本文選題：惡性高血壓 + 血栓性微血管病��； 參考：《上海交通大學》2015年博士論文

【摘要】：目的:分析比較惡性高血壓(MHT)患者的臨床、腎臟病理、治療及預(yù)后,了解MHT的疾病特點;對其中部分患者行血漿Adamts 13及補體因子檢測以探究MHT的發(fā)病機制。方法:收集2004年1月至2014年12月上海交通大學醫(yī)學院附屬瑞金醫(yī)院腎臟內(nèi)科住院患者中臨床確診MHT者92例(包括單純MHT者81例和MHT合并TMA者11例),回顧分析這些患者的臨床、治療及預(yù)后;分析其中74例腎活檢者(包括單純MHT者68例和MHT合并TMA者6例)的腎臟病理特點;對其中50例MHT患者及另外28例TMA患者,采用酶聯(lián)免疫吸附測定(ELISA)法檢測血漿Adamts 13活性、抗Adamts 13抗體濃度、CFH(補體H因子)濃度及MAC(補體攻膜復(fù)合物)濃度。結(jié)果:92例MHT中81例為單純MHT(88.04%),11例(11.96%)為MHT合并TMA。單純MHT組和MHT合并TMA組均以原發(fā)性MHT占大多數(shù)(90.12%和81.82%),既往有高血壓病史者較多(50.62%和45.45%),均以腎臟受累癥狀起病為主(67.90%和54.54%),發(fā)病時血壓明顯升高(SBP:221.70 mm Hg和216.27 mm Hg,DBP:140.00 mm Hg和141.00 mm Hg)。單純MHT組發(fā)病年齡偏大(35.00歲vs 30.00歲),男性患者比例高(83.95%vs 54.54%),BMI指數(shù)較高(26.40 kg/m2 vs 23.91 kg/m2)。MHT合并TMA組較MHT組腎功能嚴重減退(Scr:669.00μmol/l vs 287.00μmol/l,e GFR-EPI:8.10 ml?min-1?1.73m-2vs 22.20 ml?min-1?1.73m-2),血小板數(shù)明顯減少(93.00×109/L vs 203.00×109/L),血紅蛋白量顯著下降(82.73 g/L vs 120.07 g/L),VWF升高(230.86%vs163.65%),并伴大量蛋白尿(尿常規(guī)示尿蛋白2+及以上)(100.00%vs 79.01%)。MHT治療以多種降壓藥聯(lián)合應(yīng)用為主,其中MHT合并TMA組透析(血液透析或腹膜透析)率高(63.64%vs 12.34%)。經(jīng)及時有效的治療,單純MHT組和MHT合并TMA組患者的預(yù)后較好,治愈率高(64.20%和81.82%),復(fù)發(fā)率低(6.17%和10.00%),但死亡率后者高于前者(0.00%vs 18.18%)。多因素生存分析提示女性(HR=2.730,P=0.017)、血清肌酐升高(HR=1.003,P0.001)是MHT腎臟預(yù)后的獨立危險因素,而ACEI/ARB的應(yīng)用(HR=0.393,P=0.040)能保護MHT患者的腎功能,延緩其進展。本組MHT患者腎活檢率較高;單純MHT組和MHT合并TMA組的腎活檢率分別為83.94%和54.54%。兩組患者病理特征均為中度腎小球硬化(硬化率≥50%)并伴中重度腎小管間質(zhì)病變,大部分表現(xiàn)腎小血管纖維樣增生(蔥皮樣改變)及透明樣變性,且免疫熒光沉積均陰性;其中MHT合并TMA組還多伴有腎小血管內(nèi)血栓形成(50.00%)。此外,MHT患者的腎小管間質(zhì)病變嚴重程度與其血清肌酐水平呈正相關(guān)。檢測的50例MHT患者中,32例(64.00%)血漿Adamts 13活性缺失者(酶活性5%),50例(100.00%)血漿抗Adamts 13抗體均陰性。44例單純MHT者的血漿CFH濃度明顯降低(462.13μg/m L),6例MHT合并TMA者的血漿CFH濃度正常(636.88μg/m L)。兩組患者的血漿MAC濃度均明顯升高(281.83ng/m L和288.89 ng/m L)。結(jié)論:本研究為國內(nèi)大組MHT及相關(guān)性TMA的臨床、病理及實驗檢測研究。MHT是臨床危急重癥,病情表現(xiàn)重,且發(fā)展迅速。多累及青壯年,眼、腎、心、腦為典型常見受累臟器。MHT可合并TMA,且MHT合并TMA者腎功能受累尤為嚴重。經(jīng)積極及時有效的治療干預(yù),MHT疾病預(yù)后較好,存活率高,治愈率高,復(fù)發(fā)率低。本研究發(fā)現(xiàn)Adamts 13活性下降和補體旁路途徑活化可能參與MHT的發(fā)病;血漿Adamts 13聯(lián)合相關(guān)補體因子檢測能較好地輔助MHT及相關(guān)性TMA的分層診斷。
[Abstract]:Objective: to analyze the clinical, renal pathology, treatment and prognosis of patients with malignant hypertension (MHT), to understand the disease characteristics of MHT, and to investigate the pathogenesis of Adamts 13 and complement factor in some of the patients to explore the pathogenesis of MHT. Methods: to collect the hospitalization of the Department of Nephrology, Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine from January 2004 to December 2014. 92 patients with clinically confirmed MHT (including 81 patients with MHT and 11 MHT with TMA) were reviewed to analyze the clinical, treatment and prognosis of these patients, and to analyze the renal pathological features of 74 renal biopsy subjects (including 68 cases of MHT and 6 MHT with TMA), and 50 cases of MHT and 28 cases of TMA, using enzyme linked immunosorbent assay. The activity of plasma Adamts 13, the concentration of anti Adamts 13 antibody, the concentration of CFH (complement H factor) and the concentration of MAC (complement tapping complex) were detected by ELISA. Results: 81 cases of MHT in 92 cases were pure MHT (88.04%), 11 cases (11.96%) were MHT combined with TMA. simple MHT group and MHT combined group were the majority (90.12% and 81.82%), and had high blood before. There were more patients (50.62% and 45.45%) with the