A Tertiary Hospital Based Clinical Study on Thalassemia Patients in Relation to Onset of Blood Transfusions
Authors/Creators
- 1. Assistant Professor of Pediatrics, Viswabharathi Medical College, R T Nagar, Penchikalapadu, Kurnool, Andhra Pradesh
Description
Background: Among the hemoglobin disorders Thalassemia is considered as the frequently encountered disorder all over the world. Studies on clinical spectrum are varying. Aim of the study: To record the population prevalence and spectrum of thalassemia diseases. The objectives were to observe the epidemiological and social factors of this disease. Materials: Prospective and descriptive study for two years in a tertiary care Hospital included 71 Thalassemia children between 02 to 15 years. Interview with formulated questionnaire proforma consisting of social and demographic variables was used. Children given blood transfusion 08 years after and 08 years before diagnosis were grouped as A &B. Variables used: BMI, Hb, MCV, MCH and RDW-CV levels, Hb chromatogram: (HbA2 and Hb F levels) and spleen size. Results: Among 71 children 44 (61.97%) males and 27 (38.02%) were female. 77.46% of the total children belonged to below 5 years to 11 years. The mean age was 07.32±2.85 years. The mean age of diagnosis of Thalassemia was 2.36±2.05 years. 60.56% children were from rural background and 39.43% from urban. 22.53% children belonged to upper class of social status, 49.29% belonged to middle class and 20 (28.16%) of children belonged to lower social class. Conclusion: The incidence of Thalassemia was in concurrence with the national figures. >75% children were diagnosed before 11 years. Commencement of blood transfusions was not significant as the BMI and hematology profiles were same in all the children.
Abstract (English)
Background: Among the hemoglobin disorders Thalassemia is considered as the frequently encountered disorder all over the world. Studies on clinical spectrum are varying. Aim of the study: To record the population prevalence and spectrum of thalassemia diseases. The objectives were to observe the epidemiological and social factors of this disease. Materials: Prospective and descriptive study for two years in a tertiary care Hospital included 71 Thalassemia children between 02 to 15 years. Interview with formulated questionnaire proforma consisting of social and demographic variables was used. Children given blood transfusion 08 years after and 08 years before diagnosis were grouped as A &B. Variables used: BMI, Hb, MCV, MCH and RDW-CV levels, Hb chromatogram: (HbA2 and Hb F levels) and spleen size. Results: Among 71 children 44 (61.97%) males and 27 (38.02%) were female. 77.46% of the total children belonged to below 5 years to 11 years. The mean age was 07.32±2.85 years. The mean age of diagnosis of Thalassemia was 2.36±2.05 years. 60.56% children were from rural background and 39.43% from urban. 22.53% children belonged to upper class of social status, 49.29% belonged to middle class and 20 (28.16%) of children belonged to lower social class. Conclusion: The incidence of Thalassemia was in concurrence with the national figures. >75% children were diagnosed before 11 years. Commencement of blood transfusions was not significant as the BMI and hematology profiles were same in all the children.
