title: [Oromaxillofacial changes in thalassemia major].
abstract: Sixty patients (31 male and 29 female) with thalassemia major, aged between 6 and 26 years, 18 of which were splenectomized, were observed in this study evaluating the oro-maxillo-facial alterations and correlating them to transfusion indexes, serum ferritin levels, splenectomy and age. For each patient a haematologic and odontostomatologic card was filed with a view to report the medical and clinical history regarding: the haematologic picture, the prevention of caries and parodontal disease, the facies characteristics, the odonto-stomatologic examination, the orthodontic diagnosis, the skull X-rays and the orthopantomography. Poor oral hygiene as well as misknowledge of prevention were generally observed. All the patients showed carious lesions but most of them had never seen a dentist for therapy. The disharmonious growth of splanchnocranium, with the enlargement of the jaw and of its alveolar process, induced by the bone marrow hyperplasia, produced various and serious malocclusion stages (Angle's II class, deep bite, open bite), gnathologic alterations, hypodiaphanous paranasal sinuses and orbital hypertelorism, with a typical oriental-like facies. Malocclusion and the poor oral hygienic conditions determined the occurrence of marginal gingivitis, mainly localized at the level of the lower frontal teeth. In only 3 patients the oral mucous membrane was pale and atrophic. During this investigation agenesia and dental retention were reported in 30% and in 26% of the examined cases respectively, while no patients had supernumerary teeth. Tooth volume, position and shape abnormalities rarely occurred. Only in two patients was enamel hypoplasia described. The caries frequency greatly varied in number and in degree. Only five patients did not show any carious lesions. The caries index (DMF) for the permanent teeth calculated in all the 60 subjects was 5, 12 +/- 4.76. By utilizing Spearman's rank test the number of teeth with caries in the permanent dentition (DFM) and in the mixed dentition (DFM + dmf) was correlated to the average value of ferritin, with the ferritin peak, with transfusion requirements and with the age of the patient at the date of the clinical examination. A significant inverse correlation was therefore demonstrated between transfusion requirements and caries in the mixed dentition. The chi 2 test was used to assess the different frequency of the caries index (above 5) between splenectomized and non-splenectomized patients. Splenectomy proved to be associated only apparently to the total number of patients with more than 5 caries. Conversely, in the non-splenectomized group the frequency of patients with more than 5 caries was definitely lower. Indeed the overall number of caries in both groups of splenectomized and non-splenectomized subjects, of the same average age, was almost identical. Consequently, splenectomy and the higher number of caries are statistically more probable in individuals affected by thalassemia, of increasing age, without however being mutually correlated.
authors.affiliation: Dipartimento di Biomedicina dell'Età Evolutiva, Università degli Studi, Bari.
authors.forename: D
authors.initials: D
authors.lastname: De Mattia
authors.forename: P L
authors.initials: PL
authors.lastname: Pettini
authors.forename: V
authors.initials: V
authors.lastname: Sabato
authors.forename: G
authors.initials: G
authors.lastname: Rubini
authors.forename: A
authors.initials: A
authors.lastname: Laforgia
authors.forename: F
authors.initials: F
authors.lastname: Schettini
mesh_terms: D000293:Adolescent; D000328:Adult; D001803:Blood Transfusion; D002648:Child; D003131:Combined Modality Therapy; D004245:DMF Index; D019066:Facies; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007571:Jaw Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D008297:Male; D009059:Mouth Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D013156:Splenectomy; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications* / Q000628:therapy
chemical_list: D005293:Ferritins
vernacular_title: Alterazioni oro-maxillo-facciali nella thalassemia major.