onset of renal involvement (67.90% and 54.54%), and blood pressure increased significantly (SBP:221.70 mm Hg and 216.27 mm Hg, DBP:140.00 mm Hg and 141 mm Hg). The age of the simple MHT group was higher (35 years of vs 30 years), and the proportion of male patients was higher (54.54%), and the index was higher (26.40 The renal function of M2 vs 23.91 kg/m2).MHT combined with TMA group was significantly lower than that in group MHT (Scr:669.00 mu mol/l vs 287 micron mol/l, e GFR-EPI:8.10), and the number of platelets decreased significantly (93 * 203 * 203), and the amount of hemoglobin decreased significantly. A large number of albuminuria (urine routine urinary protein 2+ and above) (100.00%vs 79.01%) (100.00%vs 79.01%) was used in combination with a variety of hypotensive drugs. The rate of MHT combined with TMA group (hemodialysis or peritoneal dialysis) was high (63.64%vs 12.34%). After timely and effective treatment, the prognosis of patients with simple MHT group and MHT combined with TMA group was better (64.20% and 81.). 82%), the recurrence rate was low (6.17% and 10%), but the mortality rate was higher than that of the former (0.00%vs 18.18%). Multiple factor survival analysis suggested that women (HR=2.730, P=0.017), serum creatinine (HR=1.003, P0.001) were independent risk factors for MHT renal prognosis, and ACEI/ARB should be used (HR=0.393, P=0.040) to protect the renal function of MHT patients and postpone its progress. The renal biopsy rate of MHT patients was higher. The renal biopsy rates in group MHT and group TMA were 83.94% and 54.54%. two, respectively, and the pathological features were moderate glomerulosclerosis (sclerotic rate > 50%) and moderate to severe tubulointerstitial lesions, most of which showed renal small vascular fibroid hyperplasia (scallion dermoid change) and hyaline degeneration, and immunofluorescence precipitation. The product was negative, among which MHT combined with TMA was accompanied by small intravascular thrombus formation (50%). In addition, the severity of renal tubulointerstitial lesions in MHT patients was positively correlated with serum creatinine levels. In 50 MHT patients, 32 cases (64%) had plasma Adamts 13 activity deficiency (enzyme activity 5%), 50 cases (100%) of plasma anti Adamts 13 antibody was negative. The plasma CFH concentration in.44 patients with simple MHT was significantly decreased (462.13 g/m L), and the plasma CFH concentration in 6 cases with TMA was normal (636.88 mu g/m L). The plasma MAC concentration in group two was significantly increased (281.83ng/m and 288.89). The bed is critical and severe, and its condition is heavy and developing rapidly. The typical common affected organs.MHT can be combined with TMA in the eyes, kidney, heart and brain, and the renal function of MHT combined with TMA is especially serious. After active and effective treatment, the prognosis of MHT is better, the survival rate is high, the cure rate is high, and the recurrence rate is low. This study found Adamts 13 live. The reduction of sex and the activation of complement pathway may be involved in the pathogenesis of MHT, and the detection of plasma Adamts 13 combined with related complement factor can better assist the stratified diagnosis of MHT and associated TMA.
【學位授予單位】：上海交通大學
【學位級別】：博士
【學位授予年份】：2015
【分類號】：R544.1

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