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Pathologic study on tissue anomalies in the developing brain

发育中大脑组织异常的病理学研究

基本信息

批准号：
02045029
负责人：
HARA Hiroshi
金额：
$ 3.46万
依托单位：
Kochi Medical School
依托单位国家：
日本
项目类别：
Grant-in-Aid for international Scientific Research
财政年份：
1990
资助国家：
日本
起止时间：
1990 至 1992
项目状态：
已结题

来源：
https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-02045029/
关键词：
Brain Tissue anomalies Pathology Cretinism Cerebellum Cogenital anomalies Electron microscopy Immunohistochemistry 内耳

项目摘要

It has been well known that in endemic cretinism, the outstanding symptoms are dwarfism, deafness, and mental retardation. The propose of the present study was to examine pathomorphologic changes of brains in endemic cretinism patients and experimental cretin rats.1. Histopathological study was made on cerebellar cortex of six patients with endemic cretinism in Jixian village, Heilongjiang, China. Examination revealed increased density of granule cells and sparse myelin sheath in the granular layer. Immunohistochemistry, showed lowered reactivity to myelin basic protein in the deep molecular layer and granular layer of cerebellum. Increased density of granule cells in the patients seems to be related to underdevelopment of cerebellar glomeruli and deficient myelination in the granular layer.2. Experiment was performed on iodine deficient rats fed in Jiamusi with a diet made of the local agricultural products and drinking water from an area of high incidence of endemic cretinism. Experi … More ments were made also in Kochi using antithyroid drug, thiamazole. The mother rats were fed with the deficient diet during pregnancy and after delivery. Their offsprings fed on the same diet were sacrificed on day 1-one year of The experiments.(1) Light microscopy showed lower synaptophysin reactivity in the granule cell layer and increased density of granule cells in hypothyroid rates as compared to normal rats. Electron microscopy exhibited reduced density and number of ethanolic phosphotungstic acid-positive syanptic junctions in the cerebellar glomeruli. This finding seems to be related to lowered synaptophysin immunoreactivity.(2) Iodine deficiency led to a sluggish development and cellular degeneration of cochlear structures. These changes might be related to the pathogenesis of deafness of human endemic cretinism.3. The results suggest that iodine deficiency or hypothyroidism has effcts on cellular differentiation of cerebellar cortex end inner ear. Further studies are required to assess the full effects of iodine deficiency on the brain development. Less

众所周知，在地方性克汀病中，突出的症状是侏儒症、耳聋和智力迟钝。本研究的目的是观察地方性克汀病患者和实验性克汀大鼠的脑病理形态学变化。对黑龙江省蓟县6例地方性克汀病患者的小脑皮层进行了组织病理学研究。检查显示颗粒层颗粒细胞密度增加，髓鞘稀疏。免疫组化结果显示，大鼠小脑深层分子层和颗粒层对髓鞘碱性蛋白的反应性降低。患者颗粒细胞密度增高似乎与小脑肾小球发育不全和颗粒层髓鞘形成不足有关。本实验以佳木斯地区缺碘大鼠为研究对象，以当地农产品为食，以当地克汀病高发区的饮用水为食。在高知也用抗甲状腺药物——噻马唑制作了更多的药物。母鼠在怀孕期间和分娩后喂食缺乏营养的食物。在实验的第1天，以相同的饮食喂养它们的后代。(1)光镜下显示，甲状腺功能减退大鼠颗粒细胞层突触素反应性降低，颗粒细胞密度增加。电镜显示小脑肾小球内乙醇磷钨酸阳性突触连接密度和数量减少。这一发现似乎与突触素免疫反应性降低有关。(2)缺碘导致耳蜗结构发育迟缓和细胞变性。这些变化可能与人源性克汀病耳聋的发病机制有关。提示碘缺乏或甲状腺功能减退对内耳末端小脑皮层细胞分化有影响。需要进一步的研究来评估缺碘对大脑发育的全面影响。少