Spranger S, Spranger M, Meinck H M, Tariverdian G
Institute of Human Genetics and Anthropology, University of Heidelberg, Germany.
Am J Med Genet. 1995 Jul 3;57(3):425-8. doi: 10.1002/ajmg.1320570312.
We report on 2 sisters with an autosomal-recessive multiple pterygium syndrome, type Escobar, consisting of multiple pterygia with severe contractures, short stature, and minor facial and external genital anomalies. The striking finding was severe muscular atrophy. We speculate that a neuromuscular disorder is the underlying pathogenesis of Escobar syndrome.
我们报告了2名患有常染色体隐性遗传的埃斯科瓦尔型多发性翼状胬肉综合征的姐妹,其特征为多发性翼状胬肉伴严重挛缩、身材矮小以及轻微的面部和外生殖器异常。显著的发现是严重的肌肉萎缩。我们推测神经肌肉疾病是埃斯科瓦尔综合征的潜在发病机制。