Neurocognitive Function and Its Related Potentials in Children with Beta Thalassemia Major: An Egyptian Study

  • Inas R. El-Alameey Child Health Department, Medical Research Division, National Research Centre, Egypt
  • Fatma Alzaree Child Health Department, Medical Research Division, National Research Centre, Egypt
  • Manal A. Shehata Child Health Department, Medical Research Division, National Research Centre, Egypt
  • Mones M. Abu Shady Child Health Department, Medical Research Division, National Research Centre, Egypt
  • Mohamed Abdel Atti Child Health Department, Medical Research Division, National Research Centre, Egypt
  • Mohamed I. El-Khonezy Molecular Biology Department, Genetic Engineering and Biotechnology Division, National Research Centre, Egypt
Keywords: β-Thalassemia, Neurocognitive, Children, Egyptian, Potentials

Abstract

BACKGROUND: Repeated blood transfusions and hemolysis in β-Thalassemia major children lead to iron overload in various organs, including the brain which may cause neurodegeneration.

AIM: To evaluate intelligence quotient in children with β-thalassemia major and healthy counterparts and to assess risk factors that cause cognitive problems.

SUBJECTS AND METHODS: This case-control study was performed on 50 children aged 6-16 years old with β-thalassemia major as patients group and compared with 50 healthy children as a control group of matched age, sex, and social class. Cognitive functions were evaluated by using the Wechsler Intelligence Scale for Children. Serum ferritin and iron were measured by ELISA.

RESULTS: There were significantly lower mean performance and full-scale IQ scores of patients group in comparison with controls, whereas no significant differences between both groups as regards to a verbal IQ score. In thalassemic children, block design, comprehension and arithmetic were negatively correlated with age of disease onset, duration of illness and onset of chelation therapy. Serum iron and ferritin were negatively correlated with similarities and digit span. Serum iron levels were negatively correlated with performance IQ score.

CONCLUSION: Children with β-thalassemia major need to receive more academic attention and cognitive assessment to improve their IQ.

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References

Galanello R, Origa R. Review: beta thalassemia. Orphanet J Rare Dis. 2010; 5:11. https://doi.org/10.1186/1750-1172-5-11 PMid:20492708 PMCid:PMC2893117

El-Beshlawy A, Kaddah N, Rageb L, et al. Thalassemia prevalence and status in Egypt. Pediatr. Res. 1999; 45:760-760. https://doi.org/10.1203/00006450-199905010-00132

Borgna-Pignatti C, Cappellini MD, De Stefano P et al. Survival and complications in thalassemia. Ann. N. Y. Acad. Sci. 2005; 1054:40–47. https://doi.org/10.1196/annals.1345.006 PMid:16339650

Taher A, Isma'eel H, Mehio G et al. Prevalence of thromboembolic events among 8,860 patients with thalassemia major and intermedia in the Mediterranean area and Iran. Thromb. Haemost. 2006; 96:488–91. https://doi.org/10.1160/TH06-05-0267

Fosburg M, Nathan D. Treatment of Cooley's anemia. Blood. 1990; 76:435-444. PMid:2198956

Modell B, Khan M, Darlison M. Survival in beta-thalassemia major in the UK: data from the UK Thalassaemia Register. Lancet. 2000; 355:2051-2052. https://doi.org/10.1016/S0140-6736(00)02357-6

Poggiali E, Cassinerio E, Zanaboni L, et al. An update on iron chelation therapy. Blood Transfus. 2012; 10:411–422. PMid:22790257 PMCid:PMC3496216

Zafeiriou DI, Economou M, Athanasiou-Metaxa M. Neurological complications in b-thalassemia. Brain Dev. 2006; 28:477-481. https://doi.org/10.1016/j.braindev.2006.02.005 PMid:16574362

Metafratzi Z, Argyropoulou MI, Kiortsis DN, et al. T(2) relaxation rate of basal ganglia and cortex in patients with beta-thalassemia major. Br J Radiol. 2001; 74:407–410. https://doi.org/10.1259/bjr.74.881.740407 PMid:11388988

Chen SH, Liang DC, Lin HC, et al. Auditory and visual toxicity during deferoxamine therapy in transfusion-dependent patients. J Pediatr Hematol Oncol. 2005; 27:651–653. https://doi.org/10.1097/01.mph.0000194019.95096.b6 PMid:16344669

Economou M, Zafeiriou D, Kontopoulos E, et al. Neurophysiologic and intellectual evaluation of beta thalassemia patients. Brain Dev. 2006; 28:14-18. https://doi.org/10.1016/j.braindev.2005.03.006 PMid:15925466

Duman O, Arayici S, Fettahoglu C, et al. Neurocognitive function in patients with b-thalassemia major. Pediatr Int. 2011; 53:519-523. https://doi.org/10.1111/j.1442-200X.2010.03279.x PMid:20964788

Tanner JM, Hiernaux J, Jarman S. Growth and physique studies, in Human Biology: A Guide to Field Methods, Weiner JS and Lourie JA, Eds., IBP. London Blackwell Publications, Oxford, UK, 1969:315–340.

