ORAL MANIFESTATIONS IN PATIENTS WITH SICKLE CELL DISEASE: a systematic review and meta-analysis
Sickle Cell Disease, oral manifestations, oral health.
Sickle cell disease (SCD) is one of the most prevalent hemoglobinopathies and results from the replacement of normal hemoglobin (Hb A) by hemoglobin S (HbS), in a homozygous or heterozygous state with other abnormal hemoglobins. The Hb S variant results from the substitution of glutamic acid for valine at position 6 of the β polypeptide chain. This alteration causes profound changes in the stability and solubility of the Hb molecule, favoring the sickling phenomenon and causing serious clinical consequences. The pathophysiology of the disease involves several complications, such as intense hemolytic anemia, and any tissue or organ can be affected. The same pathological effects of SCD on the connective tissues of different parts of the body – kidneys, liver, spleen, lungs and heart – have occurred in dental tissues and in the oral cavity in general. In this context, the objective of this systematic review was to assess the prevalence of oral manifestations found in patients with SCD. The electronic databases consulted were MEDLINE (via Pubmed), EMBASE, Web of Science, Latin American and Caribbean Literature on Health Sciences (LILACS). After analyzing a total of 15 included studies (n=3658), the presence of caries, malocclusion and gingival bleeding were the most reported oral alterations. The combined prevalence of caries was 0.69 (95% CI: 0.49-0.88), malocclusion 0.86 (95% CI: 0.43-1.28), and gingival bleeding 0.62 (95% CI: 0.40-0.84). Our results revealed the association between systemic pathophysiological aspects and oral changes, which impact the lives of SCD patients and, therefore, deserve special attention from professionals who care for oral health.