原发性局灶节段性肾小球硬化的临床病理及预后

CLINICAL AND PATHOLOGICAL STUDY OF FOCAL SEGMENTAL GLOMERULOSCLEROSIS

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 investigate the clinical and pathological features and prognosis of primary focal segmental glomerulosclerosis. METHODOLOGY 114 adult FSGS patients biopsied between 1986 and 1996 were included in this study.Retrospective analysis of the clinical and pathological feature was made.Among them,45 patients were followed up for 32 47±24 49 months. RESULTS (1)Primary FSGS accounts for 2 62% of the total biopsied cases in this series.The average age of these patients was 28 54±12 34 years.(2)The most frequent manifestation of primary FSGS was proteinuria (93 0%);nephritic syndrome was presented in 1 25% of the patients;hematuria in 51 8% (marcrohematuria 14 90%,microhematuria 36 8%)and hypertension in 43 8% of the patients.Renal imsufficiency occurred in 47 4% of the patients at the time of renal biopsy.22 4% developed into uremia during the period of follow up for 32 47±24 49 months.(3)Compared to patients with normal renal function,patients with renal failure had higher blood pressure and lower urinary osmolarity,the rate of global and focal segmental sclerosis was significantly increased.Tubulointerstitial damage and cell infiltration were more severe in patients with renal failure.The responsive rate to prednison treatment was less than 10%. CONCLUSION ①Younger patients were inclined to primary FSGS.②Proteinuria is the most frequent clinical manifestation.Hypertension,focal segmental sclerosis,tubular interstitial fibrosis or cellular infiltration were associated with poor prognosis.③Response to prednison treatment is low and attention must be paid to the control of hypertension and protection of renal function.