Biochemical indices and radiological examination to evaluate bone health in children with β-thalassemia major

Madhu P. K.; Bhagwan B.

doi:10.18203/2349-3291.ijcp20190071

Authors

Madhu P. K. Department of Paediatrics, Karnataka Institute of Medical Sciences, Hubli, Karnataka, India
Bhagwan B. Department of Paediatrics, Karnataka Institute of Medical Sciences, Hubli, Karnataka, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20190071

Keywords:

Beta-thalassemia, Bone disease, Children, Stunting, Serum calcium, Vitamin D

Abstract

Background: β-Thalassemia major is a serious hematological problem requiring regular blood transfusions. In regularly transfused thalassemic patients, metabolic bone disease is an important cause of morbidity. Problems include bone pain, deformities, short stature, osteopenia/osteoporosis, rickets, osteomalacia, spinal deformities, nerve compression and fractures. This study was undertaken to evaluate the status of bone health in children with β-thalassemia major by selected biochemical indices and radiological examination.

Methods: Fifty children with β- thalassemia major were enrolled. Detailed history, examination and anthropometry were done. Serum calcium, phosphorus, magnesium, alkaline phosphatase, and vitamin D levels were measured. X-ray of skull, wrist, and knee were studied for radiological changes. Biochemical indices were analysed with appropriate statistical tests.

Results: Stunting and wasting was observed in 58% and 44% children respectively. Hypocalcaemia was seen in 22%, hyperphosphatemia in 56% and 24% children had raised alkaline phosphatase levels. Vitamin D levels were severely deficient in 12%, deficient in 50%, and insufficient in 38% of children. There was no significant difference between vitamin D levels of cases and controls. Radiologically almost all children had a range of osteopathy, like thinned out cortex (96%), medullary expansion (54%), loss of density (40%), diffuse osteoporosis (18%) and growth arrest lines were seen in 8% children.

Conclusions: Vitamin D abnormalities and radiological features suggestive of decreased bone mineralization are seen in all the children with β-thalassemia major treated with periodic blood transfusion at this institute. No biochemical predictors of bone disease in these children were identified.

Metrics

Metrics Loading ...

References

Olivieri NF, Nathan DG, MacMillan JH, Wayne AS, Liu PP, McGee A, et al. Survival in medically treated patients with homozygous beta-thalassemia. N Engl J Med. 1994;331(9):574-8.

Vichinsky EP. Changing patterns of thalassemia worldwide. Ann N Y Acad Sci. 2005;1054(1):18-24.

Madan N, Sharma S, Sood SK, Colah R, Bhatia LH. Frequency of beta-thalassemia trait and other hemoglobinopathies in northern and western India. Indian J Hum Genet. 2010;16(1):16-25.

Mahachoklertwattana P, Sirikulchayanonta V, Chuansumrit A, Karnsombat P, Choubtum L, Sriphrapradang A, et al. Bone histomorphometry in children and adolescents with beta-thalassemia disease: iron-associated focal osteomalacia. J Clin Endocrinol Metab. 2003;88(8):3966-72.

Singh K, Kumar R, Shukla A, Phadke SR, Agarwal S. Status of 25-hydroxyvitamin D deficiency and effect of vitamin D receptor gene polymorphisms on bone mineral density in thalassemia patients of North India. Hematology. 2012;17(5):291-6.

World Health Organization. Training Course on Child Growth Assessment. Geneva, WHO, 2008.

Malabanan A, Veronikis IE, Holick MF. Redefining vitamin D insufficiency. Lancet. 1998;351(9105):805-6.

Shamshirsaz AA, Bekheirnia MR, Kamgar M, Pourzahedgilani N, Bouzari N, Habibzadeh M, et al. Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran. BMC Endocr Disord. 2003;3(1):4.

Vogiatzi MG, Macklin EA, Trachtenberg FL, Fung EB, Cheung AM, Vichinsky E, et al. Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America. Br J Haematol. 2009;146(5):546-56.

Fahim FM, Saad K, Askar EA, Eldin EN, Thabet AF. Growth Parameters and Vitamin D status in Children with Thalassemia Major in Upper Egypt. Int J Hematol Oncol Stem Cell Res. 2013;7(4):10-4.

Hashemi AS, Ghilian R, Golestan M, Akhavan Ghalibaf M, Zare Z, Dehghani MA. The Study of Growth in Thalassemic Patients and its Correlation with Serum Ferritin Level. Iranian J Pediatric Hematol Oncol. 2011;1(4):147-51.

