急进性肾小球肾炎1例报告

1　病例报告患者, 男, 73岁。因畏寒、发热、乏力 4日, 加重伴气促 1日后拟诊“慢性支气管炎急性发作”入本院呼吸科治疗。体格检查: 体温 38 7℃, 脉搏 100次 /分, 呼吸 24次 /分, 血压 19 /8kPa。轻度贫血貌, 咽充血, 双侧扁桃体Ⅰ度肿大, 浅表淋巴结无肿大。两肺呼吸音粗,     

急进性肾炎12例临床分析

回顾性总结了１２例急进性肾炎（ＲＰＧＮ）患者的临床表现，实验室及病理资料及对不同治疗方法的反应。结果显示：原发性ＲＰＧＮ９例、免疫球蛋白Ａ肾病、系统性红斑狼疮、Ｇｏｏｄ－Ｐａｓｔｕｒｅ综合征各１例。１２例患者均表现不同程度的蛋白尿、血尿、，其中４例表现为肾病综合征（ＮＳ），４例呈发作性肉眼血尿，７例血压增高；３例经糖皮质激素、环磷酰胺冲击或血浆置换治疗后肾功能基本恢复，其余７例依赖长期的存活，２例     

急进性肾小球肾炎(附2例报告并文献复习)

急进性肾小球肾炎 (rapidlyprogressiveglomerulonephritis ,RPGN)又称恶性肾炎、毛细血管外增生性肾炎、新月体肾炎。1942年首先为Ellis所描述。Heptinstall 1966年明确了定义。1968年Bacani等详细阐述了本病的临床和组织学特征。RPGN是最严重的肾小球疾病之一 ,预后差 ,如不早期诊治 ,病人可在数周内从正常肾功能发展至终末期肾衰 ,甚至死亡。RPGN发生率约占肾小球肾炎的 2 %～ 5 %。近年来随着早期诊断水平的提高 ,本病的确诊人数及比例逐年增加 ,国内外文献均有报道[1～ 4 ] 。RPGN可由多种原因引起 ,包括 :①原发性 ;②继发于…     

急进性肾小球肾炎的治疗进展

1引言 RPGN是一组以急性肾炎综合征(血尿、蛋白尿、水肿、高血压)、肾功能急剧减退、早期出现少尿或无尿为主要临床表现,以新月体肾小球肾炎(50%以上肾小球出现大新月体,早期为细胞新月体,晚期为纤维新月体)为病理特征的原发性肾小球疾病.病情危重,需要尽早治疗.     

急进性肾小球肾炎的诊断和治疗

1引言急进性肾小球肾炎为一临床综合征,以急性肾炎样症状日益加重,短期内呈进行性肾衰竭,多数病人在数月内死于尿毒症为特征。在病理上称为新月体肾炎(新月体占肾小球数的50%以上)。急进性肾小球肾炎可分为3型:Ⅰ型(抗基底膜抗体肾炎)、Ⅱ型(免疫复合物肾炎)及Ⅲ型(无免疫沉积物肾炎)。目前认为Ⅲ型急进性肾小球肾炎主要由抗中性粒细胞胞浆抗体所致的原发性小血管炎,肾小球毛细血管出现节段性坏死,导致血浆及单核细胞进入肾小囊,形成新月体。其发病与多种黏附分子及细胞因子密切相关。抗中性粒细胞胞浆抗体特异性抗原是中性粒细胞中的原始颗…     

急进性肾小球肾炎的免疫学诊断

1引言 急进性肾小球肾炎（rapidly progressive glomerulonephritis，RPGN）是一组由各种原因、不同疾病引起的以肾病为主的临床综合征，病情发展急骤，在数日或数周内进行性血清肌酐升高、少尿或无尿、血压升高、贫血等，导致快速进展性肾衰竭。RPGN的发生率虽然不高，但预后较差，一般认为就诊时血清肌酐升高程度是决定肾存活的重要指标。如何早期诊断RPGN及确定其分类，是指导治疗和判断预后的基础和关键。因此，特别强调的是针对病因和发病机制的免疫学诊断。     

