Keppen L D, Gollin S M, Seibert J J, Sisken J E
Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock.
Am J Med Genet. 1991 Jan;38(1):21-4. doi: 10.1002/ajmg.1320380106.
Roberts-SC phocomelia syndrome (RS) is an autosomal recessive disorder of symmetric limb defects, craniofacial abnormalities, pre- and postnatal growth retardation, and mental retardation. Patients with RS have been reported to have premature separation of heterochromatin of many chromosomes and abnormalities in the cell-division cycle. We report an infant whose clinical and radiologic findings resemble those of RS but who lacks the cytogenetic and cell division abnormalities reported in RS. This patient may represent a variant of RS or a new syndrome.
罗伯茨-短肢畸形综合征(RS)是一种常染色体隐性疾病,其特征为对称性肢体缺陷、颅面异常、产前和产后生长发育迟缓以及智力障碍。据报道,RS患者存在许多染色体异染色质过早分离以及细胞分裂周期异常的情况。我们报告了一名婴儿,其临床和放射学表现与RS相似,但不存在RS所报道的细胞遗传学和细胞分裂异常。该患者可能代表RS的一种变异型或一种新的综合征。