Department of Organ Interaction Research Medicine, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kamigyo-Ku, Kyoto, Japan.
Clin Transplant. 2011 Jul;25 Suppl 23:53-8. doi: 10.1111/j.1399-0012.2011.01455.x.
A 15-yr-old girl with end-stage kidney disease caused by primary focal segmental glomerulosclerosis (FSGS) underwent a living-related donor kidney transplantation. The allograft functioned well immediately after reperfusion, but massive proteinuria exceeding 50 g/d appeared on day 3. Treatment with rituximab and plasma exchange (PE) successfully decreased the proteinuria to 10 g/d. A biopsy specimen on day 30 showed no segmental glomerulosclerosis but partial interstitial infiltration of inflammatory cells. An increased number of podocytes showed intracytoplasmic vacuolization, and an electron micrograph showed diffuse mild subendothelial edema and foot process effacement. The podocytes were hypertrophied but were not detached from the basement membrane. As the therapies used to reduce the patient's proteinuria were having a limited effect, intravenous steroid pulse therapy followed by low-density lipoprotein apheresis was performed. A biopsy specimen taken on day 120 showed no segmental glomerulosclerosis. Thrombus formation in one glomerulus and packed lymphocytes in the capillary loop of another glomerulus were detected. The patient's clinical course was compatible with FSGS recurrence. Although the early pathological changes were not typical of FSGS, they might be indicative of the primary lesion that subsequently progresses to typical FSGS.
一位 15 岁女孩因原发性局灶节段性肾小球硬化症（FSGS）导致终末期肾病，接受了活体亲属供肾移植。再灌注后，移植物的功能立即恢复良好，但第 3 天出现大量蛋白尿，超过 50g/d。利妥昔单抗和血浆置换（PE）治疗成功地将蛋白尿降至 10g/d。第 30 天的活检标本未见节段性肾小球硬化，但部分间质有炎症细胞浸润。大量的足细胞胞浆内有空泡化，电镜下可见弥漫性轻微的内皮下水肿和足突消失。足细胞肥大，但未从基底膜上脱落。由于降低患者蛋白尿的治疗效果有限，随后进行了静脉类固醇冲击治疗联合低密度脂蛋白吸附治疗。第 120 天的活检标本未见节段性肾小球硬化。一个肾小球内有血栓形成，另一个肾小球的毛细血管袢内有致密的淋巴细胞浸润。患者的临床病程与 FSGS 复发相符。尽管早期的病理变化不典型，但可能提示随后进展为典型 FSGS 的原发性病变。
