成人特发性局灶节段性肾小球硬化病248例临床病理分析

目的 重新评价肾活检标本中特发性局灶节段性肾小球硬化病（FSGS）的发生率、主要临床及病理特点。方法 收集1994年7月～2000年7月间248例特发性FSGS的患者，观察肾组织的光镜、 荧光及电镜的特点，根据肾小球病变将特发性FSGS分为3种亚型。结果 248例成人特发性局灶节段肾小球硬化占同期肾活检的12.4%，占成年人原发性肾小球疾病的19.0%，以18～30岁为发病高峰年龄（约占44.4%）。临床表现为不同程度的蛋白尿，以肾病综合征为表现者143例（占57.7%)。FSGS的3种病理亚型中，经典型FSGS仍占绝大多数（91.5%)，尖端型为4.4%，塌陷型为4.1%，肾活检时，约1/3以上为肾功能不全（约占35.1%）。13例尿毒症中76.9%病理亚型为塌陷型。结论 局灶节段性肾小球硬化病约占原发性肾小球病1/5，FSGS患者肾活检时，除表现为蛋白尿、肾病综合征外，肾功能不全也较常见。塌陷型FSGS常表现为进行性的不可逆的肾功能衰竭，其预后差。

Clinicopathologic study of 248 patients with FSGS

Objective To analyze the incidence, clinical presentations and pathological changes of patients withprimary focal segmental glomerulosclerosis (FSGS). Methods 248 patients with biopsy proven primary FSGS wereenrolled. They were hospitalized during the period of 1994～ 2000. All tissues were evaluated by LM, IF and EM.They were divided into three subgroups based on glomerular changes. Results The incidence of adult primary FSGSwas 12.4% in all the biopsied patients in our hospital. About 19.0% of primary glomerular nephritis were FSGS, inwhich about 44.4 % of patients aged 18-30. The patients presented with nephrotic syndrome (143/248) or varying degrees of proteinuria (105/248). More than 1/3 of patients had renal failure, 14.9 % (13/248) patients had uremia. Among the subgroups of pathologic changes, 91.5% of patients had classical FSGS, 4.4% glomerular tip lesion and 4.1% collapsing glomerulo-nephropathy. Conclusion FSGS occupies a high percentage in primary glomerular nephritis(1/5). Renal insufficiency is common, whereas peogressive irreversible renal failure is frequently seen in collapsingglomerular nephropathy with poor prognosis. (Shanghai Med J, 2001,24:89-91)