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[肾淀粉样变性患者的临床及组织学表现]

[Clinical and histologic findings in patients with renal amyloidosis].

作者信息

Rasić S, Cengić M, Golemac S, Uncanin S, Karamehić J

机构信息

Institut za nefrologiju i imunologiju, Klinicki centar Sarajevo.

出版信息

Med Arh. 1996;50(3-4):85-7.

PMID:9601760
Abstract

Renal amyloidosis is a rare disease when compared to other kidney diseases. During the period of last fifteen years, at the Institute of Nephrology and Immunology in Sarajevo renal amyloidosis was diagnosed with 15 patients. The disease occurred more often with men than with women. Only during 1988, renal amyloidosis was revealed and followed up with five patients. The most common clinical manifestations of renal amyloidosis are nephrotic syndrome and chronic renal failure, with respective laboratory findings. Using immunofluorescent analysis of the kidney biopsy material, we discovered deposits of immunoglobulins of different intensity and deposits of lambda and kappa light chains of immunoglobulins. The intensity of lambda light chains is greater than that of kappa chains. The analysis of light microscopy showed nodular mesangial deposits and deposits along GBM without proliferation. The diagnosis of amyloidosis was confirmed by staining of amyloid. Application of therapy for amyloidosis was without any effect. Although renal amyloidosis is a rare disease, we want to point out disease as being an etiologic factor in nephrotic syndrome.

摘要

与其他肾脏疾病相比,肾淀粉样变性是一种罕见疾病。在过去十五年间,萨拉热窝的肾脏病与免疫学研究所确诊了15例肾淀粉样变性患者。该疾病在男性中比在女性中更常见。仅在1988年,就发现并随访了5例肾淀粉样变性患者。肾淀粉样变性最常见的临床表现是肾病综合征和慢性肾衰竭,并伴有相应的实验室检查结果。通过对肾活检材料进行免疫荧光分析,我们发现了不同强度的免疫球蛋白沉积物以及免疫球蛋白的λ和κ轻链沉积物。λ轻链的强度大于κ链。光学显微镜分析显示结节性系膜沉积物以及沿肾小球基底膜的沉积物,且无增生现象。淀粉样变的诊断通过淀粉样蛋白染色得以证实。应用针对淀粉样变性的治疗没有任何效果。尽管肾淀粉样变性是一种罕见疾病,但我们想指出该疾病是肾病综合征的一个病因。

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