MANIFESTATIONS OF BETA THALASSEMIA IN THE CRANIOFACIAL AREA

Abstract

Beta thalassemia (thalassemic syndrome) belongs to the group of hereditary hemoglobinopathies, the main pathogenic factor of which is inhibition of beta globin purposes. The disease is accompanied by severe anemia, active but not effective erythropoiesis, excessive bone marrow and extramedullary hematopoiesis activity, leading to significant changes in the skull bones, as in the literature, as reported in the literature. Hemoglobinopathies are actual problem in the contemporary medicine as the beta thalassemia is insufficiently studied and furthermore the dental aspects of these disease are need further development and investigation in terms of improving the diagnosis and appropriate treatment. The objective of this investigation was the studying the manifestations of beta thalassemia in craniofacial area. The clinical and radiological methods were used. We examined 45 patients with beta thalassemia major aged 5-15 years (25 boys and 20 girls). The close relationship and interdependence of diversity and severity complexes of clinical manifestations of beta thalassemia in craniofacial patients was revealed. The first feature that drew attention was the change in the appearance of the patients and characteristic symptom was the deformation of the skull, where due to hyperostosis, thickening of the bones of the skull increases the shape of the head by the type "tower" skull. In general, the entire configuration of face is modified, especially the middle zone with a lowered bridge of the nose, prominent cheekbones, and excessive development of the bones of the upper jaw. We also marked the distal occlusion, open bite and interdental intervals. There were also changes traced in the form of upper lip, as triangular shape, whereby the front teeth of the upper jaw was significantly exposed. Radiographic studies revealed hyperostosis and deformity of the skull bones what giving a peculiar form of increased head. There was also attention drown marked osteoporosis of the jaw bones, with background thinning and reduction of bone trabecular and resorption of interdental septa (Fig. 1). Discussion: Based on the results of the study there were revealed a significant defeat of maxillofacial region, the deformation of the skull and facial bones, which created a characteristic symptoms in the appearance of the beta thalassemia patients. These features have important diagnostic and prognostic value. The topicality of hemoglobinopathies, including beta thalassemia, the severity and variety of clinical and radiological manifestations stresses the importance of participation of dentists in the examination of these patients. All the knowledge of characteristic symptoms in crania-and maxillofacial region may contribute to early diagnosis and rational treatment of patients with beta thalassemia.