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成人骨硬化症:两兄弟的研究

Adult osteopetrosis: study of two brothers.

作者信息

Silvestrini G, Ferraccioli G F, Quaini F, Palummeri E, Bonucci E

出版信息

Appl Pathol. 1987;5(3):184-9.

PMID:3620211
Abstract

Bone biopsies from 2 adult cases of benign osteopetrosis have been studied histologically and under the electron microscope. The most important findings were a high degree of osteosclerosis, narrowing and fibrosis of medullary spaces, and the presence of abundant uncalcified osteoid tissue with an osteomalacia-like appearance. The osteoclasts were not numerous. All of them lacked the brush border and many showed wide cytoplasmic vacuoles containing amorphous and filamentous material. This picture, which is not different from that found in cases of fetal osteopetrosis, has not been modified by ethydronate therapy.

摘要

对2例成人良性骨硬化症患者的骨活检组织进行了组织学和电子显微镜研究。最重要的发现是高度骨硬化、髓腔狭窄和纤维化,以及存在大量外观类似骨软化症的未钙化类骨质组织。破骨细胞数量不多。所有破骨细胞均缺乏刷状缘,许多破骨细胞显示含有无定形和丝状物质的宽大胞质空泡。这种情况与胎儿骨硬化症病例中发现的情况并无不同,依替膦酸盐治疗并未改变这种情况。

相似文献

1
Adult osteopetrosis: study of two brothers.成人骨硬化症:两兄弟的研究
Appl Pathol. 1987;5(3):184-9.
2
The osteopetrotic rabbit: skeletal cytology and ultrastructure.骨石化兔:骨骼细胞学与超微结构
Am J Anat. 1987 Mar;178(3):300-7. doi: 10.1002/aja.1001780310.
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[Bones and bone marrow of the newborn " op " osteopetrotic rat, and the early diagnosis of the disease].新生“op”骨硬化症大鼠的骨骼与骨髓以及该疾病的早期诊断
Ann Anat Pathol (Paris). 1979;24(2):149-60.
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A quantitative histologic study of avian osteopetrotic bone demonstrating normal osteoclast numbers and increased osteoblastic activity.一项关于禽类骨石化骨的定量组织学研究表明破骨细胞数量正常且成骨细胞活性增加。
Lab Invest. 1981 Feb;44(2):164-73.
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Osteoclast diseases.破骨细胞疾病
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Osteopetrosis in mice lacking haematopoietic transcription factor PU.1.缺乏造血转录因子PU.1的小鼠中的骨质石化症
Nature. 1997 Mar 6;386(6620):81-4. doi: 10.1038/386081a0.
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Adult osteosclerosis.成人骨硬化症
Metab Bone Dis Relat Res. 1983;5(3):111-7. doi: 10.1016/0221-8747(83)90011-5.
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Neonatal changes of osteoclasts in osteopetrosis (op/op) mice defective in production of functional macrophage colony-stimulating factor (M-CSF) protein and effects of M-CSF on osteoclast development and differentiation.骨硬化症(op/op)小鼠中破骨细胞的新生变化,该小鼠在功能性巨噬细胞集落刺激因子(M-CSF)蛋白产生方面存在缺陷,以及M-CSF对破骨细胞发育和分化的影响。
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Ultrastructural findings of bone marrow in a case with malignant osteopetrosis following successful allogeneic bone marrow transplantation.
Turk J Pediatr. 1999 Jul-Sep;41(3):353-60.
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Malignant osteopetrosis: a disease of abnormal osteoclast proliferation.
Metab Bone Dis Relat Res. 1981;3(2):99-105. doi: 10.1016/0221-8747(81)90027-8.

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Osteopetrosis complicated by osteomyelitis of the maxilla and mandible: light and electron microscopic findings.骨质石化症合并上颌骨和下颌骨骨髓炎:光镜和电镜检查结果
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