妊娠相关血栓性微血管病患者的临床和病理特征

目的:探讨妊娠相关血栓性微血管病(TMA)的临床病理特点、治疗和预后。方法:回顾性分析2004年1月至2014年3月南京军区南京总医院肾脏科11例妊娠相关性TMA患者的临床和病理资料。结果:11例患者中有2例血栓性血小板减少性紫癜(TTP),5例产后溶血尿毒综合症(HUS),1例溶血、肝酶升高、血小板减少(HELLP)综合症,3例重度子痫前期,发病时间为妊娠中晚期或产后1周内,母亲均存活,死胎5例。11例患者均达到急性肾损伤(AKI)3期;内皮功能检测提示,患者存在不同程度的内皮功能损伤。8例患者行肾脏活检病理检查,7例见间质血管病变,主要累及小叶间动脉、小动脉及入球动脉,表现为纤维素样坏死、血栓形成、内皮细胞肿胀、动脉葱皮样改变。11例患者均接受连续性血液净化治疗,5例产后HUS患者接受血浆置换,2例TTP患者接受血浆输注及免疫抑制剂治疗,5例产后HUS患者和1例HELLP综合征患者肾功能均恢复正常,其余患者进入慢性肾功能不全,其中2例需维持性血液透析治疗。结论:妊娠相关性TMA是影响产妇肾脏功能和胎儿生命的严重疾病,内皮损伤在妊娠相关性TMA中发挥重要作用;肾脏预后与血管病变程度相关。区分妊娠相关性TMA的病因,进行及时有效的个体化治疗是影响妊娠相关性TMA预后的重要因素。     

Clinical features of malignant hypertension with thrombotic microangiopathy

Thrombocytopenia, microangiopathic hemolytic anemia, and elevated serum lactate dehydrogenase (LDH) clinically characterize thrombotic microangiopathy (TMA), which is frequently recognized among patients with malignant hypertension (MH). Sixteen consecutive patients with MH were retrospectively investigated over a 7-year period and clinical features of the subjects with TMA were evaluated. We confirmed TMA relevant to MH by the normalization of the platelet count and LDH after adequate blood pressure (BP) control was achieved. Thrombotic microangiopathy was found in 7 (44%) of 16 patients. All 7 patients had an elevated plasma renin activity (PRA). Although no significant differences were observed in PRA, the patients with TMA had a significantly higher plasma aldosterone (ALDO) (median: 403 pg/ml; IR: 305 to 568) in comparison to those without TMA (median: 220 pg/ml; IR: 147 to 287; p = 0.013). Overall, ALDO correlated with LDH (r = 0.634, p = 0.0095). However, no significant association was observed between PRA and LDH (r = 0.336, p = 0.2263). The median platelet count nadir of the patients with TMA was 8.4 × 10(4) per μl (IR: 7.15 to 9.95). Thrombocytopenia and elevated LDH were normalized, along with a gradual improvement of BP within an average of 5 days and 21.7 days, respectively. These results suggest that ALDO, but not PRA, may act as a potent indicator of the magnitude of vascular and organ damage related to TMA among patients with malignant hypertension (MH).     

不同亚型狼疮性肾炎患者临床病理特征和预后的比较

目的:比较Ⅲ型与Ⅲ+Ⅴ型、Ⅳ型与Ⅳ+Ⅴ型及Ⅳ-S亚型与Ⅳ-G亚型狼疮性肾炎(LN)患者的临床病理特点和肾脏远期预后。方法:收集南京军区南京总医院肾脏科肾脏病登记系统中经肾活检明确诊断为增生型LN、随访时间≥1年的汉族成年患者肾活检时的临床和病理资料。观察终点为终末期肾病(ESRD)。病理分型参照ISN/RPS 2003病理分型方案。结果:Ⅲ型LN患者10年和20年肾脏生存率分别为92.1%和72.2%,Ⅲ+Ⅴ型分别为91.5%和88.9%,Ⅳ型分别为82.7%和60.3%(其中Ⅳ-S亚型分别为86.2%和69.6%;Ⅳ-G亚型分别为81.9%和58.9%),Ⅳ+Ⅴ型分别为82.7%和54.7%。Ⅲ+Ⅴ型和Ⅳ+Ⅴ型LN患者尿蛋白分别重于Ⅲ型和Ⅳ型患者;病理上慢性化病变分别重于Ⅲ型和Ⅳ型患者;急性病变分别轻于Ⅲ型和Ⅳ型患者。Ⅳ-S亚型LN患者抗中性粒细胞胞质抗体阳性率高于Ⅳ-G亚型患者;高血压、蛋白尿、低补体血症轻于Ⅳ-G亚型患者;病理上节段性袢坏死和新月体重于Ⅳ-G亚型患者;白金耳、毛细血管内增生轻于Ⅳ-G亚型患者。结论:Ⅲ型与Ⅲ+Ⅴ型、Ⅳ型与Ⅳ+Ⅴ型、Ⅳ-S亚型与Ⅳ-G亚型LN患者之间虽然临床病理特点不同,但肾脏生存率均无差异。     

