Abstract
Objective: In patients with thalassemia, factors such as low socioeconomic status, rapid erythrocyte turnover, endocrinopathy, chronic hypoxia, increased nutritional demands, multiple transfusions, and ineffective erythropoiesis may lead to malnutrition. This study aimed to investigate malnutrition and related factors in children with transfusion-dependent beta-thalassemia major (β-TM).
Material and Methods: The research group consisted of the medical records of 81 β-TM patients. Data were created by measuring the patients height (cm), body weight (kg), weight for age, mid-upper arm circumference (MUAC, cm), triceps skinfold thickness (TSF, mm) and blood parameters (e.g., hemoglobin, ferritin) before transfusion.
Results: The median age of children with β-TM was 8.8 (range, 4.6-13.0) years, 38 (47%) were male and 43 (53%) were female. The frequency of malnutrition among the children was 20.99% (4.94% moderate, 16.05% mild). The median age of those with malnutrition was higher (p=0.003) and the frequency of malnutrition was higher in children with β-TM aged 5 years and over (p=0.034). Children with and without malnutrition were similar in terms of body weight (p=0.074), MUAC (p=0.321), MUAC z-score (p=0.573), TSF (p=0.691), TSF z-score (p=0.846), TSF percentile (p=0.077), ferritin (p=0.945), vitamin B12 (p=0.119), 25-OH Vit D (p=0.995), and hemoglobin (p=0.563). Body mass index (p=0.026) and weight for age (p<0.001) were lower and albumin was higher (p=0.041) in children with malnourishment.
Conclusion: Malnutrition in children with transfusion-dependent β-TM is still a common clinical picture that needs to be tackled and prevented.
Keywords: Beta-thalassemia child, child nutrition disorders, ferritins malnutrition, iron chelating agents
References
- Betts M, Flight PA, Paramore LC, Tian L, Milenković D, Sheth S. Systematic Literature Review of the Burden of Disease and Treatment for Transfusion-dependent β-Thalassemia. Clin Ther 2020;42:322-37.e2. https://doi.org/10.1016/j.clinthera.2019.12.003
- Carsote M, Vasiliu C, Trandafir AI, Albu SE, Dumitrascu M-C, Popa A, et al. New entity-thalassemic endocrine disease: major beta-thalassemia and endocrine involvement. Diagnostics 2022;12:1921. https://doi.org/10.3390/diagnostics12081921
- Taher AT, Weatherall DJ, Cappellini MD. Thalassaemia. The Lancet 2018;391:155-67. https://doi.org/10.1016/S0140-6736(17)31822-6
- De Sanctis V, Soliman AT, Elsefdy H, Soliman N, Bedair E, Fiscina B, et al. Bone disease in β thalassemia patients: past, present and future perspectives. Metabolism 2018;80:66-79. https://doi.org/10.1016/j.metabol.2017.09.012
- De Sanctis V, Kattamis C, Canatan D, Soliman AT, Elsedfy H, Karimi M, et al. β-thalassemia distribution in the old world: an ancient disease seen from a historical standpoint. Mediterr J Hematol Infect Dis 2017;9:e2017018. https://doi.org/10.4084/mjhid.2017.018
- Soliman AT, Yassin M, Alyafei F, Alaaraj N, Hamed N, Osman S, et al. Nutritional studies in patients with β-thalassemia major: A short review. Acta Biomed 2023;94:e2023187.
