Groff J A, Kozak M, Boehmer J P, Demko T M, Diamond J R
Department of Medicine, Pennsylvania State University, Hershey, USA.
Am J Kidney Dis. 1997 Feb;29(2):280-4. doi: 10.1016/s0272-6386(97)90042-1.
Hemolytic uremic syndrome (HUS) is a rare, often fatal complication of mitomycin C therapy. It is generally accepted that HUS is, in part, caused by endothelial cell dysfunction. Endothelial cells modulate blood flow, blood pressure, and myointimal proliferation. Endothelial cells synthesize and release products that modulate vascular tone and regulate vascular smooth muscle cell growth. We describe a patient who developed HUS secondary to mitomycin C, resulting in end-stage renal disease and necessitating chronic hemodialysis. Over several months, the patient subsequently developed multisystem organ failure involving the heart, liver, and intestine that was associated with angiographically documented small, distal vessel occlusive disease and ultrasonographically identified coronary artery intimal hyperplasia. We propose that a diffuse ongoing endothelial cell dysfunction (ie, endotheliopathy) is the putative mechanism for this patient's clinical course. To our knowledge, this continuum of HUS presenting as a multisystem, progressive disorder has not been previously reported.
溶血性尿毒症综合征（HUS）是丝裂霉素C治疗罕见且常致命的并发症。人们普遍认为，HUS部分是由内皮细胞功能障碍引起的。内皮细胞调节血流、血压和肌内膜增殖。内皮细胞合成并释放调节血管张力和调节血管平滑肌细胞生长的产物。我们描述了一名继发于丝裂霉素C而发生HUS的患者，导致终末期肾病并需要进行慢性血液透析。在几个月的时间里，该患者随后出现了涉及心脏、肝脏和肠道的多系统器官衰竭，这与血管造影记录的小的远端血管闭塞性疾病以及超声检查发现的冠状动脉内膜增生有关。我们认为弥漫性持续的内皮细胞功能障碍（即内皮病变）是该患者临床病程的假定机制。据我们所知，此前尚未报道过HUS表现为多系统进行性疾病的这种连续情况。
