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膜性脂肪营养不良的发病机制。病例报告及文献综述。

Pathogenesis of membranous lipodystrophy. Case report and review of the literature.

作者信息

Pazzaglia U E, Benazzo F, Byers P D, Riboni L, Ceciliani L

机构信息

Clinica Ortopedica e Traumatologica dell'Università di Pavia ed I.R.C.C.S. Policlinico San Matteo, Italy.

出版信息

Clin Orthop Relat Res. 1987 Dec(225):279-87.

PMID:3677514
Abstract

Membranous lipodystrophy occurred in a 35-year-old woman and membrane-like material was found in her epiphyses and short bones. Electron microscopy allowed a study of the relationship between the pathologic tissue and normal fat, suggesting that the membrane-like material is derived from degenerated cells. Nucleation of hydroxyapatite crystals was also observed in the membranous material. Because basal ganglia calcifications were demonstrated with the use of computed tomography, brain and bone lesions may be assumed to share the same pathogenesis. Lipid and mucopolysaccharide metabolism was found to be normal.

摘要

一名35岁女性发生了膜性脂肪营养不良,在其骨骺和短骨中发现了膜样物质。电子显微镜检查有助于研究病理组织与正常脂肪之间的关系,提示膜样物质来源于退变细胞。在膜样物质中还观察到羟基磷灰石晶体的成核现象。由于计算机断层扫描显示基底节钙化,故可推测脑和骨病变具有相同的发病机制。发现脂质和黏多糖代谢正常。

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