發(fā)布時間：2018-12-17 23:32

【摘要】：目的分析系統(tǒng)性硬化癥腎危象(SRC)合并血栓性微血管病(TMA)的臨床特點。方法收集2010年1月~2015年11月診治的SRC患者4例,其中3例合并TMA,回顧性分析其臨床資料,并對相關(guān)文獻進行復習。結(jié)果 3例中女2例、男1例,年齡分別為59、67、76歲;SSc病程分別為1、2、13年,1例男性為彌漫型、2例女性為局限型,SRC罹患時間為2~4 d。3例均有急劇血壓升高、腎功能不全、乳酸脫氫酶升高、血小板減少,外周血可見破碎紅細胞,2例Coomb's試驗陰性。3例均予以血管緊張素轉(zhuǎn)換酶抑制劑,行連續(xù)腎臟替代療法、靜脈輸注新鮮冰凍血漿、血漿置換各1例。治療后病情均好轉(zhuǎn),1例1年后死亡。結(jié)論 SRC合并TMA并不少見,進展較快,病情兇險,應(yīng)及早判斷;治療除應(yīng)用血管緊張素轉(zhuǎn)換酶抑制劑外,要進行腎臟替代治療,必要時輸注新鮮冰凍血漿。
[Abstract]:Objective to analyze the clinical features of renal crisis (SRC) complicated with thrombotic microangiopathy (TMA) in systemic sclerosis. Methods from January 2010 to November 2015, 4 patients with SRC were collected. The clinical data of 3 patients with TMA, were analyzed retrospectively, and the related literatures were reviewed. Results among the 3 cases, 2 cases were female, 1 case was male, the age was 59 years old and 67 years old, respectively. The course of SSc was 1 and 13 years, one male was diffuse type and two female were localized type. The onset time of SRC was 2d 4 in 3 cases, all had sharp blood pressure, renal insufficiency, lactate dehydrogenase and thrombocytopenia. The broken red blood cells were found in peripheral blood, 2 cases were negative in Coomb's test, 3 cases were treated with angiotensin converting enzyme inhibitor, followed by continuous renal replacement therapy, intravenous infusion of fresh frozen plasma, and plasma exchange in 1 case each. One case died one year later. Conclusion SRC combined with TMA is not uncommon, rapid progression and dangerous condition, should be judged early, in addition to the use of angiotensin converting enzyme inhibitors, renal replacement therapy, if necessary, infusion of fresh frozen plasma.
【作者單位】： 山東省千佛山醫(yī)院;
【分類號】：R593.2

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