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Very Early Severe Posttransplant Recurrent Antineutrophil Cytoplasmic Antibody-Associated Glomerulonephritis after Kidney Transplantation: Two Case Reports 肾移植术后极早期严重复发性抗中性粒细胞胞浆抗体相关性肾小球肾炎2例报告
Pub Date : 2022-03-03 DOI: 10.1155/2022/9740225
Reda Laamech, Hamza Naciri-Bennani, D. Giovannini, Johan Noble, B. Janbon, P. Malvezzi, T. Jouve, L. Rostaing
Successful kidney transplantation (KTx) in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) has been reported with excellent patient and graft survival rates. The recurrence of AAV in transplant recipients is rare, and its mechanisms of action are not clearly known. The optimum time for KTx and the relevance of ANCA titer at the time of transplantation remain controversial. We report two cases of extremely rapid recurrent AAV after renal transplantation; both were still ANCA-positive at the time of transplantation, which led us to question the pathogenesis of ANCA antibodies in recurrence in a kidney allograft. Apheresis plus immunosuppressive therapies were ineffective in the first case and the patient became dialysis-dependent, whereas in the second case methylprednisone pulses plus rituximab infusions resulted in long-lasting remission.
抗中性粒细胞细胞质抗体相关血管炎(AAV)患者成功进行肾移植(KTx)已被报道具有良好的患者和移植物存活率。AAV在移植受者中的复发是罕见的,其作用机制尚不清楚。移植时KTx的最佳时间和ANCA滴度的相关性仍然存在争议。我们报告2例肾移植后极快复发的AAV;两者在移植时仍为ANCA阳性,这使我们质疑ANCA抗体在异体肾移植复发中的发病机制。在第一个病例中,单采+免疫抑制治疗无效,患者成为透析依赖,而在第二个病例中,甲基强的松脉冲+利妥昔单抗输注导致长期缓解。
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引用次数: 3
Dialysis Disequilibrium: Is Acidosis More Important than Urea? 透析不平衡:酸中毒比尿素更重要吗?
Pub Date : 2022-02-22 DOI: 10.1155/2022/4964033
Akshay Athavale, K. Wyburn, P. Snelling, S. Chadban
Dialysis disequilibrium syndrome is a severe complication associated with dialysis treatment. Manifestations may range from mild such as headache to severe such as seizures and coma. Risk factors for development include initial dialysis treatment, uraemia, metabolic acidosis, and extremes of age. We report a case of dialysis disequilibrium in a patient with a failing kidney transplant secondary to the recurrence of IgA nephropathy. Disturbance in cognition and neurologic functioning occurred six hours after the completion of initiation of intermittent haemodialysis. During two sessions of intermittent haemodialysis of 3 and 4 hours, urea was reduced by 21.9 and 17.2 mmol/L and measured serum osmolality was reduced by 25 and 14 mOsm/kg, respectively. Subsequent admission to the intensive care unit and initiation of continuous renal replacement therapy for 48 hours resulted in complete resolution of symptoms. In this case report, we discuss atypical clinical and radiologic features of dialysis disequilibrium occurring with modest reductions in urea and serum osmolality.
透析不平衡综合征是与透析治疗相关的严重并发症。其表现从轻微如头痛到严重如癫痫发作和昏迷不等。发展的危险因素包括初始透析治疗、尿毒症、代谢性酸中毒和极端年龄。我们报告一例透析不平衡的病人与一个失败的肾移植继发IgA肾病。间歇性血液透析开始后6小时出现认知和神经功能障碍。在3和4小时的间歇血液透析期间,尿素分别降低21.9和17.2 mmol/L,血清渗透压分别降低25和14 mmol/ kg。随后进入重症监护室并开始持续肾脏替代治疗48小时导致症状完全缓解。在这个病例报告中,我们讨论了不典型的临床和放射学特征的透析不平衡发生适度减少尿素和血清渗透压。
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引用次数: 1
Coexistence of Lupus Nephritis, Ulcerative Colitis, and Communicating Hydrocephalus: A Report of a 21-Year-Old Male. 狼疮性肾炎、溃疡性结肠炎和通讯性脑积水共存:一个21岁男性的报告。
Pub Date : 2022-02-04 eCollection Date: 2022-01-01 DOI: 10.1155/2022/1079300
Bushra Ali Khan, Nida Saleem, Danyal Hassan, Shabaz Kiani, Muhammad Haneef

