Stevenson David A, Carey John C
Department of Pediatrics, Division of Medical Genetics, University of Utah, Salt Lake City, Utah 84132, USA.
Am J Med Genet A. 2007 Oct 1;143A(19):2221-6. doi: 10.1002/ajmg.a.31945.
We report on the similar phenotypes and clinical course of two sisters. Both patients had an enlarged cisterna magna suggestive of cerebellar hypoplasia, agenesis/hypoplasia of the corpus callosum, Pierre Robin sequence requiring tracheostomy, camptodactyly, microphthalmia, colobomas, seizures, a distinctive facial appearance, global developmental delay, and mental retardation. We propose that the distinct pattern in these sisters constitutes a previously undescribed syndrome of likely autosomal recessive inheritance.
我们报告了两姐妹相似的表型和临床病程。两名患者均有小脑延髓池扩大提示小脑发育不全、胼胝体发育不全/发育不良、需要气管切开术的皮埃尔·罗宾序列、屈曲指、小眼症、缺损、癫痫发作、独特的面部外观、全面发育迟缓以及智力障碍。我们认为,这两名姐妹的独特模式构成了一种先前未描述的可能为常染色体隐性遗传的综合征。