目的：分析１１４ 例原发性局灶节段性肾小球硬化（ Ｆ Ｓ Ｇ Ｓ） 患者的临床、病理、免疫病理特点及预后。方法：回顾性分析１９８５ ～１９９６ 年１２ 年间，１１４ 例原发性 Ｆ Ｓ Ｇ Ｓ患者的临床表现，病理，免疫病理特点，以及其中４５例长期随访结果。　结果：１９８５ ～１９９６ 年１２ 年间，原发性 Ｆ Ｓ Ｇ Ｓ 占同期肾活检的２６２ ％ ，平均发病年龄２８５４ ±１２３４ 岁。临床表现为蛋白尿者占９３０ ％ （ 肾病综合征范围蛋白尿占２１９ ％ ） ，血尿５１８ ％ （ 肉眼血尿１４９ ％ ，镜下血尿３６８ ％ ） ，高血压４３８ ％ 。肾活检时已发生肾功能不全者占４７４ ％ ，随访中２２２ ％ 发展至尿毒症，激素治疗有效率小于１０ ％ 。肾功能不全者与肾功能正常者相比，前者高血压更为常见（ Ｐ＜ ００５） ，小管间质病变重［ 尿渗量明显降低（ Ｐ＜ ００５） ，尿溶菌酶升高（ Ｐ＜ ０００１）］ 。肾小球全球硬化的比率显著升高（ Ｐ＜ ０００１） ，节段性硬化病变更为明显，并伴有更为显著的小管间质损伤，大量炎细胞浸润。　结论：①原发性 Ｆ Ｓ Ｇ Ｓ好发于中青年，病程隐匿，进展较快；②临床上蛋白尿最为常见，其次是血尿，高血压和? OBJECTIVE: To analyze the clinical, pathological, immunopathological features and prognosis of 114 patients with primary focal segmental glomerulosclerosis (F S G S). Methods: The clinical manifestations, pathology, immunopathological characteristics of 114 patients with primary F S G S during the 12 years from 1985 to 1996 were retrospectively analyzed, and 45 of them were followed up for a long time. Results: During the 12 years from 1985 to 1996, the primary F S G S accounted for 262% of the patients with the same stage of renal biopsy and the average age of onset was 2854 ± 1234 years. Clinical manifestations of proteinuria accounted for 93  0% (nephrotic syndrome range proteinuria accounted for 21  9%), hematuria 51  8% (gross hematuria 14  9%, microscopic hematuria 36  8%), hypertension 43  8%. Kidney biopsy occurred in patients with renal insufficiency accounted for 47  4%, followed up 22  2% to uremia, hormone therapy, less than 10%. Renal insufficiency and renal function were normal, the former is more common high blood pressure (P <0  05), tubulointerstitial disease [urine volume was significantly lower (P <0  05), urine lysozyme increased (P <0001)]. Glomerular global hardening significantly increased (P <0001), segmental sclerosis more obvious, accompanied by more significant tubulointerstitial damage, a large number of inflammatory cell infiltration. Conclusions: Primary F S G S occurs predominantly in middle-aged and young patients, with an insidious course of disease and rapid progression; ② Clinical proteinuria is the most common, followed by hematuria, hypertension,