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IJPCR,Vol14,Issue6,Article68.pdf
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Additional details
Dates
- Accepted
-
2022-06-15
Software
- Repository URL
- https://impactfactor.org/PDF/IJPCR/14/IJPCR,Vol14,Issue6,Article68.pdf
- Development Status
- Active
References
- 1. AE Olufemi, OB Sola, BE Oluwaseyi, RA Ajani, MO Olusoji, HR Olubunmi. Hemoglobin F level in different hemoglobin variants," Korean J. Hematol., 2011:46 (2): 1-5. 2. R Galanello, R Origa. Beta thalassemia," Orphanet J. Rare Dis., 2010: 1-15. 3. R Galanello, A Cao. "Alphathalassemia," Genet. Med., 2011:13: 2. 4. HA Niaki, RY Kamangari, A Banihashemi, VK Oskooei, M Azizi. Hematologic Features of Alpha Thalassemia Carriers," Inernational J. Mol. Celullar Med., 2012:1 (3): 162- 167. 5. SL Thein. The emerging role of fetal hemoglobin induction in nontransfusion-dependent thalassemia, Blood Rev., 2012:16 (1): 535-539. 6. A Prakash R Aggarwal. Thalassemia Major in Adults: Short Stature, Hyperpigmentation, Inadequate Chelation, and Transfusion - Transmitted Infections are Key Features, N. Am. J. Med. Sci., 2012:4 (3): 141-144. 7. P Thongkijpreecha, O Kangsadalampai, B Pongtanakul, KMeksawan. Nutritional Status in Patients with Thalassemia Intermedia, J. Hematol. Transfus Med., 2011:21: 167-76. 8. GS Kuczmarski R, Ogden CL. CDC Growth Charts for the United States: Methods and Development.:2000: 2010. 9. WHO. Public health Appropriate body-mass index for Asian populations and its implications for policy and intervention strategies, 2004:363: 157-163. 10. PJ Ho, GW Hall, LY Luo, DJ Weatherall, SL Thein. Betathalassaemia intermedia: is it possible consistently to predict phenotype from genotype? Br. J. Haematol., 1998:100 (1): 70-78. 11. S Ali, S Jahan (2016) "Growth Failure in β-Thalassemia major Patients Undergoing Repeated Transfusions," Jiimc, 11 (3): 120- 125. 12. G Badfar, MP Nasirkandy, M Shohani. Prevalence of Short Stature, Underweight and Delayed Puberty in Iranian Patients with Thalassemia Major: A Systematic Review and Meta-Analysis, Iran J. Ped. Hematol. Oncol., 2017:7 (4): 245- 259. 13. R Origa et al. Letters To the editor: Current growth patterns in children and adolescents with thalassemia major, Blood, 2016:128 (21): 2580- 2582. 14. DJ Weatherall JB Clegg. The Thalassaemia Syndromes, 4th ed. Blackwell Science. 2001. 15. WHO. Public health Appropriate body-mass index for Asian populations and its implications for policy and intervention strategies, 2004: 363: 157-163? 16. H Kaur, AK Bhalla, A Trehan, RK Pilania, N Hansdak, R Kaur. BMI of transfusion dependent beta-thalassemia children of North-Western Indian Origin, Human Biology Review, 2018: 7 (1): 61-70. 17. Mallik S, Chatterjee C, Mandal PK, Sardar JC, Ghosh P and Manna N. Expenditure to treat thalassemia: An experience at a tertiary care hospital in India. Iran J Public Health. 2010;39(1):78-84. 18. Riewpaiboon A, Nuchpravoon I, Torcharus K, Indaratna K, Thavorncharoensap M and Ubol B. Economic burden of beta-thalassemia /Hb E and beta-thalassemia major in Thai children. BMC Res Notes. 2010; 3:29 http://www.biomedcentral.com/1756- 0500/3/29 (accepted on January 30, 2010). 19. GM Clarke, TN Higgins. Laboratory Investigation of Hemoglobinopathies and Thalassemias: Review and Update, Clin Chem., 46 2000:(8): 1284-1290. 20. Colah R, Gorakshakor A, Nadkarni A. Global burden, distribution and prevention of beta-thalassemia and hemoglobin E disorders. Expert Rev Hematol. 2010;3(1):103-117. 21. Galanello R and Origa R. Review: Beta-thalassemia. http://www.ojrd.com/content/5/1/11, published – (Accessed on 21 May, 2010). 