小儿泌尿系统疾病(4)急进性、迁延性及慢性肾小球肾炎

本文介绍肾小球肾炎的一种特殊类型急进性肾小球肾炎，简称急进性肾炎。同时介绍迁延性和慢性肾炎，从中了解小儿急性肾炎的转归。     

16例急进性肾炎临床分析

１６例急进性肾炎临床分析诸青张凤琴唐政作者单位：２１４０６２江苏省无锡市第四医院（诸青）；８３１０００新疆昌吉州医院（张凤琴）；２１０００２南京军区总医院（唐政）急进性肾炎（ＲＰＧＮ）病理又称新月体肾炎，临床表现危重，预后差，如不早期诊治，数周或数月...     

急进性肾小球肾炎首发咯血症状1例报告

患者，男，35岁。因间断咯血半个月，胸闷、气促2d，于2006年8月14日入院。患者入院前半个月无诱因，痰中带暗红色血丝，每日数口，偶发热。入院前2d胸片检查提示：“肺炎”收住入院。入院体检：一般情况正常。血常规：自细胞8．8×10^9/L，血红蛋白108g／L，血小板128×10^9/L。尿、粪常规、电解质、肝肾功能、血尿酸、自身抗体、血沉及凝血机制检查正常。胸部CT示：[第一段]     

急进性肾小球肾炎的诊治进展

急进性肾小球肾炎(RPGN)是肾小球肾炎中最严重的类型,病情发展迅速,表现为蛋白尿、血尿、少尿或无尿性急性肾功能衰竭,若不及时治疗,80%～90%以上的患者于6个月内死亡或需依赖透析生存,肾穿刺显示大多数肾小球球囊的大部分被新月体充斥,故有新月体肾炎之称。本病的发生率虽不高,但若能提高对该病的认识水平,及早诊断,及时有效的治疗,则可避免或减慢患者疾病快速演变至终末期肾功能衰竭的进程。RPGN的分类由于对RPGN的发病机制尚有不少争论,因此有待对其作进一步探讨后才能找到一种更为确切的分类方法。1.按病因分类(1)原发性:Ⅰ型,抗肾…     

"Triple-positive" renal limited vasculitis presenting with rapidly progressive glomerulonephritis: A case report

Rationale: Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) in a case of glomerulonephritis is often identified as a "double-positive" disease. Interestingly, the majority of "double positive" ANCA is myeloperoxidase (MPO)-ANCA and some of the MPO-ANCA positive cases showed intrarenal arteritis, suggesting an ANCA-associated kidney lesion. Proteinase 3-ANCA positive diseases are also rarely reported. Patients positive for all three antibodies, i.e., triple-positive patients, are extremely rare.Patient's Concern: A 53 year-old female presented with anasarca and oliguria of 2 months' duration. Diagnosis: Pauci-immune type renal limited crescentic glomerulonephritis positive for MPO-ANCA, proteinase 3-ANCA, and anti-GBM antibody (triple-positive). Interventions: Intravenous high dose cyclophosphamide, oral azathioprine, intravenous methylprednisolone, and plasma exchange as per British Health Professionals in Rheumatology Guidelines. Outcomes: After one-month follow-up, anasarca and proteinuria were lessened, serum creatinine was normalized, titers of MPO-ANCA levels were decreased, and anti-GBM antibody levels were normalized. Lessons: Triple-positive renal limited vasculitis is rare and response to combined immunosuppressive therapy and plasma exchange can contribute to successful remission.     

