D'Emma C, Crippa L, Delozier C, Michail E, Graber P
J Genet Hum. 1982 Mar;30(1):39-50.
Partial trisomy 6q (6q23 leads to qter), resulting from a non-balanced translocation 6/18, was detected in a child and his paternal uncle. Features of note in the uncle included micrognathia, scoliosis and mild mental retardation. The child presented a moderate hypertelorism, a flat nose bridge, carp-shaped mouth and minimal cutaneous syndactyly of the 2nd-3rd toes. These clinical signs have also been mentioned in the few cases of partial trisomy 6q described in the literature. In addition, our two patients present a cubital deviation of the fingers and, in the uncle, urinary tract malformations. The transmission of the 6/18 translocation in this family is discussed.
在一名儿童及其叔伯中检测到因6号与18号染色体不平衡易位导致的6号染色体长臂部分三体(6q23至qter)。叔伯的显著特征包括小颌畸形、脊柱侧弯和轻度智力发育迟缓。该儿童表现为中度眼距增宽、鼻梁扁平、鲤鱼嘴以及第二和第三脚趾轻度皮肤并指。文献中描述的少数6号染色体长臂部分三体病例也提及了这些临床体征。此外,我们的两名患者均出现手指肘状偏斜,且叔伯存在泌尿系统畸形。本文讨论了该家族中6/18易位的遗传情况。