Oral considerations in the management of sickle cell disease: a case report.

Introduction Sickle cell anaemia is a genetic disease caused by replacement of glutamic acid by valine in position 6 at the N-terminus of the beta-chain of globin, thus resulting in haemoglobin S [1-2]. Under conditions of hypoxia, erythrocytes that predominantly contain haemoglobin S take on a shape resembling a sickle [1-4]. This sickling is reversible through increased oxygen levels, although constant changes in shape result in cell membrane lesions that make the cells rigid, preventing them from returning to their normal state [5-6]. Sickle cell anaemia is diagnosed through a neonatal screening test, in which drops of blood are drawn from the newborn’s heel to permit haemoglobin electrophoresis or chromatography to be performed [7]. The reduction in oxygen-transport capacity results in circulatory difficulties, including vasoocclusive conditions, which diminishes the lifespan of the red blood cells to approximately 20 days [89]. In addition, sickled erythrocytes may occlude the microvasculature because they adhere better to the endothelium, thereby impeding the blood flow and causing tissue anoxia, necrosis and pain [10]. The main oral manifestations and complications of sickle cell disease are mucosal pallor, yellow tissue coloration, radiographic abnormalities, delayed tooth eruption, disorders of enamel and dentine mineralisation, changes to the superficial cells of the tongue, malocclusion, hypercementosis, and a degree of periodontitis that is unusual in children [9,11-14]. For dental treatment to be carried out, it is recommended that dental surgeons have an understanding of the pathophysiology of this disease, enabling them to determine treatment plans so that they can also take systemic conditions into consideration [15-17].