摘要
目的:探讨胎儿软骨发育低下(HCH)的病理学特征、主要研究方法及诊断思路。方法:对一例因四肢短小而引产的胎儿进行尸体解剖,对其主要四肢骨骼及颅骨进行直接测量,在光镜下观察这些骨骼及主要脏器,并对骨骼、气管软骨、大中小动脉分别进行AB-PAS(阿尔辛蓝-过碘酸-无色品红法)及Masson三色染色,染色结果与无异常及畸形的相同胎龄胎儿的上述组织进行形态学比较。结果:患儿的畸形主要表现为四肢长骨及喉、气管软骨的发育异常。四肢主要长骨长度明显低于正常值,且股骨骨性标志不突出。镜下长骨生长板存在,但形态发育欠佳,气管软骨细胞多呈不活跃或凋亡状态,软骨基质及胶原较正常偏少。结论:软骨发育低下属于一种畸形程度较轻的骨软骨发育异常,主要影响软骨的发育成熟及软骨化骨,通过临床表现、影像学检测及病理观察可以确诊。
Objective:To study the pathological features,methods and diagnosis of hypochondroplasia (HCH). Methods : Autopsy was performed with an induced abortive fetus who was diagnosed as shortness of all its extremities. The appendicular skeleton and cranial bone were directly measured and these bones and its main organs These bones and tracheal cartilage, arteries of various were observed under the light microscope. sizes were stained with AB-PAS and Masson trichrome staining. The measured values and histological results were compared with normal fetus in the same gestational week. Results:The fetus showed abnormalities of cartilage development in appendicular skeleton and tracheal cartilage without striking changes in other organs. The lengths of extremity bones were much shorter than normal ranges. The skeletal hallmarks of femur were not prominent. Under the microscope, the growth plate of long bone did exist, but were not formed well. The cartilage cells in trachea were not active, even apoptotic. The cartilage matrix and collagen of the above regions were less developed than normal. Conclusion:Hypochondroplasia is a kind of slight abnormality compared with other types of osteochondrodysplasia. Cartilage development and endochondral ossification are mainly influenced. It can be diagnosed by clinical features,radiological exhibition and pathological findings.
出处
《现代妇产科进展》
CSCD
北大核心
2009年第6期415-419,共5页
Progress in Obstetrics and Gynecology