Temtamy S A, Miller J D, Hussels-Maumenee I
J Pediatr. 1975 May;86(5):724-31. doi: 10.1016/s0022-3476(75)80357-x.
Eight patients in three families had mental retardation, characteristic facies and hands, and skeletal changes; the clinical features suggested to us that they had a syndrome previously thought to represent two entities described by Lowry and associates and by Coffin and associates, respectively. New findings include skeletal, orodental, and dermatoglyphic abnormalities and histopathologic changes suggesting that the syndrome is a heritable disorder of connective tissue. Severe expression in males and transmission through mildly affected females suggest X-linked or sex-influenced autosomal dominant inheritance.
三个家族中的八名患者有智力发育迟缓、特征性面容和手部表现以及骨骼变化;这些临床特征让我们认为他们患有一种先前被认为分别由洛瑞及其同事和科芬及其同事所描述的两种病症组成的综合征。新发现包括骨骼、口腔牙齿和皮纹异常以及组织病理学变化,提示该综合征是一种结缔组织遗传性疾病。男性症状严重且通过症状较轻的女性传递,提示为伴X连锁或性影响常染色体显性遗传。