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一例脯氨酰肽酶缺乏症尸检病例。

An autopsy case of prolidase deficiency.

作者信息

Sekiya M, Ohnishi Y, Kimura K

出版信息

Virchows Arch A Pathol Anat Histopathol. 1985;406(1):125-31. doi: 10.1007/BF00710562.

Abstract

A 25-year-old female who suffered from longstanding incurable leg ulcers was found to have prolidase deficiency with iminodipeptiduria. On ultrastructural studies of autopsy specimens, the lamina densa of the epidermal basement membrane was found to show irregular splitting and the basement membranes of the dermal blood vessels were lamellated with interruptions. Lamellar changes and splitting of the basement membranes of the renal tubules, interstitial blood vessels and glomerular capillaries also occurred. These morphological abnormalities seem to be one of causes of the clinical symptomatology.

摘要

一名患有长期无法治愈的腿部溃疡的25岁女性被发现患有氨肽酶缺乏症并伴有亚氨基二肽尿症。在尸检标本的超微结构研究中,发现表皮基底膜的致密层显示出不规则分裂,真皮血管的基底膜呈层状且有中断。肾小管、间质血管和肾小球毛细血管的基底膜也出现了层状变化和分裂。这些形态学异常似乎是临床症状的原因之一。

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