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目的:分析血栓性微血管病(TMA)肾脏损害临床及病理特点,探讨临床及病理改变与预后的关系。方法:27例临床及肾活检明确诊断的TMA患者,男性12例,女性15例,年龄12~53岁。分析其病因、临床表现、实验室检查特点、病理改变特征及预后。结果:起病诱因:4例有明确腹泻史;3例有丝裂霉素化疗史;2例妊娠后起病;2例食物中毒史;1例蛇咬伤,15例未发现明确病因。临床均有贫血;外周血红细胞碎片阳性;乳酸脱氢酶升高;血小板减少;肾功能不全;21例患者需行肾脏替代治疗;21例有明显高血压,其中6例达到恶性高血压。有5例患者病程中出现神经或精神症状。肾脏组织学改变主要有二种类型:14例病变以间质血管变为主,病变在小叶间动脉和入球动脉,表现为内膜增生、增厚,粘液性水肿,血栓形成,血管腔明显狭窄、闭锁,肾小球明显缺血性皱缩,其中2例为肾皮质坏死。13例以肾小球病变为主,病变主要在肾小球和入球动脉,肾小球内皮细胞肿胀,与基膜分离,内皮下增宽,分层。随访1年,6例患者肾功能完全恢复正常,11例患者进入终末期肾功能衰竭,其余10例患者仍持续慢性肾功能不全。结论:TMA的病因复杂,典型腹泻相关的溶血性尿毒症综合征少见,多数患者病因并不明确。TMA患者的肾脏损害重,预后差,近半数患者1年左右进入终末期肾衰。TMA预后与病因及间质血管病变的轻重有关。
Objective: To analyze the clinical and pathological features of renal damage in thrombotic microangiopathy (TMA) and to explore the relationship between clinical and pathological changes and prognosis. Methods: TMA was diagnosed in 27 cases with clinical diagnosis and renal biopsy. There were 12 males and 15 females, aged from 12 to 53 years old. Analysis of its etiology, clinical manifestations, laboratory features, pathological changes and prognosis. Results: The causes of onset: 4 cases had a clear history of diarrhea; 3 cases of mitomycin chemotherapy history; 2 cases of pregnancy onset; 2 cases of food poisoning history; 1 case of snake bites, 15 cases did not find a clear cause. Clinical anemia; peripheral red blood cell fragments were positive; elevated lactate dehydrogenase; thrombocytopenia; renal insufficiency; 21 patients required renal replacement therapy; 21 patients had significant hypertension, of which 6 cases of malignant hypertension. Five patients had neurological or psychiatric symptoms during their course of disease. Histological changes in kidney there are two main types: 14 cases of interstitial vascular lesions dominated lesion in the interlobular arteries and into the artery, manifested as intimal hyperplasia, thickened, myxedema, thrombosis, blood vessels were significantly Stenosis, atresia, glomerular obvious ischemic collapse, of which 2 cases of renal cortical necrosis. Thirteen cases were mainly glomerular lesions. The lesions were mainly in the glomeruli and the afferent artery. The glomerular endothelial cells were swollen, separated from the basilar membrane, widened and stratified under the endothelium. After one year of follow-up, renal function of 6 patients returned to normal completely, 11 patients entered end-stage renal failure, and the remaining 10 patients continued to have chronic renal insufficiency. Conclusion: The etiology of TMA is complex. Typical diarrhea-associated hemolytic uremic syndrome is rare, and the etiology of most patients is not clear. TMA patients with severe kidney damage, poor prognosis, nearly half of patients about 1 year into the terminal renal failure. TMA prognosis and etiology and severity of interstitial vascular disease related.