Ⅰ型神经纤维瘤病营养不良性脊柱侧凸患者椎体生长板软骨细胞的功能变化及意义

Functional change and significance of vertebral growth plate chondrocytes in patients with scoliosis secondary to type 1 neurofibromatosis

中文关键词:  神经纤维瘤病  脊柱侧凸  营养不良  神经纤维瘤蛋白  软骨细胞

英文关键词:Neurofibromatoses  Scoliosis  Dystrophia  Neurofibromin  Chondrocyte

中文摘要:

  【摘要】 目的：观察Ⅰ型神经纤维瘤病（NF1）营养不良性脊柱侧凸患者椎体生长板软骨细胞的功能变化，探讨其在脊柱营养不良性改变中的作用。方法：共有8例NF1营养不良性脊柱侧凸患者纳入本研究，男5例，女3例，年龄9~15岁，平均11.8±2.0岁。在矫形术中取营养不良区（顶椎区，A组）、非营养不良区（端椎区，B组）椎体生长板软骨及髂软骨（C组）。采用两步酶消化结合植块法进行软骨细胞培养。应用Real-time PCR法检测软骨细胞中可聚蛋白多糖、Ⅱ型胶原及神经纤维瘤蛋白的mRNA表达。结果：A组软骨细胞中可聚蛋白多糖mRNA的表达量为0.04±0.02，B组为0.09±0.04，C组为0.13±0.07，A组显著低于B组和C组（P<0.05）。A组Ⅱ型胶原的mRNA表达量为0.91±0.04，B组为0.96±0.07，C组为1.03±0.10，A组显著低于C组（P<0.05），A组与B组差别无统计学意义（P>0.05）。A组神经纤维瘤蛋白的mRNA表达量为0.39±0.30，B组为1.34±0.63，C组为1.00±0.51，A组较B、C组均明显降低（P<0.05）。各指标在B组与C组间均无统计学差异（P>0.05）。结论：NF1营养不良性脊柱侧凸患者营养不良区椎体生长板软骨细胞分化功能存在明显缺陷，其可能是脊柱营养不良性改变的基础。

英文摘要:

  【Abstract】 Objective：To investigate the biological features of the chondrocytes isolated from vertebral growth plate in patients with type 1 neurofibromatosis（NF1） and its role in the dystrophic change of vertebrae.Method：This study included 8 cases of NF1 complicated with dystrophic scoliosis.There were 5 boys and 3 girls，aged from 9 to 15 years（mean 11.8±2.0 years）.Growth plates of dystrophic vertebrae（group A） and non-dystrophic vertebrae（group B） were harvested during anterior surgery，and iliac growth plate （group C） was harvested during posterior surgery.Chondrocytes from growth plate were isolated and cultured by two-step enzymatic digestion and tissue culture system in vitro.Expressions mRNA of aggrecan and type Ⅱ collagen as well as neurofibromin were assayed in P2 chondrocytes by Real-time PCR.Result：Expression mRNA of aggrecan was significantly lower in chondrocytes of group A（0.04±0.02） than the other two groups（0.09±0.04，0.13±0.07 respectively）（P<0.05）.The level of type Ⅱ collagen in group A（0.91±0.04） was significantly lower than group C（1.03±0.10）（P<0.05），yet not significantly lower than group B（0.96±0.07）（P>0.05）.Also，lower level of neurofibromin was detected in group A（0.39±0.30） compared with group B（1.34±0.63） and C（1.00±0.51）（P<0.05）.Each parameter showed no difference between group B and C（P>0.05）.Conclusion：Dysfunction of vertebral growth plate chondrocytes may contribute to the dystrophic change of vertebrae in scoliosis associated with NF1.