Systemic lupus erythematosus (SLE) and ulcerative colitis (UC) are multisystem autoimmune disorders that rarely coexist. We report a case history of a 21-year-old male, presenting with bloody diarrhea and, later, diagnosed to have ulcerative colitis on colonic biopsy. There was clinically silent renal impairment leading to end-stage kidney disease requiring hemodialysis possibly secondary to ongoing lupus nephritis as suggested by positive lupus-specific antibodies' detection. Besides this, the diagnosis of lupus associated with early communicating hydrocephalus was made on CT brain findings which clinically responded well to the initiation of immunosuppressive therapy. It is imperative to keep in mind the remote possibility of ulcerative colitis in an SLE patient with gastrointestinal (GI) manifestations. Communicating hydrocephalus is a rare neurological manifestation of SLE leading to seizures and can respond well to the initiation of steroids and immunosuppressants. Therefore, a trial of immunosuppressant medications must be given even in a patient with end-stage renal disease (ESRD) to halter extra renal rare lupus manifestations.

系统性红斑狼疮(SLE)和溃疡性结肠炎(UC)是多系统自身免疫性疾病,很少共存。我们报告一个21岁男性的病例史,表现为出血性腹泻,后来,结肠活检诊断为溃疡性结肠炎。临床无症状的肾脏损害导致终末期肾脏疾病,需要血液透析,可能继发于持续的狼疮肾炎,狼疮特异性抗体检测阳性。除此之外,根据CT脑部表现诊断狼疮合并早期交通性脑积水,临床对免疫抑制治疗的开始反应良好。有胃肠道(GI)表现的SLE患者必须牢记溃疡性结肠炎的可能性很小。沟通性脑积水是SLE中一种罕见的神经学表现,可导致癫痫发作,对类固醇和免疫抑制剂的起始治疗反应良好。因此,即使是患有终末期肾病(ESRD)的患者,也必须进行免疫抑制药物的试验,以遏制肾外罕见的狼疮表现。
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引用次数: 0
Acute Oxalate Nephropathy Caused by Excessive Vegetable Juicing and Concomitant Volume Depletion. 过量蔬菜汁引起的急性草酸肾病。
Pub Date : 2022-01-31 eCollection Date: 2022-01-01 DOI: 10.1155/2022/4349673
Harshad Chaudhari, Jennine Michaud, Nityasree Srialluri, Smita Mahendrakar, Christine Granz, Michael Yudd

Acute oxalate nephropathy (AON) induced by high dietary intake of oxalate-rich food is a rare cause of acute kidney injury and end-stage renal disease (ESRD). We describe a 68-year-old man with adequate baseline renal function who developed severe AON and ESRD. Six months earlier, he started a daily oxalate-rich fruit and vegetable juice diet high in spinach, with a calculated daily oxalate dietary intake of 1500 mg, about 10 times a typical diet. Renal biopsy showed extensive tubular oxalate deposits and acute tubular damage; the renal tissue was relatively free of chronic changes such as glomerulosclerosis, tubular atrophy, and interstitial fibrosis. A year later, he remains dialysis dependent.

由于大量摄入富含草酸的食物而引起的急性草酸肾病(AON)是急性肾损伤和终末期肾病(ESRD)的罕见原因。我们描述了一位68岁的男性,基线肾功能正常,但发展为严重的AON和ESRD。六个月前,他开始每天吃富含草酸的水果和蔬菜汁,多吃菠菜,每天的草酸摄入量为1500毫克,大约是正常饮食的10倍。肾活检显示广泛的小管草酸沉积和急性小管损伤;肾组织相对没有慢性病变,如肾小球硬化、肾小管萎缩和间质纤维化。一年后,他仍然依赖透析。
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引用次数: 1
Candida auris Invasive Infection after Kidney Transplantation. 肾移植后耳念珠菌侵袭性感染。
Pub Date : 2022-01-28 eCollection Date: 2022-01-01 DOI: 10.1155/2022/6007607
Javier Reque, Rosa Arlandis, Nayara Panizo, Maria José Pascual, Alejandro Perez-Alba

Introduction: C. auris has been associated not only with a variety of invasive fungal infections, including candidemia, sometimes related to central venous catheter, but also with pericarditis and respiratory tract and urinary tract infections.

Materials and methods: We describe the case of a patient with persistent fever despite antibiotics, who presented with Candida isolation in blood cultures, typified as Candida auris species.