Ⅳ型狼疮性肾炎伴血栓性微血管病变的临床特点及预后

目的:回顾性分析Ⅳ型狼疮性肾炎(LN)伴肾间质血管血栓性微血管病变(TMA)患者的临床、病理及预后。方法:33例在解放军肾脏病研究所住院、经肾活检诊断为Ⅳ型LN(参照2003年ISN/RPS分型方案)伴肾间质血管TMA病变的患者,女性27例,男性6例,平均年龄(31.8±10.9)岁,回顾性分析其临床特点(肾脏表现、肾外脏器受累、免疫学异常及病理特征)及预后特征(近期疗效、远期人、肾存活率及终末期肾衰发生的危险因素)。结果:(1)27例(81.8%)入院即表现为肾功能不全[SCr(274.0±176.8)μmol/L],其中14例(42.4%)需要肾脏替代治疗。高血压、肾病范围蛋白尿、肉眼血尿的比例分别为93.9%、57.6%和24.2%。(2)微血管病性溶血性贫血(MHA)比例为60.6%,神经系统损害、心、肺受累及浆膜腔炎的发生率分别为:18.1%、36.3%、15.1%和66.7%。(3)血清ANA、抗ds-DNA及抗心磷脂抗体阳性率分别为90.9%,75.8%和33.3%。96.9%和42.4%的患者伴有低C3及C4血症。(4)肾活检病理分型Ⅳ(G)型25例(75.8%)、Ⅳ Ⅴ型7例(21.2%)、Ⅳ(S)型1例(3.0%)。袢坏死、Fribin阳性的袢内血栓及新月体比例分别为:51.5%,69.7%和60.6%。20例(60.7%)肾间质小动脉有血栓形成。中-重度小管-间质慢性化损害者达78.8%。(5)患者中位随访时间为13月(1～101月),6例(42.9%)摆脱肾脏替代治疗,6月及12月治疗有反应率分别为38.9%和55.2%。死亡3例(感染、不明死因和多器官功能衰竭各1例),10例(38.5%)进展为终末期肾衰。患者1年、3年、5年人生存率分别为96.2%,69.2%和69.2%;肾存活率分别为:62.3%,62.3%和46.7%。结论:伴TMA的Ⅳ型LN患者肾脏损害严重,部分患者可无典型MHA表现,肾小球病理类型以Ⅳ(G)和Ⅳ Ⅴ型多见,Ⅳ(S)型少见,患者预后差。应加强LN伴TMA的早期诊断,探索有效的治疗手段。     

血栓性微血管病的肾脏损害——附27例临床病理分析

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人球动脉，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人球动脉，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。     

Clinicopathological features and renal outcomes of Ig A nephropathy with acute tubulointerstitial nephropathy

<正>Objective To evaluate the clinicopathological characteristics and outcomes of IgA nephropathy (IgAN)with acute tubulointerstitial nephropathy (ATIN).Methods Patients who were diagnosed as IgAN with ATIN and IgAN without ATIN by renal biopsy in Peking University First Hospital were enrolled. Seventy four cases of IgAN with ATIN and 74 cases of IgAN without     

Severe renal failure and thrombotic microangiopathy induced by malignant hypertension successfully treated with spironolactone

