Le Goff L, Hadjadj E, Denis D
Service d'ophtalmologie, C.H.U. Nord Marseille, Chemin des Bourrely, 13015 Marseille, France.
J Fr Ophtalmol. 2002 Apr;25(4):388-92.
The authors report a case of de novo duplication 1q32-qter present in a patient with dysmorphic syndrome and developmental delay. This article describes the eighth case of partial trisomy 1q32-qter "pure", without chromosomal abnormalities. In the literature, a dysmorphic "syndrome" is described for trisomy 1q32-qter: hypertelorism, low set and malformed ears, prominent forehead, long philtrum, antimongoloid slanting, foot and digital malformations, cardiovascular abnormalities, urogenital abnormalities, and mental retardation. The ocular defects described in previously reported cases include: cataract, strabismus, hypoplasia of the optic disk, microphthalmia, epicanthal folds, ptosis, persistent tunica vasculosa lentis, and hyaloid vessels, but this seems to be nonspecific of this chromosomal abnormality.
作者报告了一例患有畸形综合征和发育迟缓的患者出现新发1q32-qter重复的病例。本文描述了第八例“单纯”1q32-qter部分三体病例,无染色体异常。文献中描述了1q32-qter三体的一种畸形“综合征”:眼距过宽、耳朵低位且畸形、前额突出、人中长、反蒙古样斜眼、足部和手指畸形、心血管异常、泌尿生殖系统异常以及智力发育迟缓。先前报道病例中描述的眼部缺陷包括:白内障、斜视、视盘发育不全、小眼症、内眦赘皮、上睑下垂、晶状体血管膜持续存在以及玻璃体血管,但这似乎并非这种染色体异常所特有的。