Saleh A, Stephen L X G
Department of Oral Medicine and Periodontology, University of the Western Cape, Cape Town, South Africa.
SADJ. 2008 Feb;63(1):028-31.
Fanconi's anaemia (FA) is a rare, inherited autosomal recessive genetic disease characterized by progressive bone marrow failure, congenital abnormalities, and high predisposition to malignancies. The incidence of the disease is unusually high in the Afrikaner population of South Africa with calculated heterozygote prevalence being approximately 1 in 77. This report describes the oral clinical and radiological findings in a 16 year old male with FA.
Medical and dental history was noted, the oral mucosa was examined and the teeth were screened for fillings and caries. Periodontal examination consisted of: measurement of probing depths, recession, mobility of teeth, furcation involvement, plaque index and gingival index. An orthopantomograph was taken.
There was a generalized black hyper-pigmentation on the buccal mucosa, tongue and palate. The patient had generalized gingival recession and most of the periodontal pockets were deeper than 5mm. Several teeth were mobile and the orthopantomograph showed loss of alveolar bone in the furcation areas of most posterior teeth. A diagnosis of severe generalized periodontitis associated with systemic disease was made.
Patients with Fanconi's anaemia are susceptible to severe dental and periodontal diseases.
范可尼贫血（FA）是一种罕见的常染色体隐性遗传性疾病，其特征为进行性骨髓衰竭、先天性异常以及患恶性肿瘤的高度易感性。在南非的阿非利卡人群体中，该疾病的发病率异常高，据计算杂合子患病率约为1/77。本报告描述了一名16岁患范可尼贫血男性的口腔临床和放射学检查结果。
记录患者的病史和牙科病史，检查口腔黏膜，并筛查牙齿的充填物和龋齿情况。牙周检查包括：测量探诊深度、牙龈退缩、牙齿松动度、根分叉病变、菌斑指数和牙龈指数。拍摄曲面体层片。
颊黏膜、舌和腭部出现全身性黑色色素沉着。患者存在全身性牙龈退缩，大多数牙周袋深度超过5mm。几颗牙齿松动，曲面体层片显示大多数后牙的根分叉区域牙槽骨吸收。诊断为与全身性疾病相关的重度广泛性牙周炎。
范可尼贫血患者易患严重的牙齿和牙周疾病。