Malignant hypertension can cause thrombotic microangiopathy (TMA) characterized by hemolytic anemia and thrombocytopenia. On the other hand, severe hypertension is sometimes associated with hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). Distinguishing these entities is important because of therapeutic implications. Plasmapheresis should be initiated as soon as possible if we are dealing with TTP. We describe the case of a 30-year-old man referred to our hospital with malignant hypertension, severe renal failure and TMA: haemoglobin = 9 g/dL, total bilirubin = 0.4 mg/dL, haptoglobin ≤ 10 mg/dL, platelet count = 59,000/μL and schistocytes on peripheral smear. He required initiation of hemodialysis. Additionally, we considered that the possible cause of TMA was malignant hypertension according to the presence of hypertensive retinopathy and thrombocytopenia which remitted only with blood pressure control, hence, plasmapheresis was not given. Renal function did not improve and the patient remained chronic hemodialysis. Intensive therapy for hypertension with a combination of antihypertensive drugs including spironolactone successfully lowered his blood pressure without developing hyperkalemia.     

POEMS综合征相关肾损害临床及病理特征

目的:分析POEMS综合征的临床表现,肾脏损害的临床和病理特征,以提高对此病认识. 方法:回顾性分析POEMS综合征患者临床表现、实验室检查特点及肾活检病理资料. 结果:12例患者中男性9例,女性3例,诊断时平均年龄44.5±9.2岁.所有患者早期均误诊,发病至确诊时间为18.4±13.2月.临床特征:(1)多发性周围神经病变:四肢麻木、肌无力12例(100％),肌电图均示神经源性改变.(2)脏器肿大:肝肿大4例(33.3％)、脾肿大11例(91.7％)、淋巴结肿大4例(33.3％)、肾脏体积增大6例(50％).(3)内分泌病变:男性性功能障碍7例(77.7％)、女性闭经2例(66.7％),甲状腺功能减低10例(83.3％),糖尿病6例(50％).(4)单克隆浆细胞增生,血清M蛋白阳性率9例(75％).(5)皮肤色素沉着:所有患者均有皮肤色素沉着.(6)肾脏损害:水肿11例(91.7％);少量蛋白尿8例(66.7％);镜下血尿1例(8.3％);血清肌酐升高6例(50％);尿酸升高11例(91.7％).4例患者行肾活检,病理特点:(1)肾小球系膜病变明显(系膜区增宽、系膜细胞及基质增生、系膜溶解);(2)肾小球内皮细胞病变突出;(3)未见免疫复合物沉积. 结论:POEMS综合征临床累及多系统,症状复杂,容易漏诊、误诊.肾脏损害并非少见,少量蛋白尿为主,可伴肾功能不全,病理活检组织学改变以肾小球内皮细胞和系膜病变突出.     

Clinicopathological features and outcomes of primary IgA nephropathy patients with chronic tonsillitis

正Objective To explore the clinicopathological features and renal outcomes of primary IgA nephropathy(IgAN)patients with chronic tonsillitis.Methods Patients with biopsy-proven primary IgAN admitted to The First Affiliated Hospital,Sun Yat-sen University from January 2006 to December 2011 were enrolled.The clinicopathological features and renal outcomes of     

Clinicopathological features and outcomes of patients with gastric cancer:A single-center experience

AIM:To evaluate the location,histopathology,stages,and treatment of gastric cancer and to conduct survival analysis on prognostic factors.METHODS:Patients diagnosed with of stomach cancer in our clinic between 2000 and 2011,with follow-up or a treatment decision,were evaluated retrospectively.They were followed up by no treatment,adjuvant therapy,or metastatic therapy.We excluded from the study any patients whose laboratory records lacked the operating parameters.The type of surgery in patients diagnosed with gastric cancer was total gastrectomy,subtotal gastrectomy or palliative surgery.Patients with indications for adjuvant treatment were treated with adjuvant and/or radio-chemotherapy.Prognostic evaluation was made based on the parameters of the patient,tumor and treatment.RESULTS:In this study,outpatient clinic records of patients with gastric cancer diagnosis were analyzed retrospectively.A total of 796 patients were evaluated(552male,244 female).The median age was 58 years(22-90 years).The median follow-up period was 12 mo(1-276 mo),and median survival time was 12 mo(11.5-12.4 mo).Increased T stage and N stage resulted in a decrease in survival.Other prognostic factors related to the disease were positive surgical margins,lymphovascular invasion,perineural invasion,cardio-esophageal settlement,and the levels of tumor markers in metastatic disease.No prognostic significance of the patient’s age,sex or tumor histopathology was detected.CONCLUSION:The prognostic factors identified in all groups and the proposed treatments according to stage should be applied,and innovations in the new targeted therapies should be followed.     

