Spinner N B, Lucas J N, Poggensee M, Jacquette M, Schneider A
Albert Einstein Medical Center, Department of Pediatrics, Philadelphia, Pennsylvania.
Am J Med Genet. 1993 Mar 1;45(5):609-13. doi: 10.1002/ajmg.1320450519.
We have studied an infant with multiple anomalies and a 46,XY,12p+ karyotype. Parental chromosomes were normal, and it was not possible to determine the identity of the extra material on chromosome 12 cytogenetically. Chromosome painting with probes from a chromosome 9 library identified this material as coming from chromosome 9, and cytogenetics established the duplication as 9q34-->qter. Comparison of this patient with others reported with partial dup(9q) documented excellent concordance of minor anomalies, most notably dolichocephaly, "deep-set" eyes, short horizontal palpebral fissures, beaked nose, micrognathia, arachnodactyly, and developmental delay. Identification of cytogenetically indeterminate abnormalities by molecular cytogenetics is very important, as it permits prognosis to be offered for families of newborn infants with unbalanced karyotypes.
我们研究了一名患有多种异常且核型为46,XY,12p+的婴儿。父母的染色体正常,通过细胞遗传学方法无法确定12号染色体上额外物质的来源。用来自9号染色体文库的探针进行染色体描绘,确定该物质来自9号染色体,细胞遗传学确定重复片段为9q34→qter。将该患者与其他报道的部分9q重复患者进行比较,发现轻微异常高度一致,最显著的是长头畸形、“深陷”眼、水平睑裂短、钩鼻、小颌畸形、蜘蛛指以及发育迟缓。通过分子细胞遗传学鉴定细胞遗传学上无法确定的异常非常重要,因为这可以为核型不平衡的新生儿家庭提供预后信息。