摘要
目的:探讨肾移植术后复发性局灶节段性肾小球硬化(FSGS)的临床病理特点及其转归,并与自体肾FSGS进行比较。方法:结合临床确诊的3例复发性FSGS的临床表现、病理特点及转归,回顾分析有自体肾、供肾和移植肾肾活检结果。结果:(1)自体肾FSGS的临床特点:3例患者自体肾FSGS的起病年龄分别为17岁(男)、23岁(女)、40岁(女),肾活检时尿蛋白定量分别为7.55g/24h,10.45g/24h,14.26g/24h,血清肌酐分别为99.0μmol/L、105.2μmol/L、265.2μmol/L。进展至ESRD的病程分别为4年、2年、4年。(2)自体肾FSGS的病理特点:3例患者球性/节段硬化的比例分别为5.3%/42.1%,0/6.9%,40%/33.3%,肾小管间质急性/慢性病变分别为0/10%、10%/0、10%/30%。例2和例3足突广泛性融合(>50%)(例1未留取电镜组织)。例1和例3为细胞型,例2为顶部型,(3)复发性FSGS的临床特点:例1和例2分别于移植术后10d、40d出现蛋白尿(分别为18.27g/24h,7.55g/24h),病例3不详;并分别在移植后130d、67d、70d经肾活检确诊为FSGS,活检时尿蛋白定量分别为:10.13g/24h、15.8g/24h、4.38g/24h,血肌酐为114.0μmol/L、484.4μmol/L、701.8μmol/L。(4)复发性FSGS的病理特点及分型:早期仅表现为足突广泛融合,无节段硬化,重复活检出现节段硬化,并伴有明显的肾小管间质急性病变;例1和例3为细胞型,移植后复发仍为细胞型,例2为顶部型,术后复发时表现为塌陷型。(5)复发性FSGS的转归:例1行血浆置换,蛋白尿部分缓解,血肌酐上升缓慢,例2行免疫吸附治疗,效果不佳,3个月时移植肾功能丧失,例3失随访。结论:复发性FSGS患者临床亦表现为大量蛋白尿,并伴有肾功能不全,病理生理机制可能与自体肾脏病变有关;FSGS患者移植后早期出现蛋白尿时,应及时进行移植肾活检,光镜下可无肾小球节段硬化,但超微结果可表现为足突广泛融合,而重复肾活检则出现肾小球节段硬化;FSGS复发后其病理分型可以与自体
Objective：In this report, we evaluated the clinical and histopathological characteristics and sequelae of posttransplant recurrent focal segmental glomeruloselerosis （FSGS）. Methodology： A retrospective analysis was carried out in three posttransplant recurrent FSGS patients. The renal biopsy of native, donor and allograft were performed to evaluated histopathologic features. The clinical and laboratory data, and response to treatment were recorded in detail. Results ： （ 1 ） There were one male and two females. Their onset ages were 17, 23, and 40 yrs. The levels of 24h urinary proteinuria excretion as native renal biopsy were 7. 55, 10. 5 and 14. 26 g/24h. The levels of serum ereatinine were 99, 105.2 and 265.2 μmol/L. The time of progression to ESRD were 4, 2 and 4yrs, respectively. （2）Proportion of glomeiulax/segmental scterosing lesions were 5.30%/42. 1%, 0/6. 90% and 40%/33.3 % , acute/chronic tubulointerstitial lesion were 0/10% , 10%/0 and 10%/30%. We observed the diffuse foot process effacement（ 〉 50% ） by electron microscopy in case 2 and 3 of native renal biopsy, while no tissue for TEM in case 3. FSGS variants in native kidneys were cellular, tip lesion, and cellular type, respectively. （3）Proteinuria occurred at 10 and 40 days after transplantation in ease 1 and 2 （ 18.3 and 7.55g/24h） , 3 cases were diagnozed recurrent FSGS at 130, 67 and 70 days after transplantation. The levels of 24h urinary proteinuria excretion were 10. 1, 15.8, and 4. 38g/24h. The levels of serum creatinine were 114.0 μmol/L, 484.4μmol/L and 701.8 μmol/L.. （4） Early graft biopsies might show normal appearing glomeruli by light microscopy but diffuse foot process effacement by electron microscopy. Segmental sclcrosing lesions may occur later in repeated renal biopsy, with acute tubulointerstitial lesion; FSGS variants in graft kidneys are cellular, collapsing, cellular, respectively. （5） Case 1 was treated with 5 cycles of plasmapheresis, and achieved a partial remission; case 2 was r
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2010年第2期125-134,共10页
Chinese Journal of Nephrology,Dialysis & Transplantation
关键词
局灶节段性肾小球硬化
肾移植
复发
肾病综合征
蛋白尿
focal segmental glomerulosclerosis renal transplantation recurrence nephrotic syndrome proteinuria
