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膜增生性肾小球肾炎与持续性低补体血症。

Membranoproliferative glomerulonephritis and persistent hypocomplementaemia.

作者信息

Cameron J S, Glasgow E F, Ogg C S, White R H

出版信息

Br Med J. 1970 Oct 3;4(5726):7-14. doi: 10.1136/bmj.4.5726.7.

Abstract

The clinical, laboratory, and histological findings of 50 patients with membranoproliferative glomerulonephritis are described. Three-quarters of the patients, who were mostly older children and young adults, presented clinically with a mixture of "nephritic" and "nephrotic" symptoms; the remaining quarter had no symptoms and were diagnosed after the discovery of proteinuria and microscopic haematuria.Though this clinical picture may occur in other forms of glomerulonephritis, the patients described here were unified as a group by their glomerular morphological appearance-namely, a combination of mesangial proliferation and capillary wall thickening, mainly due to subendothelial accumulations of mesangial matrix.In 68% serum C3 (beta10-globulin) levels were reduced initially, while a further 16% subsequently showed a fall to abnormally low levels. All patients had substantial proteinuria, usually of moderately impaired selectivity, and all but one had haematuria in addition. Children frequently presented with an illness resembling acute nephritis, whereas adults usually had a nephrotic syndrome from the start.In 31 patients, followed for periods of one to eight and a half years, serial measurements of glomerular filtration rate were made. Sixteen have experienced no deterioration of renal function, though their proteinuria continues unchanged. Fifteen have shown progressive deterioration; six of them are still well, six are on regular dialysis treatment, and three have died. Treatment with corticosteroids, azathioprine, or cyclophosphamide, alone or in combination, did not seem to influence the course of the disease, and another two patients died from complications of steroid therapy. The disease usually runs a chronic course and appears to be progressive.

摘要

本文描述了50例膜增生性肾小球肾炎患者的临床、实验室及组织学检查结果。四分之三的患者多为大龄儿童及青年成人,临床上表现为“肾炎”和“肾病”症状的混合;其余四分之一患者无症状,在发现蛋白尿和镜下血尿后被确诊。尽管这种临床表现也可能出现在其他类型的肾小球肾炎中,但此处所描述的患者因其肾小球形态外观而被归为一组,即系膜增生和毛细血管壁增厚,主要是由于系膜基质在内皮下积聚。68%的患者血清C3(β10球蛋白)水平最初降低,另有16%随后降至异常低水平。所有患者均有大量蛋白尿,通常选择性中度受损,除1例患者外均伴有血尿。儿童常表现为类似急性肾炎的疾病,而成年人通常从一开始就患有肾病综合征。对31例患者进行了为期1至8年半的随访,定期测量肾小球滤过率。16例患者肾功能未恶化,但其蛋白尿持续不变。15例患者肾功能呈进行性恶化;其中6例情况尚好,6例接受定期透析治疗,3例死亡。单独或联合使用皮质类固醇、硫唑嘌呤或环磷酰胺治疗似乎并未影响疾病进程,另有2例患者死于类固醇治疗的并发症。该疾病通常呈慢性病程,且似乎具有进行性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e138/1820529/fcfff48e77ad/brmedj02156-0023-a.jpg

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