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报告6例IgA肾病,有以下特点:青年男性,病程短,在短期内迅速进展至肾功能衰竭(肾衰),甚至终末期肾衰;大量蛋白尿伴血浆白蛋白下降,呈肾病综合征或肾病综合征倾向;本组4例以急进性或恶性高血压为首发表现。肾活检1例为新月体性肾炎,5例有肾小球硬化伴新月体形成,小管间质病变严重,免疫荧光示IgA在系膜区弥漫性沉积,IgM在毛细血管壁沉积。肾脏病理为判断此类IgA肾病的预后提供了依据。治疗上,应严格控制血压,并作好透析或肾移植的准备。
Report 6 cases of IgA nephropathy, has the following characteristics: young men, short duration, rapid progress in the short term to renal failure (renal failure), and even end-stage renal failure; massive proteinuria with decreased plasma albumin, nephrotic syndrome or Nephrotic syndrome tendency; 4 cases of this group with acute or malignant hypertension as the first performance. One case of renal biopsy was crescentic nephritis, 5 cases had glomerulosclerosis with crescent formation, tubulointerstitial lesions were severe, immunofluorescence showed diffuse deposition of IgA in mesangial area, IgM deposition in the capillary wall. Kidney pathology to determine the prognosis of IgA nephropathy provides the basis. Treatment should be strictly controlled blood pressure, dialysis or kidney transplantation and make preparations.