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[IgA肾病中的类固醇反应性肾病综合征]

[Steroid-responsive nephrotic syndrome in IgA nephropathy].

作者信息

García Camín R M, Cabezas A, Calero F, Ballarín J A

机构信息

Servicio de Nefrología, Fundación Puigvert, Barcelona, España.

出版信息

Nefrologia. 2004;24 Suppl 3:68-71.

PMID:15219073
Abstract

A 46-year-old male, smoker of half a packet a day and an alcohol intake of 80 grams a day, with an unremarkable medical history, was referred to our service in the year 1988 for a study of nephrotic syndrome. He presented normal renal function, without either microhematuria nor hypertension. In blood analysis an albuminemia of 10 g/l and proteinuria of 22 g/d was observed. A first renal biopsy was carried out which indicated minimal change disease. Steroid treatment was started, as a result the nephrotic syndrome disappeared and the blood analysis normalized. Later he had 4 new outbreaks, all of them steroid-responsive. In 1992 a second renal biopsy was performed after the fourth outbreak and the presence of mild renal failure, that this time indicated a IgA nephropathy. Steroid treatment was tried again, and this time cyclophosphamide was added to try to reduce steroid doses. This result in normalization of renal function and decrease of proteinuria to 2 g/d. The patient remained stable until 1996 when the fifth outbreak occurred, again with mild renal failure and proteinuria in nephrotic range. Therefore a third renal biopsy was performed, that confirmed the presence of IgA nephropathy, but now with signs of histological progression of the disease. Following this, he presented five outbreaks in 3 years, all of them steroid-responsive, with decrease of proteinuria although without renal function normalization. In the year 2000, at his tenth outbreak of nephrotic syndrome it was decided to add cyclosporine to the steroid treatment, achieving the stability of the patient, without further outbreaks until now, with proteinuria of 1.6 g/d and C. creat. 59 ml/min.

摘要

一名46岁男性,每天吸烟半包,每日饮酒80克,既往病史无异常。1988年因肾病综合征前来我院就诊。他的肾功能正常,无镜下血尿及高血压。血液分析显示白蛋白血症为10g/l,蛋白尿为22g/d。首次肾活检提示微小病变性肾病。开始使用类固醇治疗,结果肾病综合征消失,血液分析恢复正常。后来他又复发了4次,均对类固醇治疗有反应。1992年在第四次复发及出现轻度肾衰竭后进行了第二次肾活检,此次提示为IgA肾病。再次尝试类固醇治疗,此次加用环磷酰胺以减少类固醇剂量。这使得肾功能恢复正常,蛋白尿降至2g/d。患者病情一直稳定至1996年,第五次复发,再次伴有轻度肾衰竭及肾病范围的蛋白尿。因此进行了第三次肾活检,证实为IgA肾病,但此时已有疾病组织学进展的迹象。此后,他在3年内复发了5次,均对类固醇治疗有反应,蛋白尿有所减少,但肾功能未恢复正常。2000年,在他第十次肾病综合征复发时,决定在类固醇治疗中加用环孢素,使患者病情稳定,至今未再复发,蛋白尿为1.6g/d,肌酐清除率为59ml/min。

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[Steroid-responsive nephrotic syndrome in IgA nephropathy].[IgA肾病中的类固醇反应性肾病综合征]
Nefrologia. 2004;24 Suppl 3:68-71.
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Behcet's disease and IgA nephropathy: report of this association in a patient from Brazil and literature review.白塞病与IgA肾病:巴西一名患者的病例报告及文献综述
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Clin Nephrol. 1997 Jan;47(1):60-2.
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Long-term effects of cyclosporine in children with idiopathic nephrotic syndrome: a single-centre experience.环孢素对特发性肾病综合征患儿的长期影响:单中心经验
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Clinicopathologic characteristics of IgA nephropathy with steroid-responsive nephrotic syndrome.IgA肾病合并激素敏感性肾病综合征的临床病理特征
J Korean Med Sci. 2009 Jan;24 Suppl(Suppl 1):S44-9. doi: 10.3346/jkms.2009.24.S1.S44. Epub 2009 Jan 28.