22. Ansari SH, Shamsi TS, Ashraf M. Molecular epidemiology of betathalassemia in Pakistan: Far reaching implications. Indian J Hum Genet. 2012;18(2):193-197. 23. Khan WA. Thalassemia in Bangladesh. Dhaka Shishu (Child) Hospital Journal. 1999; 15:42-44. 24. Mannan A, Kawser J, Ahmed AMA, Sikder OF, Islam MJ, Chowdhury MA. A Demographic Approch for Understanding the Prevalence of beta Thalassemia Patterns and Other Hemoglobinopathies: Selective Study in Chittagong City Perspective. Asian J Biol Sci. 2013. 25. Khan WA, Banu B, Amin SK. Prevalence of beta-thalassemia trait and Hb-E trait in Bangladesh school children and health burden of thalassemia in our population. DhakaShishu (Child) Hospital Journal. 2005; 21:1101-1107. 26. Prasomsuk S, Jetsrisuparp A, Ratanasiri T, Ratanasiri A et al. Lived experiences of mothers caring for children with thalassaemia major in Thailand. JSPN. 2007;12(1):13-23. 27. Ho PJ, Wickramasinghe SN, Rees DC, Lee MJ, Eden A, Thein SL 1997. Erythroblastic inclusions in dominantly inherited β thalassaemias. Blood 89: 322–328 28. Das R. Micromapping the frequencies of beta-thalassemia and sickle cell anemia in India: Away forward to plan control strategies. Indian J Hum Genet. 2012 ;18(2):148-149. 29. Musallam KM, Sankaran VG, Cappellini MD, Duca L, Nathan DG, Taher AT 2012. Fetal hemoglobin levels and morbidity in untransfused patients with β-thalassemia intermedia. Blood 119: 364–367. 30. Berthelot, M., Rieker, A., & Correia, J. C. The difficulties experienced by patients with low back pain in France: a mixed methods study. Journal of Medical Research and Health Sciences, 2022:5(6), 2039–2048. 31. Uddin MK, Aziz MA, Bhuya MF, Dipta TF, Begum M, Yunus ABM et al. Electrophoretic pattern of hereditary haemoglobin disorders in Bangladesh. Medicine Today. 2009;21(2):58-60. 32. Qumruzzaman and Salahuddin. Association between the education and thalassemia- A statistical study. Pak J Stat Oper Res. 2006;11(2):103-110. 33. Bandyopadhyay B, Nandi S, Mitra K, Mandal PK, Mukhopadhayay S, Biswas AB. Comparative study on perceptions and practices among parents of thalassemic children attending two different institutions. Ind Medica. 2003;28(3) (copyright @ 2005 Indomedica). 34. Sattari MR, Shoykhi D, Nikanfur A, Pourfeizi AH, Nanari M, Dolatkhah R et al. The finantial and social impact of thalassemia and 1st treatment in Iran. Pharma Sci. 2012;18(3):171-176. 35. Arif F, Fayyaz J, Hamid A. Awareness among parents of children with thalassemia major. JPMA. 2008;58(11):621-624. 36. Jagdish PG, Palak TH, Harsha G. Awareness among parents of children with thalassemia major from Western India. Int J Med Sci Public Health. 2015;4(10):1356-1359. 37. Rahaman S, Batool S, Qadir R. Socio economic status impact thalassemia child on families of Faisalabad district. Pak J of Appl Sci.2002;2(2):202-205. 38. Uddin MM, Akhteruzzan S, Rahman T, Hasan AKM and Shekhar HU. Pattern of beta-thalassemia and other hemoglobinopathis: A cross- sectional study studied in Bangladesh. ISRN Hematol. 2012. 39. Vichinky EP. Changing pattern of thalassemia worldwide. Ann NY Acad Sci. 2005; 1054:18-24. 40. Viprakasit V, Gattermann N and Cappellini MD. Geographical variations in current clinical practice on transfusion and iron chelation therapy across various transfusiondependent anemia. Blood Transfus. 2013;11(1):108-122. 41. Samarakoon PS and Nijesuriya AP. Thalassemia in Srilanka: Implications for future health of Asian populationSrilanka Thalassemia Study Group. Lancet. 2000; 355:786-791. 42. Ward A, Caro JJ, Green TC, Huybrechts K, Arama A, Wait S et al. An international survey of patients with thalassemia major and their views about sustaining life-long desferrioxamine use. BMC Clin Pharmacol. 2002; 2:3.