Liu Gang, Zhang Zhi-yong, Zou Wan-zhong, Wang Hai-yan
Department of Nephrology, Peking University First Hospital, Beijing 100034, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2004 Apr;36(2):150-3.
To investigate the relative frequency of idiopathic focal segmental glomerulosclerosis (FSGS) in renal biopsy-proven diseases, and its criteria and significance of clinicopathological diagnosis.
We reviewed all the inpatients that were diagnosed by renal biopsies from 1990 to 2001, of whom 65 were identified as idiopathic FSGS. Their histological characteristics were analyzed together with their clinical findings.
(1) The incidence of idiopathic FSGS accounted for 2.2% of all the renal biopsies, 3.2% of primary glomerular diseases, and 5.8% of patients with massive proteinuria. (2) The subtypes of idiopathic FSGS were hilar lesion (12.3%), peripheral lesion (23.1%), mixture lesion (60.0%), collapsing lesion (3.1%), and tip lesion (1.5%), which were frequently accompanied by other morphologic variants, such as synechia of Bowman's capsule, podocyte hyperplasia and hypertrophy, segmental endothelial and mesangial proliferation, and interstitial fibrosis, etc. As the sclerotic lesions distributed segmentally and overlapped by other variants, it was difficult to get the correct diagnosis. (3) Most of the patients with segmental endothelial and mesangial proliferation had massive proteinuria.
Idiopathic FSGS was not a common glomerular disease in our study. Podocyte lesion, segmental endothelial and mesangial proliferation may play an important role in the formation of segmental sclerosis in idiopathic FSGS.
探讨特发性局灶节段性肾小球硬化（FSGS）在肾活检确诊疾病中的相对发生率及其临床病理诊断标准和意义。
回顾1990年至2001年经肾活检确诊的所有住院患者，其中65例被诊断为特发性FSGS。分析其组织学特征及临床 findings。
（1）特发性FSGS的发生率占所有肾活检的2.2%，原发性肾小球疾病的3.2%，大量蛋白尿患者的5.8%。（2）特发性FSGS的亚型为肾门病变（12.3%）、外周病变（23.1%）、混合病变（60.0%）、塌陷病变（3.1%）和顶端病变（1.5%），常伴有其他形态学变异，如鲍曼囊粘连、足细胞增生肥大、节段性内皮和系膜增生以及间质纤维化等。由于硬化病变呈节段性分布且与其他变异重叠，难以做出正确诊断。（3）大多数节段性内皮和系膜增生患者有大量蛋白尿。
在我们的研究中，特发性FSGS不是常见的肾小球疾病。足细胞病变、节段性内皮和系膜增生可能在特发性FSGS节段性硬化的形成中起重要作用。