Wechsler D. Wechsler Intelligence Scale for Children, Third Edition. San Antonio, TX: Psychological Corporation; 1991.

Zafeiriou DI, Prengler M, Gombakis N, Kouskouras K, Economou M, Kardoulas A, et al. Central nervous system abnormalities in asymptomatic young 1patients with Sbeta-thalassemia. Ann Neurol. 2004; 55(6):835-9. https://doi.org/10.1002/ana.20104 PMid:15174017

Vichinsky EP, Neumayr LD, Gold JI, Weiner MW, Rule RR, Truran D, et al. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2010; 303(18):1823-31. https://doi.org/10.1001/jama.2010.562 PMid:20460621 PMCid:PMC2892214

Rafaat N, El Safy, U, Khater N, HassanT, Hassan B, Siam A, Youssef A, El Shabrawy A: Assessment of Cognitive Function in Children With Beta-Thalassemia Major: A Cross-Sectional Study. Journal of Child Neurology 2015; 30 (4):417-422. https://doi.org/10.1177/0883073814550827 PMid:25296920

Ai Y, Zhao SR, Zhou G, et al. Hemoglobin status associate with Performance IQ but not Verbal IQ in Chinese pre-school children. Pediatr Int. 2012; 54:669-675. https://doi.org/10.1111/j.1442-200X.2012.03648.x PMid:22507306 PMCid:PMC3404215

Fadlyana E, Fathiyah Ma'ani, Monalisa Elizabeth, Lelani Reniarti: Correlation between Serum Ferritin Level and Growth Disorders in Children with Thalassemia. American Journal of Clinical Medicine Research. 2017; 5(3):31-35.

De P, Mistry R, Wright C, Pancham S, Burbridge W, Gangopadhayay K, et al. A review of endocrine disorder in thalassemia. Open J Endocr Met Dis. 2014; 4:25-34. https://doi.org/10.4236/ojemd.2014.42003

Moayeri H, Oloomi Z: Prevalence of growth and puberty failure with respect to growth hormone and gonadotropins secretion in beta-thalassemia major. Arch Iranian Med. 2006; 9(4):329-34. PMid:17061604

Hashemi G, Golestan H, Ghalibaf A, Zare D. The study of growth in thalassemic patients and its correlation with serum ferritin level. Iranian J Pediatr Hematol Oncol. 2011; 1(4):147-51.

Andayani SH, Sekarwana N, Fadil R. Association between age and serum ferritin level with bone age deficit in children with thalassemia major. Paediatr Indones. 2008; 48(1):33-6. https://doi.org/10.14238/pi48.1.2008.33-6

Majmoodi NH, and Farahanian E. Relation between bone mineral density and serum ferritin levels in patients with thalassemia major. Caspian J Pediatr. 2016; 2(2):158-63.

Monastero R, Monastero G, Ciaccio C, Padovani A, Camarda R. Cognitive deficits in beta-thalassemia major. Acta Neurol Scand. 2000; 102(3):162-8. https://doi.org/10.1034/j.1600-0404.2000.102003162.x PMid:10987375

Karimi M, Yarmohammadi H, Cappellini MD. Analysis of intelligence quotient in patients with homozygous beta-thalassemia. Saudi Med J. 2006; 27:982-985. PMid:16830015

Khairkar P, Malhotra S, Marwaha R. Growing up with the families of b-thalassaemia major using an accelerated longitudinal design. Indian J Med Res. 2010; 132:428-437. PMid:20966522

Homayouni SM, Seyednezhad SH, Meymandi MH et al. A Comparison of Intelligence Quotient in Children with and without β-Thalassemia Major. GMJ. 2015; 4(4):132-38.

Canatan D, Ratip S, Kaptan S, Cosan R. Psychosocial burden of beta thalassaemia major in Antalya, south Turkey. Soc Sci Med. 2003; 56(4):815-9. https://doi.org/10.1016/S0277-9536(02)00080-1

Shehata GA, Elsayh KI, Nafisa H. Rafaat NH, Mohamed AO, Rageh TA: Study of Beta-Thalassemia Biomarkers and Their Relationship to Cognition Among Children. Journal of Child Neurology 2010; 25(12) 1473-1479. https://doi.org/10.1177/0883073810368996 PMid:20519673

Published
2019-01-16
How to Cite
1.
El-Alameey IR, Alzaree F, Shehata MA, Abu Shady MM, Atti MA, El-Khonezy MI. Neurocognitive Function and Its Related Potentials in Children with Beta Thalassemia Major: An Egyptian Study. Open Access Maced J Med Sci [Internet]. 2019Jan.16 [cited 2021Jan.25];7(3):322-8. Available from: https://www.id-press.eu/mjms/article/view/oamjms.2019.077
Section
B - Clinical Sciences