Isik P, Yarali N, Tavil B, Demirel F, Karacam GB, Sac RU, et al. Endocrinopathies in Turkish children with Beta thalassemia major: results from a single center study. Pediatr Hematol Oncol. 2014;31(7):607-15.

Van Schoor NM, Lips P. Worldwide vitamin D status. Best Pract Res Clin Endocrinol Metab. 2011;25(4):671-80.

Anuj S Modi, Poornima R.T, Jayaprakash Murthy D; Serum Calcium And Phosphorus levels in patients with β-thalassemia major: IJPBS. 2012;2(4):156-60.

Karim MF, Ismail M, Hasan AM, Shekhar HU. Hematological and biochemical status of Beta-thalassemia major patients in Bangladesh: A comparative analysis. Int J Hematol Oncol Stem Cell Res. 2016;10(1):7-12.

de Vernejoul MC, Girot R, Gueris J, Cancela L, Bang S, Bielakoff J, et al. Calcium phosphate metabolism and bone disease in patients with homozygous thalassemia. J Clin Endocrinol Metab. 1982;54(2):276-81.

Saffari F, Mahyar A, Jalilolgadr S. Endocrine and metabolic disorders in beta-thalassemiamajor patients. Caspian J Intern Med. 2012;3(3):466-72.

Salama OS, Al-Tonbary YA, Shahin RA, Eldeen OA. Unbalanced bone turnover in children with beta-thalassemia. Hematology. 2006;11(3):197-202.

Soliman A, Adel A, Wagdy M, Al Ali M, ElMulla N. Calcium homeostasis in 40 adolescents with beta-thalassemia major: a case-control study of the effects of intramuscular injection of a megadose of cholecalciferol. Pediatr Endocrinol Rev: PER. 2008;6(1):149-54.

Holick MF. The role of vitamin D for bone health and fracture prevention. Curr Osteoporos Rep. 2006;4(3):96-102.

Lips P, van Schoor NM. The effect of vitamin D on bone and osteoporosis. Best Pract Res Clin Endocrinol Metab. 2011;25(4):585-91.

Sultan S, Irfan SM, Ahmed SI. Biochemical Markers of Bone Turnover in Patients with beta-Thalassemia Major: A Single Center Study from Southern Pakistan. Adv Hematol. 2016;2016.

Meena MC, Hemal A, Satija M, Arora SK, Bano S. Comparison of Bone Mineral Density in Thalassemia Major Patients with Healthy Controls. Adv Hematol. 2015;2015.

Chan YL, Pang LM, Chik KW, Cheng JC, Li CK. Patterns of bone diseases in transfusion-dependent homozygous thalassaemia major: predominance of osteoporosis and desferrioxamine-induced bone dysplasia. Pediatr Radiol. 2002;32(7):492-7.

Sharma R, Anand R, Chandra J, Seth A, Pemde H, Singh V. Distal ulnar changes in children with thalassemia and deferiprone related arthropathy. Pediatr Blood Cancer. 2013;60(12):1957-62.

Dresner Pollack R, Rachmilewitz E, Blumenfeld A, Idelson M, Goldfarb AW. Bone mineral metabolism in adults with beta-thalassaemia major and intermedia. Br J Haematol. 2000;111(3):902-7.

Resnick D. Diagnosis of Bone and Joint Disorders. 4th ed, vol. 3. Philadelphia: Saunders. 2002. p. 2167-2176.

Abu Alhaija ES, Hattab FN, al-Omari MA. Cephalometric measurements and facial deformities in subjects with beta-thalassaemia major. Eur J Orthod. 2002;24(1):9-19.

Goni MH, Markussis V, Tolis G. Bone mineral content by single and dual photon absorptiometry in thalassaemic patients. In: Endocrine disorders in thalassaemic patients. Ando S, Brancatti C, editors. Berlin Heidelberg, NY: Springer. 1995. p. 47-81.

Giardina P J, Schneider R, Lesser M, Simmons B, Rodriguez A, Gertner J, et al. In Abnormal bone metabolism in thalassaemia. Ando et al. editors. Berlin: Springer. p. 39-46

Chan YL, Li CK, Pang LM, Chik KW. Desferrioxamine-induced long bone changes in thalassaemic patients - radiographic features, prevalence and relations with growth. Clin Radiol. 2000;55(8):610-4.