Assessment of growth retardation among transfusion-dependent Thalassemia patients in Peshawar, Pakistan
Article Sidebar
Main Article Content
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.
Article Details
This work is licensed under a Creative Commons Attribution 4.0 International License.
Work published in KMUJ is licensed under a
Creative Commons Attribution 4.0 License
Authors are permitted and encouraged to post their work online
(e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
Funding data
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