快速进展型结核性心包炎的临床治疗

目的探讨不同治疗方案对于快速进展为缩窄性心包炎的结核性心包炎（快速进展型结核性心包炎）治疗效果、并发症、死亡率、患者预后的影响,以期对结核性心包炎的治疗获得更好的效果。 方法回顾性分析2014年6月至2019年5月成都市第三人民医院心脏大血管外科对38例快速进展型结核性心包炎的治疗方式、外科手术干预时机、手术方式及疗效、并发症、死亡率和随访情况等,治疗方式包括药物治疗、心包穿刺引流术、心包开窗术、心包剥脱术等。同期观察对慢性缩窄性心包炎行外科手术治疗患者115例。 结果对于快速进展型结核性心包炎采用不同的治疗方案,随访1~5年。所有患者均采用抗结核药物治疗,其中单纯药物治疗5例,全部进展为缩窄性心包炎;早期行心包穿刺引流术12例,治愈1例,其余11例进展为慢性缩窄性心包炎;早期行心包开窗术10例,进展为慢性缩窄性心包炎1例,治愈9例;早期行心包剥脱术11例,无进展为慢性缩窄性心包炎病例。全组无围术期死亡病例。与同期行慢性缩窄性心包炎外科手术治疗患者相比,快速进展型结核性心包炎外科手术治疗患者低心排综合征发生率较低（4.8% vs 24.3%,P＜0.05）,患者心功能改善情况较好（100.0% vs 80.7%,P＜0.05）,术后5年随访生存率较高（100% vs 78%,P＜0.05）。 结论快速进展型结核性心包炎单纯采用药物治疗和心包穿刺术治疗,有很高比例的患者会进展为缩窄性心包炎,从而严重影响患者的预后。早期外科干预能显著降低快速进展型结核性心包炎进展为缩窄性心包炎的概率,从而改善患者总体预后,改善患者生存质量,减轻社会负担。     

急进性肾炎的诊治进展

急进性肾小球肾炎(rapidly progressive glomerulonephritis,RPGN)以急性肾炎综合征伴肾功能急剧恶化为临床特征,病理为新月体肾炎.早期诊断主要依靠血清免疫学检查及尽早肾活检.早期治疗应根据病理类型及患者情况采用个体化的治疗方案,包括强化治疗和免疫抑制治疗.     

3例抗MDA5抗体阳性皮肌炎合并快速进展型间质性肺病患者的临床特点附文献复习

回顾性分析2020年7月至2021年10月在攀枝花学院附属医院呼吸与危重症医学科住院的3例抗MDA5抗体阳性皮肌炎合并快速进展型间质性肺病患者的临床资料、治疗经过、预后特点,结合文献复习进行研讨。3例患者中男1例,女2例,年龄（557±21）岁,病程（38±02）月。临床主要表现为典型的皮疹,进行性呼吸困难;血清肌酶均增高,血清抗MDA5抗体、抗组氨酰合成酶抗体（Jo 1）、抗Ro 52抗体均阳性;皮疹部位皮肤肌肉活检均提示炎性改变;短期内影像学呈进行性加重的肺间质纤维化。3例患者均使用了激素冲击+丙种球蛋白+环磷酰胺治疗,1例使用了利妥昔单抗治疗。3例患者分别在住院治疗后27、37、48天死亡,2例死于急性呼吸衰竭,1例死于脓毒性休克。抗MDA5阳性皮肌炎合并快速进展型间质性肺病是一种病情进展快、疗效差、死亡率高的疾病。其临床主要表现是典型的皮疹和进行性呼吸困难,血清肌酶增高,抗MDA5抗体阳性（3例患者同时合并抗组氨酰合成酶抗体Jo 1 、抗Ro 52抗体阳性）。影像学均短期内呈进行性加重的肺间质纤维化。以激素冲击、免疫调节、靶向阻断等为主的药物治疗,未能成功挽救患者的生命。治疗后免疫低下所导致的严重感染,也是死亡的重要原因。早期识别,早期干预,以及疾病后期的ECMO支持、肺移植,可能是降低死亡率的有效手段。     

蛋白A免疫吸附治疗系统性红斑狼疮1例

报告1例应用蛋白A免疫吸附治疗系统性红斑狼疮病人的个案,总结治疗的护理要点,包括治疗前病人评估与用物准备,治疗中预冲、吸附、回浆、洗脱、平衡、二次预冲循环过程的操作、病情观察及吸附柱的储存,治疗结束后的治疗效果评价。通过该病例的护理总结,为临床应用蛋白A免疫吸附治疗系统性红斑狼疮的护理提供参考。     

Rare complication of a commonly used antihypertensive agent: A case of hydralazine‐induced ANCA‐associated vasculitis presenting as rapidly progressive glomerulonephritis

Hydralazine‐induced ANCA‐associated vasculitis is a rare clinical entity, with complications including rapidly progressive glomerulonephritis, pulmonary hemorrhage, and pulmonary‐renal syndrome. We present this case to highlight the clinical features that support this challenging diagnosis and to emphasize the importance of prompt recognition and aggressive intervention given its significant morbidity and mortality.     

