Antich J, Plaza J, Geán E
An Esp Pediatr. 1981 Nov;15(5):469-73.
A male infant with a ring chromosome identified by R, D and G banding techniques is reported. Karyotype was 46, XY,r(13)(p12q22). The main clinical features were severe mental retardation, microcephaly, frontal bossing a peculiar Greek profile, microphtalmia, coloboma, high and narrowed palate, low-set ears and genital anomalies. The three groups in the 13q deletion syndrome proposed by Niebuhr and Ottosen (1973) are commented. These groups are based on clinical features and loss of segment in the long arms of chromosome 13. Our patient has many of the clinical features of the first group.
本文报道了一名通过R、D和G显带技术鉴定出具有环状染色体的男婴。核型为46, XY,r(13)(p12q22)。主要临床特征为严重智力发育迟缓、小头畸形、额部隆起(特殊的希腊人面容)、小眼症、缺损、高而窄的腭、低位耳和生殖器异常。对Niebuhr和Ottosen(1973年)提出的13q缺失综合征的三组情况进行了评论。这些组是基于临床特征和13号染色体长臂节段的缺失。我们的患者具有第一组的许多临床特征。