Ahn S-G, Lim Y-S, Kim D-K, Kim S-G, Lee S-H, Yoon J-H
Oral Biology Research Institute, College of Dentistry, Chosun University, Gwangju, South Korea.
Int J Oral Maxillofac Surg. 2004 Jul;33(5):458-62. doi: 10.1016/j.ijom.2003.11.001.
This article describes a pooled analysis of Korean individuals with nevoid basal cell carcinoma syndrome (NBCCS). The data upon which this review is based has been retrieved from published case reports in Korean dental and medical literature between the years 1981 to 2002. We found 33 subjects who met the diagnostic criteria for NBCCS. Relative frequencies of associated complications are presented and compared with those of the English literature. Odontogenic keratocyst (OKC) and palmar and/or plantar pits, and hypertelorism were the most frequently observed anomalies. OKCs are often the first signs of NBCCS and can be detected in patients younger than 20 years of age. However, the incidence and clinical manifestations of NBCCS in Korean individuals were found to be rather different from those of other countries. The relatively low frequency of basal cell carcinomas and falx calcification among the major criteria were two major differences. The frequencies of the minor criteria concur in general with the ranges given by some others. It is concluded that these differences may be attributed to genetic and geographic differences.
本文描述了一项对韩国痣样基底细胞癌综合征（NBCCS）患者的汇总分析。本综述所依据的数据取自1981年至2002年间韩国牙科和医学文献中发表的病例报告。我们发现33名符合NBCCS诊断标准的受试者。文中列出了相关并发症的相对频率，并与英文文献中的数据进行了比较。牙源性角化囊肿（OKC）、掌跖凹陷和眼距过宽是最常观察到的异常情况。OKC通常是NBCCS的首发症状，在20岁以下的患者中即可检测到。然而，韩国人NBCCS的发病率和临床表现与其他国家的情况存在相当大的差异。主要标准中基底细胞癌和大脑镰钙化的相对低发率是两个主要差异。次要标准的频率总体上与其他一些研究给出的范围一致。得出的结论是，这些差异可能归因于遗传和地理差异。
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