- Canatan D. Thalassemias and hemoglobinopathies in Türkiye. Hemoglobin 2014;38:305-7. https://doi.org/10.3109/03630269.2014.938163
- Fung EB, Sushrita N, Haines D, Schroepfer C, Lal A. Dietary intake insufficient to support nutritional adequacy in patients with thalassemia. Blood 2014;124:1361. https://doi.org/10.1182/blood.V124.21.1361.1361
- Ayukarningsih Y, Amalia J, Nurfarhah G. Thalassemia And Nutritional Status In Children. J Health Dent Sci 2022;2:39-52. https://doi.org/10.54052/jhds.v2n1.p39-52
- Sherief LM, El-Salam A, Sanaa M, Kamal NM, Almalky MA, Azab SF, et al. Nutritional biomarkers in children and adolescents with Beta-thalassemia-major: An Egyptian center experience. Biomed Res Int 2014;2014:261761. https://doi.org/10.1155/2014/261761
- Training Course on Child Growth Assessment, WHO Child Growth Standards, World Health Organization. Available from: https://apps.who.int/iris/bitstream/handle/10665/43601/9789241595070_JobaidWeighing_eng.pdf?sequence=11&isAllowed=y, access date:15.08.2023.
- Dasgupta A, Butt A, Saha TK, Basu G, Chattopadhyay A, Mukherjee A. Assessment of Malnutrition Among Adolescents:Can BMI be Replaced by MUAC. Indian J Community Med 2010;35:276-9. https://doi.org/10.4103/0970-0218.66892
- WHO. WHO child growth standards: length/height-for-age, weight-for-age, weight-for-length, weight-for-height and body mass index-for-age: methods and development. World Health Organization, 2006.
- Ozturk A, Budak N, Cicek B, Mazicioglu MM, Bayram F, Kurtoglu S. Cross-sectional reference values for mid-upper arm circumference, triceps skinfold thickness and arm fat area of Turkish children and adolescents. Int J Food Sci Nutr 2009;60:267-81. https://doi.org/10.1080/09637480701663854
- Elalfy MS, Ebeid FSE, El Gendy YG, Zaki MM, Kalifa ASA. Body Composition in Egyptian Children With Transfusion-dependent Thalassemia: The Impact of Nutrition and Metabolic Profile. J Pediatr Hematol Oncol 2020;42:e334-e9. https://doi.org/10.1097/MPH.0000000000001712
- Qaisar I, Rehman A, Jillani KS. Assessment of Nutritional Status of Children with Beta Thalassemia Major with BMI. National Editorial Advisory Board 2020;31:107.
- Lidoriki I, Stavrou G, Fotis L, Kapelouzou A, Kokkota S, Tsaousi G, et al. Nutritional Status of β-Thalassemia major patients. 2022; 14(8):e27985. https://doi.org/10.21203/rs.3.rs-1516088/v1
- Pemde HK, Chandra J, Gupta D, Singh V, Sharma R, Dutta AK. Physical growth in children with transfusion-dependent thalassemia. Pediatric Health Med Ther 2011;2:13-9. https://doi.org/10.2147/PHMT.S15305
- Trehan A, Sharma N, Das R, Bansal D, Marwaha RK. Clinicoinvestigational and Demographic Profile of Children with Thalassemia Major. Indian J Hematol Blood Transfus 2015;31:121-6. https://doi.org/10.1007/s12288-014-0388-y
- Mirhosseini NZ, Shahar S, Ghayour-Mobarhan M, Kamaruddin NA, Banihashem A, Yusoff NaM, et al. Factors affecting nutritional status among pediatric patients with transfusion-dependent beta thalassemia. Med J Nutrition Metab 2013;6:45-51. https://doi.org/10.1007/s12349-012-0112-0
- Fahim FM, Saad K, Askar EA, Eldin EN, Thabet AF. Growth Parameters and Vitamin D status in Children with Thalassemia Major in Upper Egypt. Int J Hematol Oncol Stem Cell Res 2013;7:10-4.
- Biswas B, Naskar NN, Basu K, Dasgupta A, Basu R, Paul B. Malnutrition, Its Attributes, and Impact on Quality of Life: An Epidemiological Study among β-Thalassemia Major Children. Korean J Fam Med 2021;42:66-72. https://doi.org/10.4082/kjfm.19.0066
- 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;11:262-5.
