电子致密物沉积病的临床及病理研究

目的：探讨电子致密物沉积病（DDD）的临床及病理特点。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例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C3降低，2例表现为慢性肾炎综合征，组织学1例呈MPGN伴有血C3降低；另1例呈系膜增生性病变，血C3正常，4例免疫荧光表现为以C3为主沿毛细血管壁呈颗粒状沉积，并有系膜区闭块状沉积，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。

Clinicopathologic characteristics of dense deposit disease

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.