Results: A 57-year-old male receiving peritoneal dialysis underwent kidney transplantation which was complicated by primary nonfunction due to arterial thrombosis necessitating graft nephrectomy. During the postoperative period, he presented with Pseudomonas aeruginosa pneumonia that was treated with levofloxacin and catheter-related Enterococcus faecalis bacteremia treated with linezolid. After hospital discharge, he then presented with herpes zoster infection treated with valacyclovir. Ten days later, he developed peritonitis and exit site infection with multidrug-resistant Pseudomonas aeruginosa treated with intraperitoneal aztreonam and peritoneal dialysis catheter removal. Despite broad-spectrum antibiotic therapy, the patient remained febrile. All microbiology laboratory tests were negative, so it was decided to stop antibiotic therapy for 48 hours and repeat cultures in order to avoid possible false negatives. In new blood cultures performed after suspension of antibiotic therapy, candidemia was observed, later typified as Candida auris species. After completing antifungal treatment (three weeks with intravenous amphotericin B 100 mg qd and two weeks of intravenous anidulafungin 100 mg qd), microbiological cultures remained negative and the patient made uneventful recovery.

Conclusion: Candida auris invasive infection has been mainly described in patients with severe underlying comorbidities and immunocompromise. Multidrug-resistant clusters of Candida auris are increasingly emerging.

导读:C. auris不仅与多种侵袭性真菌感染有关,包括念珠菌病,有时与中心静脉导管有关,还与心包炎、呼吸道和尿路感染有关。材料和方法:我们描述的情况下,患者持续发烧,尽管抗生素,谁提出了念珠菌分离在血液培养,典型的念珠菌耳种。结果:一名接受腹膜透析的57岁男性患者行肾移植手术,因动脉血栓形成导致原发性肾功能不全,需行移植肾切除术。术后患者出现铜绿假单胞菌肺炎,用左氧氟沙星治疗,导管相关性粪肠球菌菌血症用利奈唑胺治疗。出院后,他又出现带状疱疹感染,用伐昔洛韦治疗。10天后,患者出现腹膜炎和出口部位感染,并发多药耐药铜绿假单胞菌,经腹腔注射氨曲南和腹膜透析导管拔除治疗。尽管进行了广谱抗生素治疗,患者仍处于发热状态。所有微生物实验室检测均为阴性,因此决定停止抗生素治疗48小时并重复培养,以避免可能的假阴性。在停用抗生素治疗后进行的新血培养中,观察到念珠菌,后来被分型为耳念珠菌。在完成抗真菌治疗(静脉注射两性霉素B 100 mg qd 3周,静脉注射阿尼杜拉芬100 mg qd 2周)后,微生物培养结果为阴性,患者顺利康复。结论:耳念珠菌侵袭性感染主要发生在伴有严重潜在合并症和免疫功能低下的患者中。耐多药的金黄色念珠菌群越来越多地出现。
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引用次数: 5
A Novel Homozygous Mutation in the COL4A4 Gene (Gly1436del) Causing Alport Syndrome Exposed by Pregnancy: A Case Report and Review of the Literature. 妊娠暴露导致Alport综合征的COL4A4基因(Gly1436del)新纯合突变1例报告及文献复习
Pub Date : 2022-01-04 eCollection Date: 2022-01-01 DOI: 10.1155/2022/5243137
Ulrich Jehn, Cornelie Müller-Hofstede, Barbara Heitplatz, Veerle Van Marck, Stefan Reuter, Hermann Pavenstädt, Britta George

Background: Alport syndrome results from a hereditary defect of collagen IV synthesis. This causes progressive glomerular disease, ocular abnormalities, and inner ear impairment. Case Presentation. Herein, we present a case of Alport syndrome in a 28-year-old woman caused by a novel mutation (Gly1436del) in the COL4A4 gene that was not unveiled until her first pregnancy. Within the 29th pregnancy week, our patient presented with massive proteinuria and nephrotic syndrome. Light microscopic examination of a kidney biopsy showed typical histological features of segmental sclerosis, and electron microscopy revealed extensive podocyte alterations as well as thickness of glomerular basement membranes with splitting of the lamina densa. One and a half years after childbirth, renal function deteriorated to a preterminal stage, whereas nephrotic syndrome subsided quickly after delivery.

Conclusion: This case report highlights the awareness of atypical AS courses and emphasizes the importance of genetic testing in such cases.

