Sens Y A, Miorin L A, Silva H G, Malheiros D M, Filho D M, Jabur P
Department of Medicine, Faculdade de Ciencias Medicas, Santa Casa de São Paulo, Brazil.
Ren Fail. 1997 Mar;19(2):279-82. doi: 10.3109/08860229709026289.
Hemolytic uremic syndrome is characterized by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and renal failure. Clinical/ pathologic data, along with the treatment and outcome of 8 adult patients with HUS, are described. There were 7 females and 1 male, age 30.7 +/- 12 years; 7 were White and 1 Black. Three patients were kidney graft recipients, 2 of whom were receiving cyclosporine; 2 patients were postpartum; 1 case followed an abortion; 1 occurred with prodromic infection; and 1 case was without a causal factor. All patients presented with hematuria and 6 with oligoanuria. Laboratory data showed hemolytic anemia with schistocytes, LDH values were 2584 +/- 2191 U/L, platelets were 79,000 +/- 40,000/mL, creatinine concentrations were 5.9 +/- 2.5 mg/dL. Renal biopsy showed thrombotic microangiopathy. Two had predominant glomerular involvement. 2 showed renal cortical necrosis, 4 were marked by predominant arteriolar involvement. In 5 patients dialytic therapy was performed. All were treated with fresh-frozen plasma infusion and 6 with plasmapheresis. Three patients died, 2 without recovery of renal function. In conclusion, the trigger events were related to renal transplant in 3.2 of them taking cyclosporine; 3 with pregnancy; 1 to precedent infection; and 1 with no causal factor. There was no correlation between histological form and outcome in this group of patients. The benefit of plasmapheresis was evident in the recovery of the extrarenal manifestations, although it did not change the renal outcome. The prognosis is poor, with a high mortality (37.5%) and/or end-stage renal failure (37.5%). Complete recovery of renal function was obtained in 25%.
溶血尿毒综合征的特征是同时出现溶血性贫血、血小板减少和肾衰竭。本文描述了8例成年溶血尿毒综合征患者的临床/病理资料以及治疗情况和预后。患者中7例为女性，1例为男性，年龄30.7±12岁；7例为白人，1例为黑人。3例患者为肾移植受者，其中2例正在接受环孢素治疗；2例患者为产后；1例患者在流产后发病；1例患者发病前有感染；1例患者无诱发因素。所有患者均有血尿，6例患者出现少尿。实验室检查数据显示存在伴有裂体细胞的溶血性贫血，乳酸脱氢酶值为2584±2191 U/L，血小板为79,000±40,000/mL，肌酐浓度为5.9±2.5 mg/dL。肾活检显示血栓性微血管病。2例以肾小球受累为主。2例显示肾皮质坏死，4例以小动脉受累为主。5例患者接受了透析治疗。所有患者均接受了新鲜冷冻血浆输注治疗，6例患者接受了血浆置换治疗。3例患者死亡，2例患者肾功能未恢复。总之，诱发事件中3例与肾移植有关，其中2例正在服用环孢素；3例与妊娠有关；1例与先前感染有关；1例无诱发因素。在这组患者中，组织学类型与预后之间无相关性。血浆置换对于肾外表现的恢复有明显益处，尽管它并未改变肾脏预后。预后较差，死亡率高（37.5%）和/或终末期肾衰竭发生率高（37.5%）。25%的患者肾功能完全恢复。
