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聚乙烯吡咯烷酮储存病。光镜、超微结构及化学鉴定

Polyvinylpyrrolidone-storage disease. Light microscopical, ultrastructural and chemical verification.

作者信息

Reske-Nielsen E, Bojsen-Moller M, Vetner M, Hansen J C

出版信息

Acta Pathol Microbiol Scand A. 1976 Sep;84(5):397-405.

PMID:970128
Abstract

The light and electron microscopical findings in the polyvinylpyrrolidine-storage disease are reported on the basis of biopsies of skin, striated muscle, bone marrow and liver from one patient and a subcutaneous nodule from another patient. Both patients suffer from diabetes insipidus and have been treated for several years with Insipidin retard, which contains polyvinylpyrrolidone (PVP) as the retarding agent. Deposits of PVP have been demonstrated in all the tissues examined and can easily be recognized by certain staining qualities. The combination of haematoxylin-eosin, elastin (Weigert), alkaline Congo red and Sirius red for amyloid and PTAH is specially to be recommended. The ultrastructural findings consist of intracellular vacuoles containing a granular material and probably representing lysosomes. The final identification of the nature of the deposits has been made by spectrophotometric analysis. The suspicion of a case of PVP-storage disease should result in a skin biopsy which usually is sufficient for the diagnosis.

摘要

基于对一名患者的皮肤、横纹肌、骨髓和肝脏活检以及另一名患者的皮下结节活检结果,报告了聚乙烯吡咯烷酮储存病的光镜和电镜检查结果。两名患者均患有尿崩症,多年来一直使用含有聚乙烯吡咯烷酮(PVP)作为阻滞剂的长效尿崩停进行治疗。已在所有检查的组织中证实了PVP的沉积,并且通过某些染色特性很容易识别。苏木精-伊红、弹性蛋白(魏格特氏法)、碱性刚果红和用于淀粉样蛋白及磷钨酸苏木精的天狼星红联合染色特别值得推荐。超微结构检查结果显示细胞内有空泡,其中含有颗粒状物质,可能代表溶酶体。沉积物性质的最终鉴定通过分光光度分析完成。怀疑患有PVP储存病的病例应进行皮肤活检,这通常足以做出诊断。

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