Mankinen C B, Sears J W, Alvarez V R
Birth Defects Orig Artic Ser. 1976;12(5):131-6.
A 3-year-old Latin female is reported with a terminal deletion of the No. 1 chromosome, karyotype formula 46, XX, del(1) (q43). Principle clinical features include: Anatomic - microcephaly; bilateral, convergent strabismus; epicanthus; brachycephaly; bulbar nose; sparse hair; partial soft tissue syndactylism between 2nd and 3rd fingers which are slightly tapered; whorls on all 10 fingers; mild prognathism; solitary kidney; vaginal stenosis; vesicoureteral reflux; asymmetric feet; and subluxation of peroneal tendons around the fibula with severe pronation and heal valgus deformity. Neurologic - moderate motor and mental retardation; high-pitched, shrill cry; absent pincer grasp at 3 years; and grand mal seizures documented from 9 months of age.
据报道,一名3岁拉丁裔女性存在1号染色体末端缺失,核型公式为46, XX, del(1) (q43)。主要临床特征包括:解剖学特征——小头畸形;双侧会聚性斜视;内眦赘皮;短头畸形;球根状鼻;头发稀疏;第二和第三指之间部分软组织并指,手指略呈锥形;10个手指均有涡纹;轻度突颌;单肾;阴道狭窄;膀胱输尿管反流;足部不对称;腓骨周围腓骨肌腱半脱位伴严重内旋和足跟外翻畸形。神经学特征——中度运动和智力发育迟缓;高音调、尖锐哭声;3岁时无捏物动作;9个月大时记录有癫痫大发作。