大骨节病病因病理学研究  

DOI:10.7652/jdyxb202305022 An etiogeno-pathological study on Kashin-Beck disease

作　　者：毕华银[1] BI Huayin(Institute for Endemic Diseases,School of Public Health of Xi’an Jiaotong University,Xi’an 710061,China)

机构地区：[1]西安交通大学公共卫生学院地方病研究所,陕西西安710061

出　　处：《西安交通大学学报（医学版）》2023年第5期817-822,共6页Journal of Xi’an Jiaotong University（Medical Sciences）

摘　　要：目的寻找并认定大骨节病(Kashin-Beck disease,KBD)的病因。方法复查本所KBD剖检档案16例的骨切片。光镜观察KBD患儿末梢血涂片中白细胞的病变,电镜观察患儿白细胞的超微病变。观察KBD患儿血浆引发培养兔软骨细胞损伤的实验结果。结果KBD剖检例的软骨细胞、骨髓血细胞及KBD患儿的白细胞,这三类细胞病变的特征及其全过程完全一致,均显示特异的凝固性坏死,细胞核增大,核内出现由少到多、由小到大的嗜酸性(红色)包涵体,伴随以细胞核染色质的溶解减少;若病变持续恶化进展,则整个细胞核可转化成一个红染的大团块;其后出现该包涵体的后续性系列变化;细胞体缩小,细胞质红染。患儿血浆引发培养兔软骨细胞复制出等同于剖检例软骨细胞特异坏死病变的细胞模型,且在其细胞核内检可见病毒核衣壳。在患儿白细胞的核内亦可见相同的病毒核衣壳。在剖检例的骨髓内可见充血、水肿、纤维素渗出,造血主质和骨小梁的灶性坏死以及骨髓纤维化等。结论在KBD剖检例病变的软骨细胞和骨髓血细胞的核内及KBD病患儿末梢血白细胞的核内均显示相同的包涵体形成;包涵体形成是病毒杀细胞性感染最为熟知的病理形态学结果;特别是以KBD患儿血浆作用于培养软骨细胞所出现的阳性实验结果,均提示该三类细胞坏死的病因理应是两次检出的同一病毒,该病毒就应是KBD的病因。剖检例的骨髓内有急性骨髓炎伴发传染性迟发型超敏反应。KBD是该病毒引起的全身性传染病;软骨病变、骨髓病变和血液病变都仅是其全身病变的一部分。Objective To find and identify the cause of Kashin-Beck disease(KBD).Methods We reviewed the bone slices of the KBD autopsy cases preserved in our institute,and observed the leukocyte lesions in peripheral blood smears of KBD children under a light microscope and ultrastructural lesions of leukocytes in KBD children under an electron microscope.We also observed the damages in cultured chondrocytes induced by plasma of children with KBD in an experiment.Results From the chondrocytes,bone marrow blood cells in KBD autopsy cases and leukocytes of KBD children,the entire pathological process in all the three type cells showed the same specific coagulation necrosis:the nucleus was enlarged;eosinophilic red inclusion bodies in varying sizes appeared in the nucleus,which were accompanied by dissolution and decrease of nuclear chromatin.If the lesion continued to deteriorate and progress,the entire nucleus would transform into a large red mass,and then subsequent series of changes of the inclusion bodies occurred;the cell body shrank and the cytoplasm was stained red.The cultured chondrocytes had replicated a cytopathic model equivalent to specific chondrocyte necrosis in the autopsy cases of KBD,and the viral nucleocapsids were detected in the nuclei of the cultured chondrocytes,with outcomes induced by plasma of the KBD children in the experiment.The same viral nucleocapsids as previously mentioned were also found in the nuclei of the white blood cells of the children with KBD.In the bone marrows of the autopsy cases,hyperemia,edema,fibrin exudation,focal necrosis of hematopoietic matrix and trabecular bone,and fibrosis all appeared.Conclusion The inclusion body formations were showed in the nuclei of necrotic chondrocytes,bone marrow blood cells of KBD autopsy cases,and in the nuclei of leukocytes in peripheral blood smears of KBD children.The inclusion body formation is the most well-known pathomorphological result of the viral cytocidal infection.Especially important is the positive results of cultured chondrocytes

关 键 词：大骨节病 病毒病因 病毒核衣壳 嗜酸性包涵体 软骨病变 全身性传染病 

分 类 号：R684.1[医药卫生—骨科学]