Brandt C A, Hertz J M, Petersen M B, Vogel F, Noer H, Mikkelsen M
Institute of Human Genetics, University of Aarhus, Denmark.
J Med Genet. 1992 Oct;29(10):704-8. doi: 10.1136/jmg.29.10.704.
A stillborn male child with anencephaly and multiple malformations was found to have the karyotype 46,XY,r(13) (p11q21.1). The breakpoint at 13q21.1, determined by high resolution banding, is the most proximal breakpoint ever reported in patients with ring chromosome 13. In situ hybridisation with the probe L1.26 confirmed the derivation from chromosome 13 and DNA polymorphism analysis showed maternal origin of the ring chromosome. Our results, together with a review of previous reports of cases with ring chromosome 13 with identified breakpoints, could neither support the theory of distinct clinical syndromes based on different breakpoints on 13q nor correlate the severity of symptoms with instability of the ring.
一名患有无脑畸形和多种畸形的死产男婴,其核型为46,XY,r(13)(p11q21.1)。通过高分辨率显带确定的13q21.1处的断点,是环状染色体13患者中报道过的最靠近近端的断点。用探针L1.26进行原位杂交证实该环状染色体源自13号染色体,DNA多态性分析显示该环状染色体来自母亲。我们的结果,连同对先前报道的具有已确定断点的环状染色体13病例的回顾,既不支持基于13q上不同断点的不同临床综合征理论,也无法将症状的严重程度与环状染色体的不稳定性相关联。