De Mattia D, Pettini P L, Sabato V, Rubini G, Laforgia A, Schettini F
Dipartimento di Biomedicina dell'Età Evolutiva, Università degli Studi, Bari.
Minerva Pediatr. 1996 Jan-Feb;48(1-2):11-20.
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.
本研究观察了60例年龄在6至26岁之间的重型地中海贫血患者（31例男性，29例女性），其中18例已行脾切除术，旨在评估口腔颌面改变，并将其与输血指标、血清铁蛋白水平、脾切除术及年龄相关联。为每位患者建立了血液学和口腔牙科学卡片，以记录以下医学和临床病史：血液学情况、龋齿和牙周疾病的预防、面部特征、口腔牙科学检查、正畸诊断、颅骨X线片和全景曲面断层片。普遍观察到口腔卫生差以及对预防知识的误解。所有患者均有龋损，但大多数患者从未看过牙医进行治疗。内脏颅骨的不协调生长，由骨髓增生导致颌骨及其牙槽突增大，产生了各种严重的错牙合阶段（安氏II类、深覆牙合、开牙合）、颌学改变、鼻窦透亮度降低和眶距增宽，呈现典型的东方人面容。错牙合和不良的口腔卫生状况导致边缘性牙龈炎的发生，主要局限于下前牙水平。仅3例患者口腔黏膜苍白且萎缩。在本次调查中，分别有30%和26%的受检病例报告有牙胚缺失和乳牙滞留，而无患者有多生牙。牙齿体积、位置和形状异常很少发生。仅2例患者描述有釉质发育不全。龋病的发生频率在数量和程度上差异很大。仅5例患者未显示任何龋损。在所有60名受试者中计算的恒牙龋病指数（DMF）为5.12±4.76。利用Spearman秩检验，恒牙列（DFM）和混合牙列（DFM+dmf）中患龋牙齿的数量与铁蛋白平均值、铁蛋白峰值、输血需求以及临床检查时患者的年龄相关。因此，在混合牙列中输血需求与龋病之间显示出显著的负相关。采用卡方检验评估脾切除和未脾切除患者中龋病指数（高于5）的不同频率。脾切除术仅表面上与龋病超过5颗的患者总数相关。相反，在未脾切除组中，龋病超过5颗的患者频率明显较低。实际上，脾切除组和未脾切除组中年龄相同的受试者的龋病总数几乎相同。因此，在年龄增加的地中海贫血患者中，脾切除术和较高的龋病发生率在统计学上更有可能出现，但二者并无相互关联。
