Journal of South Asian Association of Pediatric Dentistry

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VOLUME 7 , ISSUE 1 ( January-April, 2024 ) > List of Articles

CASE REPORT

Evaluating the Concept of Oral Manifestation of Thalassemia Major and Its Dental Consideration: A Report on Two Cases and a Literature Review

Nidhi Yadav, Ajay Parihar, Prashanthi Reddy, Arun D Sharma

Keywords : Case report, Chipmunk facies, Dental consideration, Gingival hypertrophies, Salt and pepper appearance, Thalassemia major

Citation Information : Yadav N, Parihar A, Reddy P, Sharma AD. Evaluating the Concept of Oral Manifestation of Thalassemia Major and Its Dental Consideration: A Report on Two Cases and a Literature Review. J South Asian Assoc Pediatr Dent 2024; 7 (1):31-37.

DOI: 10.5005/jp-journals-10077-3292

License: CC BY-NC 4.0

Published Online: 27-04-2024

Copyright Statement:  Copyright © 2024; The Author(s).


Abstract

Background: Thalassemia is one of the most prevalent inherited diseases in the world, posing public health and societal challenges in high-risk locations. Thalassemia can be classified into two types—α-thalassemia and β-thalassemia, which are caused by abnormalities in the synthesis of hemoglobin (Hb) α- or β-polypeptide chains. Reduced synthesis of one of the two globin polypeptides leads to diminished Hb production, resulting in hypochromic microcytic anemia with erythrocyte dysplasia and destruction. The case reports presented and evaluated here show distinctive orofacial features, classic radiographic features, and dental considerations in thalassemia major patients. Conclusion: Thalassemia major causes several orofacial and systemic problems, which worsen with age. Early diagnosis and management result in a better prognosis and decreased morbidity. Clinical significance: Dental practitioners, particularly those working in multiracial populations, need to comprehend the disease's complications and therapy.


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  1. Vij R, Machado RF. Pulmonary complications of haemoglobinopathies. Chest 2010;138(4):973–983. DOI: 10.1378/chest.10-0317
  2. Wang Y, Yu-Fong Chang J, Wu Y, et al. Oral manifestations and blood profile in patients with thalassemia trait. J Formos Med Assoc 2013;112(12):761–765. DOI: 10.1016/j.jfma.2013.09.010
  3. Bajwa H, Basit H. Thalassemia. StatPearls. Treasure Island (FL): StatPearls Publishing; 2023.
  4. Kharsa MA. Orthodontic characteristics of thalassemia patients: Orthod Cyber Journal. 2008.
  5. Madhok S, Madhok S. Dental considerations in thalassaemic patients. J Dent Med Sci 2014;13(6):57–62. DOI: 10.9790/0853-13645762
  6. Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis 2010;5:11. DOI: 10.1186/1750-1172-5-11
  7. Tari K, ValizadehArdalan P, Abbaszadehdibavar M, et al. Thalassemia an update: Molecular basis, clinical features, and treatment. Int J Biomed Public Health 2018;1(1):48–58. DOI: 10.22631/IJBMPH.2018.56102
  8. Weatherall DJ. The inherited diseases of haemoglobin are an emerging global health burden. Blood 2010;115(22):4331–4336. DOI: 10.1182/blood-2010-01-251348
  9. Greenberg MS, Glick M. Burket's Oral Medicine: Diagnosis & Treatment, 10th edition. 2003
  10. Salehi MR, Farhud DD, Tohidast TZ, et al. Prevalence of orofacial complications in Iranian patients with ß-thalassemia major. Iran J Public Health 2007;36(2):43–46.
  11. Mariani R, Trombini P, Pozzi M, et al. Iron metabolism in thalassemia and sickle cell disease. Mediterr J Hematol Infect Dis 2009;1(1):e2009006. DOI: 10.4084/MJHID.2009.006
  12. Fung EB, Harmatz P, Milet M, et al. Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: a report from the multi-center study of iron overload. Am J Hematol 2007;82(4):255–265. DOI: 10.1002/ajh.20809
  13. Nemeth E. Hepcidin in beta-thalassemia. Ann N Y Acad Sci 2010;1202:31–35. DOI: 10.1111/j.1749-6632.2010.05585.x
  14. Hattab FN, Hazza'a AM, Yassin OM, et al. Caries risk in patients with thalassaemia major. Int Dent J 2001;51(1):35–38. DOI: 10.1002/j.1875-595x.2001.tb00815.x
  15. Ja'afar MN, Al-Aswad FD. Oro-facial manifestations, microbial study, and salivary enzyme analysis in patients with β-thalassemia major. J Bagh Coll Dent 2012;24(1):52–56.
  16. Helmi N, Bashir M, Shireen A, et al. Thalassemia review: features, dental considerations, and management. Elect Physic 2017;9(3):4003–4008. DOI: 10.19082/4003
  17. Lova AT, Chemiawan E, Dewi TS. Clinical appearance of oral mucous in children with β-major thalassemia. Padjadjaran J Dent 2011;23(2). DOI: 10.24198/pjd.vol23no2.14019
  18. Gujja P, Rosing DR, Tripodi DJ, et al. Iron overload cardiomyopathy: better understanding of an increasing disorder. J Am Coll Cardiol 2010;56(13):1001–1012. DOI: 10.1016/j.jacc.2010.03.083
  19. Goldfarb A, Nitzan DW, Marmary Y. Changes in the parotid salivary gland of /3-thalassemia patients due to hemdsiderin deposits. Int J Oral Surg 1983;12(2):115–119. DOI: 10.1016/s0300-9785(83)80007-6
  20. Langlais RP, Bricker SL, Cottone JA, et al. Oral Diagnosis, Oral Medicine and Treatment Planning. Philadelphia: WB Saunders Company; 1984.
  21. Van Dis ML, Langlais RP. The thalassemias: oral manifestations and complications. Oral Surg Oral Med Oral Pathol 1986;62(2):229–233. DOI: 10.1016/0030-4220(86)90055
  22. Bassimitci S, Yücel Eroglu E, Akalar M. Effects of thalassemia major on components of the craniofacial complex. Br J Orthod 1996;23(2):157–162. DOI: 10.1179/bjo.23.2.157
  23. Einy S, Hazan-Molina H, Ben-Barak A, et al. Orthodontic consideration in patients with beta-thalassemia major: case report and literature review. J Clin Pediatr Dent 2016;40(3):241–246. DOI: 10.17796/1053-4628-40.3.241
  24. Muhammad Ali DS, Mahmood Haider PDS. B thalassemia; a case report of oral and maxillofacial manifestation from population of Karachi Pakistan. Profess Med J 2017;24(2):352–356. DOI: 10.17957/TPMJ/17.3556
  25. Hattab FN. Thalassemia major and related dentomaxillofacial complications: clinical and radiographic overview with reference to dental care. Int J Exp Dent Sci 2017;6(2):95–104. DOI: 10.5005/jp-journals-10029-1163
  26. AlDallal S, AlKathemi M. Orodental considerations in thalassemia patients. J Hematol Blood Disord 2016;2(2):205. DOI: 10.15744/2455-7641.2.205
  27. Gaudio A, Morabito N, Catalano A, et al. Pathogenesis of thalassemia major-associated osteoporosis: a review with insights from clinical experience. J Clin Res Pediatr Endocrinol 2019;11(2):110–117. DOI: 10.4274/jcrpe.galenos.2018.2018.0074
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