Cherstvoy E D, Lurie I W, Shved I A, Lazjuk G I, Ostrowskaya T I, Usoev S S
Eur J Pediatr. 1980;133(1):57-61. doi: 10.1007/BF00444756.
A case of "short rib-polydactyly" (SRP) syndrome (type II-Saldino-Noonan) with a detailed study of the bones, muscles and peripheral nerves in the extremities is presented. Analysis of the literature (35 cases with different forms of SRP) and the comparison of clinical, genetic and morphological data lead us to consider that different types of SRP may be related to different alleles of the same gene. Genetic heterogeneity (mutations of different genes) is also possible.
本文报告一例“短肋多指畸形”(SRP)综合征(II型-萨尔迪诺-努南型)病例,并对四肢骨骼、肌肉和周围神经进行了详细研究。通过对文献(35例不同形式的SRP)的分析以及临床、遗传和形态学数据的比较,我们认为不同类型的SRP可能与同一基因的不同等位基因有关。基因异质性(不同基因的突变)也有可能。