Müller-Navia J, Nebel A, Oehler D, Theile U, Zabel B, Schleiermacher E
Institut für Anthropologie, Johannes Gutenberg-Universität Mainz, Germany.
Prenat Diagn. 1996 Oct;16(10):915-22. doi: 10.1002/(SICI)1097-0223(199610)16:10<915::AID-PD966>3.0.CO;2-V.
Reverse chromosome painting has become a powerful tool in clinical genetics for the characterization of cytogenetically unclassifiable aberrations. In this report, the application of a sensitive and rapid procedure for the complete and precise identification of four different de novo structural chromosome abnormalities is presented. These chromosome rearrangements include a marker derived from chromosome 3(cen-q11), an interstitial deletion of chromosome 13 [del(13)(q14q22)], an unbalanced translocation [46,XY, -4, +der(4)t(4;8)(p 15.2;p21.1)] leading to Wolf-Hirschhorn syndrome, and a partial inverted duplication in conjunction with a partial deletion of chromosome 5p [46,XX, -5, +der(5)(:p13-p15.1::p15.1-qter)] which is responsible for the manifestation of the cri-du-chat syndrome. The importance of a fast and reliable evaluation of complex chromosome aberrations in pre- and postnatal diagnosis with regard to comprehensive genetic counselling is emphasized.
反向染色体描绘已成为临床遗传学中用于表征细胞遗传学上无法分类的畸变的有力工具。在本报告中,介绍了一种灵敏且快速的程序,用于完整、精确地鉴定四种不同的新发结构染色体异常。这些染色体重排包括一条源自3号染色体(cen-q11)的标记染色体、13号染色体的中间缺失[del(13)(q14q22)]、导致Wolf-Hirschhorn综合征的不平衡易位[46,XY, -4, +der(4)t(4;8)(p15.2;p21.1)]以及与5号染色体短臂部分缺失相关的部分反向重复[46,XX, -5, +der(5)(:p13-p15.1::p15.1-qter)],后者导致猫叫综合征的表现。强调了在产前和产后诊断中对复杂染色体畸变进行快速可靠评估对于全面遗传咨询的重要性。