蛋白A免疫吸附治疗重症肌无力的临床观察

目的探讨蛋白A免疫吸附治疗重症肌无力（MG）的临床疗效。方法采用病例对照方法,将24例MG患者非随机分为2组。治疗组12例,对照组12例,治疗组在传统治疗方法的基础上,给予蛋白A免疫吸附治疗,每周3次,共4周。对照组不进行蛋白A吸附治疗。治疗前和治疗后抽取清晨空腹血,测IgG、IgA、IgM、补体C3、C4和采用酶联免疫吸附法测乙酰胆碱受体抗剂（AChR-Ab）,并根据疗效标准临床相对评分法进行评分。结果治疗组总有效率为91.67%（11/12）,对照组总有效率为75%（9/12）,差异有统计学意义（P〈0.05）。治疗组免疫球蛋白IgG、IgA、IgM和AchR-Ab下降程度较对照组明显降低（P〈0.01）。结论蛋白A免疫吸附能有效降低MG血液中AchR-Ab和免疫球蛋白水平,比单纯药物治疗疗效好,是治疗MG的有效方法。     

Minimal toxicity during protein A immunoadsorption treatment of malignant disease: an outpatient therapy

Extracorporeal removal or modulation of circulating immune complexes (CIC) from plasma of animals and humans with malignant disease may be associated with induction of immune-mediated anti-tumor responses. Immunoadsorption columns containing heat-killed and formalin-fixed Staphylococcus aureus or staphylococcal protein A have been used for this purpose but treatments have often been associated with cardiopulmonary toxicity. Recently, an immunoadsorption device containing highly purified protein A covalently attached to a silica matrix (PROSORBA column) was used to treat 142 patients with refractory malignancies and 22 of 104 patients evaluated for anti-tumor response had objectively measurable reduction in tumor burden. In contrast to earlier experience with other devices, the procedures used in this trial were well tolerated and could be performed on an outpatient basis. The most common side effects observed among 1,306 treatments were chills (28% of treatments), low grade fever (28%), and musculoskeletal pain (16%). Side effects were mild to moderate and required no treatment or only symptomatic treatment. Treatment schedules were interrupted due to side effects for only six patients and there were no treatment-related deaths. Of 64 patients available for long-term follow-up evaluation (mean of 11 months), none exhibited evidence of long-term treatment-related side effects. None of the patient deaths in that period were associated with short or long-term treatment-related side effects. Protein A-silica (PROSORBA columns) can be used safely for development of further experimental treatments of malignant disease.     

A case of rapidly progressive necrotizing pneumonia caused by community-acquired methicillin-resistant Staphylococcus aureus

We present the case of a patient with a necrotizing multilobar pneumonia caused by community-acquired methicillin-resistant Staphylococcus aureus (MRSA). The patient presented with shortness of breath and a productive cough of 3 days duration. On arrival to the emergency department she was intubated for increased work of breathing and given vasopressors for hypotension refractory to fluid resuscitation. Blood cultures taken at admission, sputum cultures from the patient's endotracheal tube, and bronchoalveolar lavage cultures all grew S. aureus resistant to penicillinase-resistant penicillins. Over the following days the patient's respiratory function deteriorated as she grew progressively hypoxemic and hypercarbic despite aggressive mechanical ventilation and intravenous antibiotics. On day 4 of her hospitalization a computed tomogram revealed extensive pulmonary necrosis consistent with necrotizing pneumonia. The patient's family elected to withdraw support, and the patient rapidly died following cessation of mechanical ventilation.