A study of growth pattern in regularly transfused thalassaemic children of age group of 2 years to 12 years

Authors

  • Shriharsha Badiger Department of Paediatrics, SDM Medical College, Dharwad, Karnataka, India
  • Aditi Baruah Department of Paediatrics, AMCH, Dibrugarh, Assam, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20192758

Keywords:

Growth, Thalassemia, Transfusion

Abstract

Background: ß-thalassemia is an autosomal recessive single gene disorder. Physical growth failure is one of the most important complications of thalassemia. Very few data regarding growth pattern of thalassemic children is available from India especially North East part. Keeping this in mind, present study was undertaken. To study the growth pattern of transfusion dependent thalassaemic children and to compare growth pattern between regularly and irregularly transfused children.

Methods: A cross-sectional observational study was done on 38 thalassaemic patients (aged 2 years-12 years) who attended Department of Pediatrics, Assam Medical College Dibrugarh. History, physical examination and investigations were done and filled in predesigned proforma.  Anthropometric measurements like weight and height were taken from all patients. Sexual maturity rating was done in girls ≥10 years and boys ≥11 years. Lab parameters included pre-transfusion hemoglobin (Hb), serum ferritin, LFT, RFT, Thyroid profile. Percentile for weight, height and body mass index were calculated using WHO (2007) reference data. Collected data were compared with age and sex matched normal children.

Results: About 34.21% transfusion dependent children had under-nutrition and 50% had stunting. 42% had thinness. Stunting was more in irregularly transfused children (81.25%) as compared to regularly transfused children which was highly significant (p<0.001). Under-nutrition among irregularly transfused children was more (40%) compared to regularly transfused children (28.57%). Pubertal spurt was delayed in 66% children. Those who had Hb <5 gm/dl had 100% stunting and under-nutrition.

Conclusions: Regular blood transfusion with growth monitoring and appropriate iron chelation (Sr. Ferritin >1000 ng/ml) is of utmost importance in transfusion dependent thalassaemic children.

References

Sarnaik SA. Thalassemia and related hemoglobinopathies. Indian J Paediatric. 2005;72(4):319-24.

Kumar V. Disease of red cells and bleeding disorders. In: Robbins textbook of pathology basis of disease. Cotran RS, Kumar V, Robbins SL. 8th ed. India: WB Saunders; 2010: 583-627.

Weintrob BN, Olivieri NF, Tyler B, Andrews DF, Freedman MH, Holland FJ. Effect of age at the start of iron chelation therapy on gonadal function in β-thalassemia major. N Engl J Med. 1990;323(11):713-9.

Skordis N. The multifactorial origin of growth failure in Thalassaemia. Pediatr Endocrinol Rev. 2011;8:271-7.

Agarwal DK, Agarwal KN, Upadhaya SK. Physical sexual growth of affluent Indian children from 5-18 years. Indian Pediatr. 1992;29(10):1203-84.

Kattamis A, Uakopoulou C. Growth and development in children with thalassaemia major. Acta Paedia Scandinavia. 1990;366:111-7.

Pignatti BC, Stefano P, Zonta L, Vullo C, Sanctis V, Melevendi C, et al. Growth and sexual maturation in thalassaemia major. J Paediatr. 1985;106(1):150-5.

Oerter KE, Kamp GA, Munson PJ, Nienhuis AW, Cassorla FG, Manasco PK. Multiple hormone deficiencies in children with hemochromatosis. The J Clin Endocrinol Metab. 1993;76(2):357-61.

Spiliotis BE. β-Thalassemia and normal growth: are they compatible?. Euro J Endocrinol. 1998;139(2):143-4.

Lokeshwar MR, Manglani M, Shaw N, Swathi Kanakia S. Modern trends in the management of Thalassemia. Indian J Pract Pediatr. 1997;5:287-96.

Choudhry VP, Desai N, Pati HP, Nanu A. Current Management of homozygous ß Thalassemia. Indian Paediatr. 1991;28(10):1221-9.

WHO. Child growth standards. Available at: http://www.who.int/childgrowth/standards/en/. Accessed 2007.

Kliegman R, Cromer B. Adolescent medicine, Nelson Textbook of Pediatrics. 19th ed.

Gombe S. Comparative efficacy of desferrioxamine, deferiprone and in combination on iron chelation in thalassemic children. J Indian Pediatrics. 2006;43:503-5.

Pemde HK, Chandra J, Gupta D, Singh V, Sharma R, Dutta AK. Physical growth in children with transfusion-dependent thalassaemia. Dove Press J Pediatr Health Med Therapeut. 2011:2;13-9.

Singhal B, Shama N, Mathur R. Iron overload and growth of thalassemic patients in marwar region. Int J Pharmaceut Sci Res. 2012;3(7):2043.

Hashemi AS, Ghilian R, Golestan M, Ghalibaf AM, Zare Z, Dehghani MA. The study of growth in thalassaemic patients and its correlation with serum ferritin level. Iranian J Pediatr Hematol Oncol. 2011;1(4):147-51.

Baker N, Alnakashabandi A, Alsaqy AH, Alrabaty A. Growth pattern and sexual maturation rate in β-thalassemia major patients from thalassemia center Erbil. Iraqi Acad Sci J. 2013;12(1):40-4.

Yesilipek MA, Bircan I, Oygür N, Ertug H, Yegin O, Güven AG. Growth and sexual maturation in children with thalassemia major. Haematol. 1993;78(1):30-3.

Saxena A. Growth retardation in thalassemia major patients. Int J Human Gen. 2003;3(4):237-46.

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Published

2019-06-27

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Original Research Articles