Maroteaux P, Stanescu R, Stanescu V, Fontaine G
Eur J Pediatr. 1980 May;133(3):227-31. doi: 10.1007/BF00496081.
A 9-year-old boy from an incestuous union presented with mild clinical and roentgenological manifestations of pseudoachondroplasia. Tibial growth cartilage was obtained during a routine orthopedic procedure. Microscopic, histochemical and biochemical analysis showed changes identical to those of 6 sporadic cases of typical pseudoachondroplasia. The results support a concept of pathogenetic homogeneity of pseudoachondroplasia.
一名来自乱伦结合家庭的9岁男孩出现了轻度的临床和X线表现的假性软骨发育不全。在一次常规骨科手术中获取了胫骨生长软骨。显微镜、组织化学和生化分析显示的变化与6例典型假性软骨发育不全散发病例相同。这些结果支持假性软骨发育不全发病机制同质性的概念。