Frappaz D, Bourgeois J, Berthier J C, Laurent C, Bethenod M
Pediatrie. 1983 Jun;38(4):261-70.
With one personal case and thirteen cases from literature about distal deletion of the long arm of chromosome 4 (4 q-), authors try to describe a clinical syndrome related to deletion of segment 4 q 31 leads to q ter. This syndrome includes a normal intrauterine growth, a growth and mental retardation. Morphological abnormalities consist in microcephaly, palato-cheiloschisis with micrognathia, hypertelorism with epicanthald folds, large nose bridge with anteverted nases, various anomalies of ears; clinodactily of Vth finger and toe, various cardiac defects. New banding techniques (prometaphase) have led to more precise delineation of break point, but this very distal deletion could not bring any new information for the gene mapping.
通过一个个人病例以及文献中13例关于4号染色体长臂远端缺失(4q-)的病例,作者试图描述一种与4q31至q末端片段缺失相关的临床综合征。该综合征包括正常的宫内生长、生长发育迟缓和智力发育迟缓。形态学异常包括小头畸形、伴有小颌畸形的腭裂唇裂、伴有内眦赘皮的眼距过宽、鼻梁宽大且鼻孔前倾、耳部各种异常;第五指和趾弯曲,各种心脏缺陷。新的显带技术(早中期)使得断点的描绘更加精确,但这种非常远端的缺失未能为基因定位带来任何新信息。