背景:Alport综合征是由胶原合成的遗传性缺陷引起的。这会导致进行性肾小球疾病、眼部异常和内耳损伤。案例演示。在这里,我们提出了一个28岁的妇女Alport综合征的病例,由COL4A4基因的一种新的突变(Gly1436del)引起,直到她第一次怀孕才揭晓。在妊娠第29周,我们的患者出现了大量蛋白尿和肾病综合征。肾活检的光镜检查显示典型的节段性硬化症的组织学特征,电镜显示广泛的足细胞改变以及肾小球基底膜的厚度,并伴有层状致密的分裂。分娩后1年半,肾功能恶化至终末期,而肾病综合征在分娩后迅速消退。结论:本病例报告强调了对非典型AS病程的认识,并强调了基因检测在此类病例中的重要性。
{"title":"A Novel Homozygous Mutation in the <i>COL4A4</i> Gene (Gly1436del) Causing Alport Syndrome Exposed by Pregnancy: A Case Report and Review of the Literature.","authors":"Ulrich Jehn,&nbsp;Cornelie Müller-Hofstede,&nbsp;Barbara Heitplatz,&nbsp;Veerle Van Marck,&nbsp;Stefan Reuter,&nbsp;Hermann Pavenstädt,&nbsp;Britta George","doi":"10.1155/2022/5243137","DOIUrl":"https://doi.org/10.1155/2022/5243137","url":null,"abstract":"<p><strong>Background: </strong>Alport syndrome results from a hereditary defect of collagen IV synthesis. This causes progressive glomerular disease, ocular abnormalities, and inner ear impairment. <i>Case Presentation</i>. Herein, we present a case of Alport syndrome in a 28-year-old woman caused by a novel mutation (Gly1436del) in the <i>COL4A4</i> gene that was not unveiled until her first pregnancy. Within the 29<sup>th</sup> pregnancy week, our patient presented with massive proteinuria and nephrotic syndrome. Light microscopic examination of a kidney biopsy showed typical histological features of segmental sclerosis, and electron microscopy revealed extensive podocyte alterations as well as thickness of glomerular basement membranes with splitting of the lamina densa. One and a half years after childbirth, renal function deteriorated to a preterminal stage, whereas nephrotic syndrome subsided quickly after delivery.</p><p><strong>Conclusion: </strong>This case report highlights the awareness of atypical AS courses and emphasizes the importance of genetic testing in such cases.</p>","PeriodicalId":9604,"journal":{"name":"Case Reports in Nephrology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8752291/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39909634","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Simultaneous Pancreas-Kidney Transplant Complicated by Kidney Allograft Torsion and Pseudoaneurysms of the Y-Allograft: A Case Report and Review of the Literature. 胰肾联合移植并发肾扭转及y -异体假性动脉瘤1例报告及文献复习。
Pub Date : 2022-01-01 DOI: 10.1155/2022/1748141
Sarah L Tan, Rachel Y P Tan, Gabrielle Cehic, Michael Wu, John Kanellis, Jeffrey Barbara

Background: We report and review the literature of two rare complications of simultaneous pancreas-kidney transplantation (SPKT) occurring in one patient. Case Report. A 39-year-old man with dialysis-dependent kidney failure secondary to type 1 diabetes mellitus underwent successful SPKT in October 2018. Three months later, he presented with an acute kidney injury (AKI) and returned to dialysis. Kidney scintigraphy showed a central photopenic region, and angiograms showed absent flow in the kidney transplant artery without treatable thrombus and the incidental finding of two pseudoaneurysms of the pancreatic Y-graft. He remained dialysis-dependent for three weeks before spontaneous partial recovery of allograft function; repeat kidney scintigraphy showed significant improvement in perfusion. However, in April 2019 he was readmitted with a sudden deterioration in kidney allograft function again necessitating haemodialysis. Repeat imaging confirmed that the kidney allograft had shifted from the left iliac fossa to the midline. He underwent surgical exploration, during which torsion of the kidney allograft was confirmed and a nephropexy was performed. The kidney allograft was originally implanted in the left retroperitoneum via a midline transperitoneal approach, which likely predisposed it to torsion. The pseudoaneurysms of the pancreatic Y-graft were managed conservatively, and surveillance imaging demonstrated that they remained stable in size. The patient regained reasonable kidney allograft function (estimated glomerular filtration rate, eGFR, of 45 mL/min) and maintained normal pancreatic allograft function.

Conclusion: Kidney allograft torsion should be considered post-SPKT in patients with AKI and absent arterial flow. Although most case reports describe surgical management of pseudoaneurysms post-SPKT, our case demonstrates successful conservative management.

