Eden M S, Thelin J W, Michalski K, Mitchell J A
Clin Genet. 1985 Nov;28(5):375-84. doi: 10.1111/j.1399-0004.1985.tb02210.x.
This report describes an adult male with a partial trisomy 6p(p211-pter) and a partial monosomy 9p(9p24-pter) resulting from a de novo unbalanced translocation. This patient does not show the classical featured of the 9p partial monosomy syndrome, thus disputing the claim of Hoo et al. (1982) that 9p24 is the critical segment for the monosomy syndrome. Partial trisomy for 6p has only been previously reported in children. In addition to the chromosomal anomalies, the patient has autosomal recessive spinal muscular atrophy with a different age of onset than two affected sibs. Finally, he shows unusual audiologic and ophthalmologic signs nor previously reported as part of the 9p monosomy or 6p trisomy syndromes.
本报告描述了一名成年男性,其因新发的不平衡易位导致6号染色体短臂部分三体(p211 - pter)和9号染色体短臂部分单体(9p24 - pter)。该患者未表现出9号染色体短臂部分单体综合征的典型特征,因此对胡等人(1982年)关于9p24是单体综合征关键片段的说法提出了质疑。6号染色体短臂部分三体此前仅在儿童中报道过。除了染色体异常外,该患者患有常染色体隐性遗传性脊髓性肌萎缩症,其发病年龄与两名患病同胞不同。最后,他还表现出一些不寻常的听力学和眼科学体征,这些体征此前并未作为9号染色体单体或6号染色体三体综合征的一部分被报道过。