Schimmenti L A, Higgins R R, Mendelsohn N J, Casey T M, Steinberger J, Mammel M C, Wiesner G L
Department of Pediatrics, University of Minnesota, Minneapolis, USA.
Am J Med Genet. 1995 May 22;57(1):52-6. doi: 10.1002/ajmg.1320570112.
Partial deletion of the short arm of chromosome 9 (p24-->pter) and partial duplication of the long arm of chromosome 5 (q32-->qter) were observed in an abnormal boy who died at age 8 weeks of a complex cyanotic cardiac defect. He also had minor anomalies, sagittal craniosynostosis, triphalangeal thumbs, hypospadias, and a bifid scrotum. Two other infants with similar cytogenetic abnormalities were described previously. These patients had severe congenital heart defect, genitourinary anomalies, broad nasal bridge, low hairline, apparently low-set ears, short neck, and triphalangeal thumbs, in common with our patient. We suggest that combined monosomy 9p23,24-->pter and trisomy 5q31,32-->qter may constitute a clinically recognizable syndrome.
在一名8周大死于复杂性青紫型心脏缺陷的异常男婴中,观察到9号染色体短臂(p24→pter)部分缺失和5号染色体长臂(q32→qter)部分重复。他还存在轻微异常,矢状缝早闭、拇指三节指骨、尿道下裂和阴囊裂。之前还描述过另外两名具有类似细胞遗传学异常的婴儿。这些患者与我们的患者一样,患有严重先天性心脏缺陷、泌尿生殖系统异常、宽鼻梁、低发际线、明显低位耳、短颈和拇指三节指骨。我们认为,9号染色体p23、24→pter单体和5号染色体q31、32→qter三体合并可能构成一种临床可识别的综合征。