Satar M, Atici A, Bişak U, Tunali N
Department of Pediatrics, Faculty of Medicine University of Cukurova, Adana, Turkey.
Clin Genet. 1994 Feb;45(2):107-8. doi: 10.1111/j.1399-0004.1994.tb04004.x.
Roberts-SC phocomelia syndrome (RS) is an autosomal recessive disorder with symmetric limb defects, craniofacial abnormalities, pre- and postnatal growth retardation and mental retardation. Patients with RS were reported to have premature separation of heterochromatin of many chromosomes. We report an infant whose clinical, radiologic and chromosomal findings resemble those of RS, with rudimentary gallbladder and accessory spleen. This patient may represent a variant of RS.
罗伯茨-短肢畸形综合征(RS)是一种常染色体隐性疾病,具有对称性肢体缺陷、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的过早分离。我们报告了一名婴儿,其临床、放射学和染色体检查结果与RS相似,伴有胆囊发育不全和副脾。该患者可能代表RS的一种变异型。