Abstract
Objective
To determine the prevalence of growth abnormality and endocrine dysfunction in a group of multi transfused thalassemic children and to correlate these with their body iron stores.
Methods
This cross sectional study included 35 Thalassemia Major patients, aged 13 to 24 years. Growth and puberty were assessed clinically and the laboratory values of various hormone levels were stratified with their age and sexual maturity.
Results
57.14% patients were short, 60% had not attained puberty, and 87.5% of the girls had primary amenorrhea. 14.29% had low FSH and 2.86% low LH levels. 89.47% of the boys had low free testosterone and 43.75% of the girls had low estradiol levels. While 20% had high TSH levels, 40% had high PTH levels, of which 92.8% had low levels of Vitamin D. Low levels of IGF-1 were noted in 51.43%.
Conclusions
In this study analysis, short stature and hypogonadism were frequent findings. These results support the need for vigilant clinical evaluation of growth and puberty, as well as appropriate hormonal evaluation in poly transfused thalassemic children in order to detect and treat endocrine dysfunction early. The authors also recommend aggressive and adequate chelation from early life so that permanent damage to the endocrine glands can be prevented.
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References
Cunningham MJ, Macklin EA, Neufeld EJ, Cohen AR. Complications of β-thalassemia major in North America. Blood. 2004;104:34–9.
Aydinok Y, Darcan S, Polat A, et al. Endocrine complications in patients with β-thalassemia major. J Trop Pediatr. 2002;48:50–4.
Lo L, Singer ST. Thalassemia: Current approach to an old disease. Pediatr Clin North Am. 2002;49:1165–91.
Borgna-Pignatti C, De Stefano P, Zonta L, et al. Growth and sexual maturation in thalassemia major. J Pediatr. 1985;106:150–5.
Low LC. Growth of children with beta-thalassemia major. Indian J Pediatr. 2005;72:159–64.
Kattamis C, Liakopoulou T, Kattamis A. Growth and development in children with thalassaemia major. Acta Paediatr Scand Suppl. 1990;366:111–7.
Low LC. Growth, puberty and endocrine function in beta-thalassaemia major. J Pediatr Endocrinol Metab. 1997;10:175–84.
George A, Bhaduri A, Sen S, Choudhry VP. Physical growth parameters in thalassemic children. Indian J Pediatr. 1997;64:861–71.
De Virgiliis S, Cossu P, Toccafondi C, et al. Effect of subcutaneous desferrioxamine on iron balance in young thalassemia major patients. Am J Pediatr Hematol Oncol. 1983;5:73–7.
Fargion S, Taddei MT, Gabutti V, et al. Early iron overload in beta-thalassaemia major: when to start chelation therapy? Arch Dis Child. 1982;57:929–33.
Wu KH, Tsai FJ, Peng CT. Growth hormone (GH) deficiency in patients with beta-thalassemia major and the efficacy of recombinant GH treatment. Ann Hematol. 2003;82:637–40.
Karydis I. Karagiorga-Lagana M, Nounopoulos C, Tolis G. Basal and stimulated levels of growth hormone, insulin-like growth factor-I (IGF-I), IGF-I binding and IGF-binding proteins in beta-thalassemia major. J Pediatr Endocrinol Metab. 2004;17:17–25.
Low LC, Postel-Vinay MC, Kwan EY, Cheung PT. Serum growth hormone (GH) binding protein, IGF-I and IGFBP-3 in patients with beta-thalassaemia major and the effect of GH treatment. Clin Endocrinol (Oxf). 1998;48:641–6.
Ghosh S, Bandyopadhyay SK, Bandyopadhyay R, Roy D, Maisnam I, Ghosh MK. A study on endocrine dysfunction in thalassaemia. J Indian Med Assoc. 2008;106:655–9.
Flynn DM, Hoffbrand AV, Politis D. Subcutaneous desferrioxamine: the effect of three years’ treatment on liver, iron, serum ferritin, and comments on echocardiography. Birth Defects Orig Artic Ser. 1982;18:347–53.
Agarwal MB, Shah S, Vishwanathan C, et al. Thyroid dysfunction in multi-transfused iron loaded thalassemia patients. Indian Pediatr. 1992;29:997–1002.
Hader A, Bashir N, Hasan Z, Khatib S. Thyroid function in children with beta-thalassemia major in north Jordan. J Trop Pediatr. 1993;39:107–10.
Cavallo L, Licci D, Acquafredda A, et al. Endocrine involvement in children with beta–thalassaemia major. Transverse and longitudinal studies. I. Pituitary–thyroidal axis function and its correlation with serum ferritin levels. Acta Endocrinol (Copenh). 1984;107:49–53.
Filosa A, Di Maio S, Aloj G, Acampora C. Longitudinal study on thyroid function in patients with thalassemia major. J Pediatr Endocrinol Metab. 2006;19:1397–404.
Gulati R, Bhatia V, Agarwal SS. Early onset of endocrine abnormalities in beta-thalassemia major in a developing country. J Pediatr Endocrinol Metab. 2000;13:651–6.
Italian Working Group on Endocrine Complications in Non–Endocrine Diseases. Multicentre study on prevalence of endocrine complications in thalassaemia major. Clin Endocrinol (Oxf). 1995;42:581–6.
Chern JP, Lin KH. Hypoparathyroidism in transfusion-dependent patients with beta-thalassemia. J Pediatr Hematol Oncol. 2002;24:291–3.
Srivatsa A, Marwaha RK, Muralidharan R. Trehan A. Assessment of Adrenal Endocrine Function in Asian Thalassemics: Indian Pediatrics. 2005;42:31–5.
Tyagi S, Kabra M, Tandon N, Saxena R, Pati H, Choudhry VP. linico haematological profile of thalassemia Intermedia patients. Int. J Hum Genet. 2003;3:251–8.
Jain M, Sinha RS, Chellani H, Anand NK. Assessment of thyroid functions and its role in body growth in thalassemia major. Indian Pediatr. 1995;32:213–9.
Agarwal MB. Deferasirox: oral, once daily iron chelator–an expert opinion. Indian J Pediatr. 2010;77:185–91.
Agarwal MB. Advances in management of thalassemia. Indian J Pediatr. 2009;76:177–84.
Choudhry VP, Naithani R. Current status of iron overload and chelation with deferasirox. Indian J Pediatr. 2007;74:759–64.
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RM was the principal investigator who conceptualized and designed the study. Retrieval of relevant studies, pooling of results and manuscript writing was done by AS and JA. RM will be guarantor of the study
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Merchant, R.H., Shirodkar, A. & Ahmed, J. Evaluation of Growth, Puberty and Endocrine Dysfunctions in Relation to Iron Overload in Multi Transfused Indian Thalassemia Patients. Indian J Pediatr 78, 679–683 (2011). https://doi.org/10.1007/s12098-010-0351-3
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DOI: https://doi.org/10.1007/s12098-010-0351-3