Gencík A, Brönniman U, Tobler R, Auf der Maur P
J Med Genet. 1983 Apr;20(2):107-11. doi: 10.1136/jmg.20.2.107.
Two children with monosomy 10p13 are reported. In the first case the monosomy was the result of a maternal balanced translocation t(3;10) (p27;p13) while the second case was a de novo mutation. We reviewed clinical details of cases reported so far and found that certain symptoms are typical of the deletion of a comparatively large segment of chromosome 10 short arms. These symptoms include mental and growth retardation, skull abnormalities, antimongoloid slant of the eyes, ear abnormalities, anteverted nostrils, abnormalities of the hands and feet, cryptorchidism in boys, and, primarily, hypoplasia or aplasia of the olfactory bulbs and olfactory tracts as well as narrow palpebral fissures or eyelid ptosis.
报告了两名患有10p13单体性的儿童。第一例中,单体性是由母亲的平衡易位t(3;10)(p27;p13)导致的,而第二例是新发突变。我们回顾了迄今为止报道病例的临床细节,发现某些症状是10号染色体短臂相对大片段缺失的典型表现。这些症状包括智力和生长发育迟缓、颅骨异常、眼的反蒙古样倾斜、耳部异常、鼻孔前倾、手足异常、男孩隐睾,以及主要的嗅球和嗅束发育不全或缺失,还有睑裂狭小或眼睑下垂。