Oro-dental clinical aspects in children with thalassemia

摘要

Introduction . Thalassemias are the most common monogenic diseases. They are divided in two major categories: alpha – thalassemias (minor thalassemia), that do not have a special symptomatology and beta-thalassemias, which are a form of hereditary disease of hemoglobin (hemoglobinopathy), a severe form of anemia that requires treatment for life. In the case of major thalassemias, ketogenic analysis, the genetic defect occurs through deletions and point mutations of the gene that controls the synthesis of alpha or beta chains in messenger RNA at chromosome 11 level. It is an autosomal recessive disease. Homozygous patients with two beta-mutant genes have severe forms of diseases; heterozygous patients with a single beta-mutant allele have mild disease forms, but it is very important that they should be properly evaluated and diagnosed for genetic counseling. The objective was to describe dental abnormalities that occur in case of thalassemias. Materials and method . Two clinical cases of two patients aged 2 years and 12 years, diagnosed with beta-thalassemia and alpha-thalassemia, respectively, are presented. Results. The disruptions that occurred, have resulted in physiognomic dysfunction, masticatory dysfunction, for which patients have requested dental care. The main problem is the optimization of the masticatory function, that can be solved at the age of the investigated patients, and will also solve the phonation disorder, followed by the supervision of the growth process and the intervention with other treatment solutions after the closure of the process. Conclusions. The clinical picture of the patients in the study reproduces the data from the specialized literature: structural abnormalities causing severe evolving dental cavities. Regular follow-ups at the dental office in this case are very important but beneficial at the same time. In a proper feeding and nutritional state – against the background of severe chronic anemia, we need to avoid the onset of hemolytic crises caused by outbreaks of dental infection.