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

Abstract

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.1,2 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.5,6 Despite the maxillofacial alterations, SCD has little influence on oral health and no influence on the incidence of dental decay7–10 demonstrating that known risk factors for caries influence oral health more markedly than factors related to SCD.11,12 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