肾移植术后新生隐球菌病的临床特点及转归

目的:分析肾移植术后新生隐球菌病的临床特点,旨在提高诊治水平。方法:回顾性分析6例肾移植术后新生隐球菌感染患者的临床特点、实验室检查结果、治疗及转归。结果:6例患者中5例原发病为慢性肾小球肾炎,1例为原发性高草酸尿症(I型);全部患者均系首次肾移植,发病中位时间为移植术后6年。6例患者HIV均阴性,2例乙肝表面抗原携带者,1例丙型病毒性肝炎;3例合并糖尿病,且血糖控制不佳;3例合并高血压;3例合并皮肤感染;1例有鸽子接触史。所有患者均存在发热和头痛症状,且有不同程度意识障碍,其中3例癫痫;5例脑膜刺激征阳性;2例视力下降,1例听力下降。本组患者均体型消瘦,T淋巴细胞亚群CD4+、CD8+计数偏低,存在不同程度贫血,白蛋白和前白蛋白低下,移植肾功能不全。6例患者均颅内高压(23.5～41cmH2O),脑脊液蛋白含量均高于正常,但葡萄糖降低;5例脑脊液墨汁染色找到新生隐球菌,4例脑脊液培养出新生隐球菌;3例新生隐球菌菌血症,3例皮损活检组织培养新生隐球菌阳性,2例合并肺部酵母菌感染,4例诊断符合全身播散性新生隐球菌病。4例采用氟康唑治疗,1例伊曲康唑,1例氟康唑续伏立康唑;经上述药物治疗后,3例治愈并行维持性血液透析治疗,余3例死亡。结论:肾移植术后新生隐球菌易播散至皮肤,常因非特异性皮肤改变而延误诊断,因此有必要早期行皮肤组织活检以及特殊染色。早期明确诊断和及时治疗是提高此病救治成功率的关键。     

Bilateral nephrectomy for the treatment of refractory lupus nephritis with features overlapping with thrombotic microangiopathy resembling thrombotic thrombocytopenia purpura

We report a patient with a diagnosis of systemic lupus erythematosus who concurrently developed a syndrome of thrombotic microangiopathy that resembled thrombotic thrombocytopenic purpura. The patient underwent plasma exchange and immunosuppressive therapy for months before clinical improvement was finally achieved through bilateral nephrectomy. Ultimately, our patient died of disseminated aspergillosis from prolonged immunosuppression. We believe that recognition of bilateral nephrectomy as a potential treatment earlier in her course would have spared her this unfortunate demise. We hope that this review of current literature will help the reader to consider bilateral nephrectomy in patients with refractory systemic lupus erythematosus with clinical overlap of thrombotic microangiopathy resembling thrombotic thrombocytopenic purpura.     

Neurological manifestations of thrombotic microangiopathy syndromes in adult patients

Journal of Thrombosis and Thrombolysis - The objective of this study was to compare the frequency and severity of neurologic manifestations in adult patients diagnosed with thrombotic...     

血栓性微血管病发病机制及治疗

血栓性微血管病是常见的一组临床病理综合征,因其发病机制不清且缺乏有效治疗手段,患者预后常较差.近年有关该病的发病机制及治疗均有许多新进展,本文将就此作一综述.     

药物性肝损伤临床与组织病理学特点

正药物性肝损伤(drug-induced liver injury,DILI)是指由药物、小分子化合物、生物制剂、传统中药、天然药物、保健品和膳食补充剂(herbals and dietary supplements,HDS)等所引发的肝损伤~([1,2])。美国和欧洲DILI是急性肝衰竭最常见的原因,其比例