- Mahmoud RA, Khodeary A, Farhan MS. Detection of endocrine disorders in young children with multi-transfused thalassemia major. Ital J Pediatr 2021;47:165. https://doi.org/10.1186/s13052-021-01116-2
- Dey P, Konwar G, Sarkar B. Body mass index in thalassemia children. J Evol Med Dent Sci 2019;8:1537-40. https://doi.org/10.14260/jemds/2019/341
- Behera S, Dixit S, Bulliyya G, Kar SK. Fat-soluble antioxidant vitamins, iron overload and chronic malnutrition in β-thalassemia major. Indian J Pediatr 2014;81:270-4. https://doi.org/10.1007/s12098-013-1162-0
- Harish G, Pasha SJ. Correlation of serum ferritin levels with liver function tests and anthropometric measurements in transfusion dependent beta-thalassemia major children: a cross sectional study. Pediatr Oncall J 2019;16:101-4. https://doi.org/10.7199/ped.oncall.2019.33
- Deng L, Mo M-Q, Zhong J, Li Z, Li G, Liang Y. Iron overload induces islet β cell ferroptosis by activating ASK1/P-P38/CHOP signaling pathway. PeerJ 2023;11:e15206. https://doi.org/10.7717/peerj.15206
- Koohi F, Kazemi T, Miri-Moghaddam E. Cardiac complications and iron overload in beta thalassemia major patients-a systematic review and meta-analysis. Ann Hematol 2019;98:1323-31. https://doi.org/10.1007/s00277-019-03618-w
- Yousefian S, Aliabad GM, Saleh R, Khedmati M. Association of Body mass index and serum ferritin level in pediatrics with Beta-thalassemia major disease. Iran J Pediatr Hematol Oncol 2022;12:34-40.https://doi.org/10.18502/ijpho.v12i1.8359
- Isik P, Yarali N, Tavil B, Demirel F, Karacam GB, Sac RU, et al. Endocrinopathies in Turkish children with Beta thalassemia major: results from a single center study. Pediatr Hematol Oncol 2014;31:607-15.https://doi.org/10.3109/08880018.2014.898724
- Moiz B, Habib A, Sawani S, Raheem A, Hasan B, Gangwani M. Anthropometric measurements in children having transfusion-dependent beta thalassemia. Hematology 2018;23:248-52. https://doi.org/10.1080/10245332.2017.1396044
- Altincik A, Akin M. Prevalence of Endocrinopathies in Turkish Children With β-Thalassemia Major: A Single-Center Study. J Pediatr Hematol Oncol 2016;38:389-93. https://doi.org/10.1097/MPH.0000000000000573
- Jin LW, Ain MTN, Li SC, Chun GY. The Impact of Chelation Compliance in Health Outcome and Health-Related Quality of Life in Thalassaemia Patients: A Systematic Review. Health Qual Life Outcomes 2024;22:14. https://doi.org/10.1186/s12955-023-02221-y
- Fung EB. Nutritional deficiencies in patients with thalassemia. Ann N Y Acad Sci 2010;1202:188-96.https://doi.org/10.1111/j.1749-6632.2010.05578.x
- Manolopoulos P, Lavranos G, Mamais I, Angouridis A, Giannakou K, Johnson E. Vitamin D and bone health status in beta thalassemia patients-systematic review. Osteoporos Int 2021;32:1031-40. https://doi.org/10.1007/s00198-021-05821-w
- Fung EB, Xu Y, Trachtenberg F, Odame I, Kwiatkowski JL, Neufeld EJ, et al. Inadequate Dietary Intake in Patients with Thalassemia. J Acad Nutr Diet 2012;112:980-90. https://doi.org/10.1016/j.jand.2012.01.017
- Fung EB. The importance of nutrition for health in patients with transfusion‐dependent thalassemia. Ann N Y Acad Sci 2016;1368:40-8. https://doi.org/10.1111/nyas.13003
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