摘要
目的 探讨电子致密物沉积病（ＤＤＤ）的临床及病理待点。方法 通过５例ＤＤＤ的临床及病理资料并结合文献复习，对其临床表现和组织形态学的多样性以及与治疗和预后的关系进行综合分析。结果ＤＤＤ占经肾活检证实的原发性肾小球疾病的０．２％，占膜增殖性肾炎的２％。５例中的３冽临床表现为肾病综合征，组织学呈膜增生性肾炎（ＭＰＧＮ），并伴有血Ｃ＿３降低。２例表现为慢性肾炎综合征，组织学 １例呈 ＮＩＰＧＮ伴有血 Ｃ＿３降低；另 １例呈系膜增生性病变，血 Ｃ＿３正常。４例免疫荧光表现为以Ｃ＿３为主沿毛细血管壁呈颗粒状沉积，并有系膜区团块状沉积．电镜下均可见电子致密物在肾小球毛细血管基底膜呈弥漫、均匀沉积，其中４例包曼囊及肾小管基底膜亦可见弥漫或节段电子致密物沉积。２例呈肾病综合征表现者对糖皮质激素治疗不敏感。结论 电子致密物沉积病是超微结构的诊断，其临床表现呈多样性，大量蛋白尿、严重高血压和较重的病理改变预示其预后不佳。
Objective To investigate the clinicopathologic characteristics of dense deposit disease (DDD) . Methods Clinical and pathologic data from 5 patients with DDD were collected and related references were reviewed Results Among 2499 patients with pathologically proven primary glomerular dieases DDD accounted for 0. 2% and 2% of membranoproliferative glomerulonephritis(MPGN). Three of the 5 DDD patients with nephrotic syndrome had MPGN. Other 2 patients with chronic nephritic syndrome had MPGN or mesangial proliferative glomerulonephritis respectively. Depressed serum C _3 were present in patients with MPGN. Immunofluorescent revealed granular C_3 deposit heavily alone capillary wall of glomeruli and large globular masses of C_3 in many mesangial areas in 4 patients. By electron microscopy 5 patients had the characteristic alterations of diffuse and uniform electron-dense deposits in the glomerular capillary basement membranes. The electron-dense transformation also affected the basement membranes of Bowman's capsules and proximal tubules in 4 patients. Predisone did not show effect on the treatment in 2 DDD patients with nephrotic syndrome. Conclusions DDD has variable clinical features with different prognosis. Electron microscopic examination to demonstrate the intramembranous dense deposits is definitive diagnosis. Massive proteinuria, severe hypertension and pathologic changes may be unfavorable prognostic factors.
出处
《中华肾脏病杂志》
CAS
CSCD
北大核心
2001年第1期16-19,共4页
Chinese Journal of Nephrology
