Assessment of growth retardation among transfusion-dependent Thalassemia patients in Peshawar, Pakistan
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Abstract
OBJECTIVE: To investigate the relationship between repeated blood transfusions and growth retardation in children with transfusion-dependent Thalassemia in Peshawar, Pakistan.
METHODS: This cross-sectional study was conducted on transfusion-dependent thalassemia patients receiving treatment at the Fatimid Foundation, Peshawar Pakistan, between February and August 2022. Participants were categorized by age (<1 year, 1–2 years, and >2 years) to assess and measure growth patterns over six months.
RESULTS: Out of 93 children with thalassemia major, 55 (59.1%) males and 38 (40.9%) females. Mean age of the participants was 10.86 ± 5.72 years, and mean age at their first transfusion was 8.13 ± 5.78 months. Mean body mass index was 16.38 ± 1.82 kg/m². Short stature was observed in 49 patients (52.7%), of whom 57.1% (n=28) were male and 42.9% (n=21) were female. Serum ferritin levels were significantly elevated in patients with short stature, with 57.1% (n=28/49) having ferritin levels >4001 μg/L compared to 40.9% (n=18/44) with normal stature. Only 3.2% of patients had normal ferritin levels. The ROC analysis identified a ferritin cut-off of 1636 μg/L for predicting short stature (sensitivity 86%, specificity 68%). Growth assessment showed that 71% of the children were <50th percentile. Hemoglobin levels and early transfusion age were also associated with short stature, highlighting the impact of iron overload on growth.
CONCLUSION: This study highlights the detrimental effects of thalassemia on growth in transfusion-dependent children, primarily from iron overload and high ferritin levels, emphasizing the importance of better management strategies to prevent complications and promote healthy development.
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References
1. Valentine WN, Neel JV. Hematologic and genetic study of the transmission of thalassemia:(cooley's anemia; mediterranean anemia). Arch Intern Med (Chic) 1944;74(3):185-96. https://doi.org/10.1001/ARCHINTE.1944.00210210032005
2. Bayanzay K, Alzoebie L. Reducing the iron burden and improving survival in transfusion-dependent thalassemia patients: current perspectives. J Blood Med 2016;7:159-69. https://doi.org/10.2147/JBM.S61540
3. Rathaur KV, Imran A, Pathania M. Growth pattern in thalassemic children and their correlation with serum ferritin. J Family Med Prim Care 2020;9(2):1166-9. https://doi.org/10.4103/jfmpc.jfmpc_951_19
4. Atmakusuma TD, Hasibuan FD, Purnamasari D. The correlation between iron overload and endocrine function in adult transfusion-dependent beta-thalassemia patients with growth retardation. J Blood Med 2021;12:749-53. https://doi.org/10.2147/JBM.S325096
5. Hamidah A, Arini MI, Zarina AL, Zulkifli SZ, Jamal R. Growth velocity in transfusion dependent prepubertal thalassemia patients: results from a thalassemia center in Malaysia. Southeast Asian J Trop Med Public Health 2008;39(5):900-5.
6. Al-Tayar AAK, Al-Zaazaai AAM. Growth pattern among Yemeni children suffering from Β thalassemia major in relation to serum ferritin the Yemeni Society for thalassemia and genetic blood disorder - Sana’a Yemen. J Endocrinol Diab 2019;6(3):1-7. https://doi.org/10.15226/2374-6890/6/3/001136
7. Wonke, B, De Sanctis V. Clinical aspects of transfusional iron overload. Rev Clin Exp Hematol 2000;4(4):322-36. https://doi.org/10.1046/j.1468-0734.2000.00023.x
8. Moiz B, Habib A, Sawani S, Raheem A, Bilal H, Gangwani M. Anthropometric measurements in children having transfusion-dependent beta thalassemia. Hematology 2018:23(4):248-52. https://doi.org/10.1080/10245332.2017.1396044
9. Shahid Z, Hassan S, Ghazanfar S, Kaneez M, Khan MS, Tariq HT, et al. Investigating the role of ferritin in determining sexual underdevelopment in beta-thalassemia major patients: a cross-sectional analysis from Pakistan. Cureus 2021:13(6):e15572. https://doi.org/10.7759/cureus.15572
10. Sheikh MA, Shakir MU, Shah M. The assessment of nutritional status of children with beta thalassemia major with body mass index. Pak J Med Health Sci 2017:11(1):262-5.
11. Sultan S, Irfan SM, Ahmed SI. Biochemical markers of bone turnover in patients with β‐thalassemia major: a single center study from Southern Pakistan. Adv Hematol 2016;2016:5437609. https://doi.org/10.1155/2016/5437609
12. Muhammad R, Shakeel M, Rehman S, Lodhi MA. Population-based genetic study of β-thalassemia mutations in Mardan Division, Khyber Pakhtunkhwa Province, Pakistan. Hemoglobin 2017;41(2):104-9. https://doi.org/10.1080/03630269.2017.1330210
13. Jaffri SA, Irfan SM, Sultan S, Usman SM, Nadeem S. Bone Mineral Density among adolescent's patients with β-thalassemia major. Int J Endorsing Health Sci Res; 2021; 9 (1):42-7. https://doi.org/10.29052/IJEHSR.v9.i1.2021.42-4
14. Beatrix W, Sanctis VD. Clinical aspects of transfusional iron overload. Rev Clin Exp Hematol 2000; 4(4):322-36. https://doi.org/10.1046/j.1468-0734.2000.00023.x
15. Wonke B, De Sanctis V. Clinical aspects of transfusional iron overload. Reviews in Clinical and Experimental Hematology. 2000 Dec;4(4):322-36. https://doi.org/10.1046/j.1468-0734.2000.00023.x
16. Yousefian S, Mirialiabad G, Saleh R, Khedmati M. Association of Body mass index and serum ferritin level in pediatrics with Beta-thalassemia major disease. Iranian J Pediatric Hematol Oncol 2022;12(1):34-40. https://doi.org/10.18502/ijpho.v12i1.8359
17. Shalitin, S, Carmi D, Weintrob M, Philip M, Miskin H, Kornreich L, et al. Serum ferritin level as a predictor of impaired growth and puberty in thalassemia major patients. Eur J Hematol 2005;74(2):93-100. https://doi.org/10.1111/j.1600-0609.2004.00371.x
18. Hatamleh MI, Chenna VSH, Contractor H, Krishna Mohan GV, Tirumandyam G, Dammas N, Khan MW, Hirani S. Efficacy of Hydroxyurea in Transfusion-Dependent Major β-Thalassemia Patients: A Meta-Analysis. Cureus. 2023 Apr 26;15(4):e38135. https://doi.org/10.7759/cureus.38135