原发性肾病综合征并发重症特发性急性肾衰竭的临床特征

THE SERIOUS IDIOPATHIC ACUTE RENAL FAILURE IN PATIENTS WITH PRIMARY NEPHROTIC SYNDROME: CLINICOPATHOLOGICAL FEATURES AND TREATMENT

目的:探讨原发性肾病综合征(PNS)并发重症特发性急性肾衰(iARF)的临床病理特点与治疗。方法:采用腹膜透析(PD)联合激素、免疫抑制剂、抗凝、降脂等综合治疗7例PNS并发重症iARF患者,总结分析其临床病理资料。结果:患者均有典型的NS表现,其中少尿型iARF 6例、非少尿型1例;4例伴大量腹水,血肌酐(Scr)316—1857μmol/L,5例血清纤维蛋白原升高。病理改变为局灶节段系膜增生性肾炎及弥漫系膜增生性肾炎各3例,微小病变1例。均伴有不同程度肾小管间质病变。3例iARF后肾活检者均有肾间质水肿,炎细胞浸润,其中2例小灶性肾小管上皮坏死。PD10～40天,1例死亡、6例iARF治愈;PNS完全缓解(CR)、部分缓解(PR)及无效(NR)各2例;随访15～96个月,CR4例、PR及NR各1例。结论:PNS并发iARF重症患者以少尿型为主,伴高凝状态,多见于肾小球病变较轻者,且伴小管间质的急性病变。PD联合激素、抗凝等治疗可使多数患者病情逆转。

Objective： To explore the chnicopathological features of serious idiopathic acute renal failure （iARF） in patients with primary nephrotic syndrome （PNS） and their treatments. Method： The 7 cases with PNS accompanied iARF were treated by peritonesldialysis （PD）, prednisone, i agents, anticoagulafive agent and hypolipidic agent. The dinicopatho logical data were analyzed.Results： All patients had topical, clinical manifestations of PNS, oliguric iARF in 6 patients and no - oliguric iARF in 1 patients.4 cases had mess.ascltes. Serum Creatinine was 316 - 1857μmol/L. serum fibrinogen increased in 5 cases. Among 7 cases, focal segmental mesangial proliferative nephtiris was found in 3 eases, meaangial proliferative nephtiris in 3 cases and minimal change diseaze in 1 cases. Renal tubulointerstitial changes were found in all patients.The renal interstitial edema and imflammatory infikration were present in the 3 patients whose renal biopsy was performed after iARF happened, micro focal necrosis of renal tubular epithelia was found in 2 cases. The lasting time of PD was between 10 days and 40 says. 1 patient died and iARF was cured in 6 eases.A mong these patients, the complete remisson （CR） of PNS, partial remission （PR） and no responsive （NR） to treatment were present in 2 cases respeetivdy.After the followup of 15--59 months, CR was found in 4 cases, PR and NR in 1 ease respectively. Coneloslon： The most of serious iARF in ptients with PNS are ollgurie, accompanied with hypercoagulability, happen in the patients with slight glomerular lesion, present acute tubular injury.The iARF and PNS can be ameliorated by PD, predenisone and anticoagulatlve agent.