AN AUTOPSY CASE OF PROLIDASE DEFICIENCY
AN AUTOPSY CASE OF PROLIDASE DEFICIENCY
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DOI：
10.1007/bf00710562
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发表时间：
1985-01-01
期刊：
影响因子：
--
通讯作者：
KIMURA, K
中科院分区：
文献类型：
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作者：
SEKIYA, M;OHNISHI, Y;KIMURA, K
一名25岁的女性，患有长期无法治愈的腿部溃疡，被发现患有氨基二肽硬尿的脯氨酸酶缺乏症。尸检标本超微结构观察发现，真皮基底膜致密层呈不规则裂开，真皮血管基底膜呈片状中断。肾小管、间质血管和肾小球毛细血管的基底膜也发生板层改变和裂开。这些形态异常似乎是引起临床症状的原因之一。
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.