosis Int 2001;12(7):570-5.
15. Yazigi A, Malouf G, Inati-Khoriati A, Tamin H, Saab C. Bone mineral density in beta thalassemic Lebanese children. J Musculoskelet Neuronal Interact 2002;2(5):463-8.
16. Kidson-Gerber GL, Francis S, Lindeman R. Management and clinical outcomes of transfusion dependent thalassemia major in an Australian tertiary referral clinic. Med J Aust 2008;188(2):72-5.
17. Pratelli L, Verri E, Fortini M, Marconi S, Zolezzi C, Fornasari PM, et al. Chelation therapy and bone metabolism markers in thalassemia major. J Pediatr Endocrinol Metab 2006;19(11):1335-42.
18. Eren E, Yılmaz N, Biochemical markers of bone turnover and bone mineral density in patients with beta thalassemia major. Int J Clin Pract 2005;59(1):46-51.
19. Shamshirsaz AA, Bekheirnia MR, Kamgar M, Pakbaz Z, Tabatabaie SM, Bouzari N, et al. Bone mineral density in Iranian adolescents and young adults with beta thalassemia major. Pediatr Hematol Oncol 2007;24(7): 469-79.
20. Benigno V, Bartelloni S, Baroncelli GI, Bertacca L, Di Peri S. Effects of thalassemia major on bone mineral density in late adolescence. J Pediatr Endocrinol Metab 2003;16 Suppl 2:337-42.