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Revista Brasileira de Hematologia e Hemoterapia

versão impressa ISSN 1516-8484versão On-line ISSN 1806-0870

Rev. Bras. Hematol. Hemoter. vol.38 no.2 São Paulo abr./jun. 2016 


Comment on: "Oral health-related quality of life in children and teens with sickle cell disease"

Maria Elvira Pizzigatti Correa1  *  

1Universidade Estadual de Campinas (UNICAMP), Campinas, SP, Brazil

Sickle cell disease (SCD) is an inherited red blood cell disorder, characterized by chronic hemolysis, vaso-occlusive complications and progressive multi-organ damage, with major impact on the patients' life expectancy and quality of life.1and2 The incidence of SCD is estimated as more than 300 000 new cases worldwide per year.3 The estimated incidence of SCD in Brazil is 3500 new cases per year.4

SCD presents important maxillofacial features. Hypoxia related to SCD has been associated with osteomyelitis of the jaws, particularly the mandible. Neuropathies of the mental nerve due to osteomyelitis of the mandible cause numbness in the lower lip and chin. Additionally, a diminished blood supply to teeth may cause necrosis of the dental pulp. Bone marrow hyperplasia may result in depression of the nasal bridge, midfacial overgrowth and malocclusion in this patient population. SCD has been associated with moderate to very severe malocclusion, with anterior tooth loss, anterior sparing, overjet, anterior crossbite and open bite.5and6

Despite the maxillofacial alterations, SCD has little influence on oral health and no influence on the incidence of dental decay7,8,9and10 demonstrating that known risk factors for caries influence oral health more markedly than factors related to SCD.11and12

On the other hand, in SCD, alterations related to dental occlusion have a strong influence on the quality of life of many patients. In a study of SCD involving 35 five-year-old children and 36 adolescents of both genders aged between 12 and 18 years, the prevalence of malocclusion in the preschool children was 62.9%. The main malocclusions observed in this age group were Class II (37.1%), increased overjet (28.6%), reduced overbite (28.6%), and open bite (17.1%). All 12- to 18-year-old subjects had malocclusion, with the most prevalent types of malocclusion being maxillary overjet (63.9%) and maxillary misalignment (58.3%). It is noteworthy that the majority of adolescents (80.6%) had very severe or disabling malocclusions.13

In the current issue of the Revista Brasileira de Hematologia e Hemoterapia, Fernandes et al. present a study in which they investigated the influence of SCD, socioeconomic characteristics, and oral conditions on oral health-related quality of life (OHRQoL) of children and teens. The study demonstrated no significant difference in the negative impact on OHRQoL between SCD patients and the healthy control group. However, there was a greater negative impact due to malocclusion in adolescents with SCD compared to healthy controls.

This result has also been observed in other patient populations. It was shown that children and adolescents expect that orthodontic treatment will improve their dental appearance and quality of life.14 According to Thiruventadam et al., children who sought orthodontic treatment had lower quality of life scores than those who never had or never sought orthodontic treatment.15 The same observation was found by Kragt et al., who demonstrated a clear inverse association of malocclusion with patients' OHRQoL. These authors also showed the strength of the association was dependent on the age of the children and their cultural environment.16

According to the authors, the aesthetic factors that had strong relationships with the need for orthodontic treatment were: (1) crooked, crowded, or spaced teeth, (2) worries when speaking or smiling, (3) breath smell and halitosis, and (4) the desire to use braces in order to be like other people or for fashionable reasons.17

There is no doubt about the maxillofacial alterations in SCD patients and the importance of orthodontic treatment for quality of life. These studies highlight the need for a better understanding of the role and involvement of all dental specialties on the comprehensive treatment of the SCD patient population.


1. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994;330(23):1639-44. [ Links ]

2. Panepinto JA, O'Mahar KM, DeBaun MR, Loberiza FR, Scott JP. Health- related quality of life in children with sickle cell disease: child and parent perception. Br J Haematol. 2005;130(3):437-44. [ Links ]

3. Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions. PLOS Med. 2013;10(7):e1001484. [ Links ]

4. Ministério da Saúde do Brasil, [Internet] Available from: Available from: [accessed 28.2.16]. [ Links ]

5. Costa CP, Carvalho HL, Souza Sde F, Thomaz EB. Is sickle cell anemia a risk factor for severe dental malocclusion? Braz Oral Res. 2015;29. [ Links ]

6. Dacosta OO, Kehinde MO, Ibidapo MO. Occlusal features of sickle cell anaemia patients in Lagos, Nigeria. Niger Postgrad Med J. 2005;12(2):121-4. [ Links ]

7. Ralstrom E, da Fonseca MA, Rhodes M, Amini H. The impact of sickle cell disease on oral health- related quality of life. Pediatr Dent. 2014;36(1):24-8. [ Links ]

8. Fernandes ML, Kawachi I, Corrêa- Faria P, Pattusi MP, Paiva SM, Pordeus IA. Caries prevalence and impact on oral health- related quality of life in children with sickle cell disease: cross-sectional study. BMC Oral Health. 2015; 15:68. [ Links ]

9. Al- Alawi H, Al- Jawad A, Al- Shayeb M, Al-Ali A, Al-Khalifa K. The association between dental and periodontal diseases and sickle cell disease. A pilot case-control study. Saudi Dent J. 2015;27(1):40-3. [ Links ]

10. Passos CP, Santos PR, Aguiar MC, Cangussu MC, Toralles MB, da Silva MC, et al. Sickle cell disease does not predispose to caries or periodontal disease. Spec Care Dent. 2012;32(2): 55-60. [ Links ]

11. Javed F, Correa FO, Nooh N, Almas K, Romanos GE, Al-Hezaimi K. Orofacial manifestations in patients with sickle cell disease. Am J Med Sci. 2013;345(3):234-7. [ Links ]

12. Laurence B, George D, Woods D, Shosanya A, Katz RV, Lanzkron S, et al. The association between sickle cell disease and dental caries in African Americans. Spec Care Dent . 2006;26(3):95-100. [ Links ]

13. Alves e Luna AC, Godoy F, de Menezes VA. Malocclusion and treatment need in children and adolescents with sickle cell disease. Angle Orthod. 2014;84(3):467-72. [ Links ]

14. Twigge E, Roberts RM, Jamieson L, Dreyer CW, Sampson WJ. The psycho-social impact of malocclusions and treatment expectations of adolescent orthodontic patients. Eur J Orthod. 2015, pii:cjv093. [ Links ]

15. Thiruvenkadam G, Asokan S, John JB, Geetha Priya PR, Prathiba J. Oral health- related quality of life of children seeking orthodontic treatment based on child oral health impact profile: a cross-sectional study. Contemp Clin Dent. 2015;6(3):396-400. [ Links ]

16. Kragt L, Dhamo B, Wolvius EB, Ongkosuwito EM. The impact of malocclusions on oral health- related quality of life in children - a systematic review and meta- analysis. Clin Oral Investig. 2015 [Epub ahead of print]. [ Links ]

17. Atisook P, Chuacharoen R. The relationship between demand and need for orthodontic treatment in high school students in Bangkok. J Med Assoc Thail. 2014;97(7):758-66. [ Links ]

See paper by Mazzola et al. in Rev Bras Hematol Hemoter. 2015;37(5):336-40

* E-mail address:

Conflicts of interest The author declares no conflicts of interest.

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