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特发性成人局灶节段性肾小球硬化症:一项临床病理研究及对类固醇的反应

Idiopathic adult focal segmental glomerulosclerosis: a clinicopathological study and response to steroid.

作者信息

Agarwal S K, Dash S C, Tiwari S C, Bhuyan U N

机构信息

Department of Nephrology and Pathology, All India Institute of Medical Sciences, New Delhi.

出版信息

Nephron. 1993;63(2):168-71. doi: 10.1159/000187177.

DOI:10.1159/000187177
PMID:8450907
Abstract

A total of 65 adult cases (53 males, 12 females) with biopsy-proven focal segmental glomerulosclerosis (FSGS) were studied. Hypertension, ascites and haematuria were seen in 13, 12 and 24 cases, respectively. Decreased creatinine clearance at presentation was found in 9 cases. Mean proteinuria per day, serum cholesterol and total protein were 7.5 +/- 4.3 g, 388.95 +/- 213.4 mg% (10.11 +/- 5.55 mmol/l) and 5.27 +/- 1.1 g% (0.527 +/- 0.11 milligram), respectively. Mesangial proliferation was seen in 13 cases and hilar sclerosis in 5. Fifty percent showed positive immunofluorescence; IgM in 10, C3 in 8, and IgG in 2. Forty-two cases could be followed (mean 32 months), out of which 38 had nephrotic syndrome and were treated with prednisolone; 58% showed response (31% complete remission and 27% partial remission). One patient in each group of responders and nonresponders had renal failure at the end of follow-up. Hypertension, degree of proteinuria, mesangial proliferation, degree of tubular atrophy and immunofluorescence findings did not significantly affect the response to steroids. We conclude that a group of patients with idiopathic adult FSGS has a favourable response to steroids, which cannot be predicted clinically.

摘要

共研究了65例经活检证实为局灶节段性肾小球硬化(FSGS)的成年患者(53例男性,12例女性)。分别有13例、12例和24例出现高血压、腹水和血尿。9例患者就诊时肌酐清除率降低。每日平均蛋白尿、血清胆固醇和总蛋白分别为7.5±4.3g、388.95±213.4mg%(10.11±5.55mmol/L)和5.27±1.1g%(0.527±0.11mg)。13例可见系膜增生,5例可见肾门硬化。50%的患者免疫荧光呈阳性;10例为IgM阳性,8例为C3阳性,2例为IgG阳性。42例患者得到随访(平均32个月),其中38例患有肾病综合征并接受泼尼松龙治疗;58%的患者有反应(31%完全缓解,27%部分缓解)。随访结束时,反应者组和无反应者组各有1例患者出现肾衰竭。高血压、蛋白尿程度、系膜增生、肾小管萎缩程度和免疫荧光结果对类固醇治疗反应无显著影响。我们得出结论,一组特发性成人FSGS患者对类固醇治疗反应良好,这在临床上无法预测。

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