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[肾淀粉样变性患者肾病综合征的特点]

[The characteristics of the nephrotic syndrome in patients with renal amyloidosis].

作者信息

Shishkin A N, Shulutko T A

出版信息

Lik Sprava. 1993 May-Jun(5-6):80-3.

PMID:8209508
Abstract

68 cases of histologically confirmed renal amyloidosis associated clinically with the nephrotic syndrome (NS) were studied. The age of patients (over 40 years), mounting syndrome's manifestations in developing renal failure, absence of correlation between the quantity of amyloid deposits and completeness of the syndrome's picture were characteristic of the nephrotic syndrome in amyloidosis. NS in amyloidosis was most frequently the sign of extended process in kidneys and did not differ from other nephrotic syndromes by the disorders of lipid and protein metabolism. Direct correlation between nephrotic proteinuria and severity of tubulointerstitial lesions was found. 5-year survival from the onset of NS was 48%. Vital verification of NS caused by amyloidosis is complicated, nephrobiopsy being the most informative.

摘要

对68例经组织学确诊为肾淀粉样变性且临床上伴有肾病综合征(NS)的患者进行了研究。患者年龄(40岁以上)、肾衰竭进展时综合征表现加重、淀粉样沉积物数量与综合征表现的完整性之间缺乏相关性是淀粉样变性中肾病综合征的特征。淀粉样变性中的NS最常是肾脏广泛病变的征象,在脂质和蛋白质代谢紊乱方面与其他肾病综合征并无差异。发现肾病性蛋白尿与肾小管间质病变的严重程度直接相关。自NS起病后的5年生存率为48%。由淀粉样变性引起的NS的确诊很复杂,肾活检是最具信息量的检查。

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