Sickle cell anemia oral manifestations in a Venezuelan population

Sickle cell anemia (SCA) is a well known hemoglobinopathy which results from a substitution of amino acids in the polypeptidic chain. SCA was considered endemic in certain areas of the world. It has been recognized now that it may have a wide geographic distribution. Few studies have dealt with dental manifestations or complications of SCA (Cox and Soni, 1984). Nevertheless none of them have showed epidemiological data for a large series of oral manifestations. To date, no epidemiological data of our country is available in the literature. The aim of this study was to determine the oral manifestations of SCA in a Venezuelan population. Seventeen patients affected were examined at the University Hospital and the Dental Clinic. Age ranged between 1 1/2-48 years. Each patient was haematologically diagnosed by hemoglobin electrophoresis and only homozygous individuals were selected. Each patient was analyzed according to general clinical history, as well as, dental history; clinical and radiological examination using periapical, panorex and bite-wings radiographs. Our results showed that the most affected group was between 20 to 30 years (41.18%). According to sex, females were more affected than males (64.71%). The most common phenotype was mestizo (47.31%). The most frequent type of hemoglobinopathy was Hg-SS and Hg SS-F. The most common soft tissue oral manifestation was buccal mucosa pallor in 77.05%. In addition, the hard tissue findings involved enlarged medullary spaces (70.58%). Cicatritial infarcts were present in 77.05% of cases and the step-ladder effect was demonstrated in 82.35% of cases. Our observations could be due to genetic, environmental, nutritional and geographical factors.