1/2. Associated orthodontic, surgical and hematological management of Cooley s anemia. Report of a case.Cooley s anaemia or b-thalassaemia or mediterranean anaemia is a blood disease characterised by malformation of the skull and long bones, which confers a typical appearance on the patient. The complete development of facial abnormalities can be prevented by an intensive blood transfusion programme or by bone-marrow-transplantation. At the present time these therapeutic strategies would be able to help these patients grow and develop, live a prolonged life and avoid bad surgical RESULTS. The aim of this study was to evaluate the feasibility of orthodontic and maxillofacial surgical corrective treatment associated with an appropriate transfusion therapy in a b-thalassemic patient. The patient enrolled in the study was affected by major b-thalassemia and diagnosis was performed as an infant. She was referred to our centre at puberty for dento-maxillofacial disorders. Clinical, haematological and radiographic evaluation permitted a complete diagnosis. She received a combined haematological, orthodontic and maxillofacial surgical treatment. Controls for evaluating the statement of results obtained were performed at different times after the end of the therapy and have shown that the therapeutic objectives had been achieved and maintained. At the present time, complete diagnostic and therapeutic haematological strategies cannot be carried out on a large scale, especially in countries where health resources are limited. This objective reason, associated with possible low patient compliance, explains why we still encounter severe facial deformities resulting from erythroid hyperplasia. Our results suggest that this facial disfigurement requires surgical and orthodontic correction by consolidated surgical-orthodontic techniques performed according to the appropriate anaemia therapy. Although this is a preliminary study, initial encouraging results show no relapse three years after the end of the therapy.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
2/2. Homozygous beta thalassemia in an African-American pediatric patient.Homozygous beta-thalassemia in an African-American pediatric patient is rare. In homozygous, beta-thalassemia there are characteristic changes due to severe chronic hemolytic anemia. Orofacial characteristics of homozygous beta-thalassemia result from expansion of bone marrow causing skull and facial deformities. This expansion causes clinically recognizable maxillary hyperplasia, severe protrusion of the middle third of the face, and anterior displacement of the incisors producing a typical faces historically referred to as "Cooley's face." The orofacial characteristics of an African American pediatric patient are described in detail.- - - - - - - - - - ranking = 7keywords = thalassemia (Clic here for more details about this article) |