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[快速进展性肾小球肾炎综合征:病程、病理形态学及转归(考纳斯医科大学医院1996 - 2002年数据)]

[Rapidly progressive glomerulonephritis syndrome: course, pathomorphology and outcome (data of Kaunas University of Medicine Hospital 1996-2002)].

作者信息

Maslauskiene Rima, Urbanaviciene Jūrate

机构信息

Clinic of Nephrology, Kaunas University of Medicine Hospital, Kaunas, Lithuania.

出版信息

Medicina (Kaunas). 2003;39 Suppl 1:33-40.

Abstract

Rapidly progressive glomerulonephritis is rare but severe clinical syndrome, which results in end-stage renal disease if not treated. The prognosis can be improved with early diagnosis and aggressive treatment. The aim of our study was to analyze retrospectively all cases of rapidly progressive glomerulonephritis, diagnosed in Kaunas University of Medicine Hospital since 1996 and to evaluate the clinical, laboratory, immunological, pathomorphological data, treatment and outcome in these patients. In period of 1996-2002, 23 patients were treated in Departments of Nephrology, Rheumatology, and Pediatric Nephrology. Rapidly progressive glomerulonephritis was observed in elderly persons, the mean age 46.48+/-5.28 years. In 21.74% of cases, patients were diagnosed Goodpasture syndrome, in 21.74% of cases immune complex disease and in 56.52%--pauci immune glomerulonephritis which was mainly limited in kidney as necrotizing crescentic glomerulonephritis. Rapidly progressive glomerulonephritis in most cases manifested by weakness, hypertension, fever, involvement of lower airways, infection. Renal biopsies were performed in 18 (78.26%) cases, lung biopsy was made for one patient. Biopsies were made on 30.56+/-7.41 day of hospitalization. Twenty-two (95.65%) patients received intravenous pulses with methylprednisolone and/or cyclophosphamide; 56.52% patients required dialysis. Renal function improved in 47.83% patients; 8 (34.78%) patients died. The causes of death were infections complications, progression of main disease and cardiac arrest.

摘要

快速进展性肾小球肾炎是一种罕见但严重的临床综合征,若不治疗会导致终末期肾病。早期诊断和积极治疗可改善预后。我们研究的目的是回顾性分析自1996年以来在考纳斯医科大学医院诊断的所有快速进展性肾小球肾炎病例,并评估这些患者的临床、实验室、免疫学、病理形态学数据、治疗及预后情况。1996年至2002年期间,23例患者在肾病科、风湿科和小儿肾病科接受治疗。快速进展性肾小球肾炎多见于老年人,平均年龄为46.48±5.28岁。21.74%的病例诊断为Goodpasture综合征,21.74%为免疫复合物病,56.52%为寡免疫性肾小球肾炎,主要表现为坏死性新月体性肾小球肾炎,局限于肾脏。快速进展性肾小球肾炎多数病例表现为乏力、高血压、发热、下呼吸道受累、感染。18例(78.26%)患者进行了肾活检,1例患者进行了肺活检。活检在住院第30.56±7.41天进行。22例(95.65%)患者接受了甲泼尼龙和/或环磷酰胺静脉冲击治疗;56.52%的患者需要透析。47.83%的患者肾功能改善;8例(34.78%)患者死亡。死亡原因是感染并发症、主要疾病进展和心脏骤停。

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