背景:我们报道并回顾了发生在一名患者的两种罕见的胰肾联合移植(SPKT)并发症。病例报告。2018年10月,一名患有继发于1型糖尿病的透析依赖性肾衰竭的39岁男性成功接受了SPKT治疗。3个月后,他出现急性肾损伤(AKI),并返回透析。肾显像显示中央光性减退区,血管造影显示肾移植动脉无血流,无可治疗的血栓,y型胰腺移植处偶然发现两个假性动脉瘤。在同种异体移植物功能自发部分恢复之前,他仍然依赖透析三周;重复肾显像显示灌注明显改善。然而,2019年4月,他再次因同种异体肾脏功能突然恶化而再次入院,需要进行血液透析。重复成像证实肾移植已从左髂窝转移到中线。他接受了手术探查,在此期间证实了肾移植扭转并进行了肾固定。同种异体肾移植最初通过中线经腹膜入路植入左侧腹膜后,这可能使其易发生扭转。胰腺y型移植物的假性动脉瘤被保守处理,监视成像显示它们的大小保持稳定。患者恢复了合理的肾移植功能(估计肾小球滤过率,eGFR为45 mL/min),并维持了正常的胰腺移植功能。结论:急性肾损伤合并无动脉血流的患者在spkt后应考虑同种异体肾移植扭转。虽然大多数病例报告描述手术治疗假性动脉瘤后spkt,我们的病例显示成功的保守管理。
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引用次数: 0
An Unusual Case of Kidney Injury in a Young Woman with a Connective Tissue Disease. 年轻女性结缔组织疾病肾损伤的罕见病例。
Pub Date : 2022-01-01 DOI: 10.1155/2022/3833649
Roberto Rivera, Salvador Vila

A 32-year-old female was admitted to our institution with thrombocytopenia, fever, serositis, hepatosplenomegaly, diffuse lymphadenopathy, and renal insufficiency. A diagnosis of systemic lupus erythematosus was made. Due to recalcitrant thrombocytopenia, serositis, and renal insufficiency methylprednisolone was prescribed in high doses. In addition to proteinuria and hematuria, she was found to have uric acid crystals in her urinalysis. A serum uric acid was found elevated at 18 mg/dL. Rasburicase infusions were started. Within 5 days of commencing rasburicase and continuing high-dose methylprednisolone, her serum creatinine normalized and proteinuria resolved. The microhematuria disappeared within 2 weeks of beginning rasburicase. The rapid reversal of renal insufficiency and all urinary abnormalities after the start of rasburicase infusions suggests that the renal injury was most likely due to uric acid-mediated renal injury and not lupus nephritis. Our case illustrates the co-occurrence of 2 distinct clinical entities, one common for the patient's age, sex, and foremost clinical findings, while the other uncommon and unexpected, but both associated to kidney injury. Clinicians must be aware that careful evaluation of symptoms and laboratory tests is needed to make a thorough differential diagnosis and provide the right treatment at the most opportune moment.

一名32岁女性因血小板减少、发热、浆液炎、肝脾肿大、弥漫性淋巴结病和肾功能不全而入院。诊断为系统性红斑狼疮。由于顽固性血小板减少症,浆液炎和肾功能不全,甲强的松龙被开大剂量。除了蛋白尿和血尿外,她在尿液分析中还发现尿酸结晶体。血清尿酸升高至18 mg/dL。开始滴注Rasburicase。在开始使用rasburicase和持续使用大剂量甲基强的松龙的5天内,她的血清肌酐恢复正常,蛋白尿得到缓解。小血尿在开始皮疹后2周内消失。肾功能不全和所有尿路异常在开始刷毛酶输注后迅速逆转,提示肾损伤很可能是尿酸介导的肾损伤,而不是狼疮性肾炎。我们的病例说明了两种不同临床实体的共同发生,一种在患者的年龄、性别和最重要的临床表现中很常见,而另一种不常见且出乎意料,但都与肾损伤有关。临床医生必须意识到,需要仔细评估症状和实验室检查,才能做出彻底的鉴别诊断,并在最适当的时候提供正确的治疗。
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引用次数: 0
Anaphylactic Reactions Caused by Nafamostat Mesylate during Hemodialysis before Surgery for Carpal Tunnel Syndrome. 甲磺酸那莫他酯在腕管综合征术前血液透析中的过敏反应。
Pub Date : 2021-12-29 eCollection Date: 2021-01-01 DOI: 10.1155/2021/1148156
Yuta Nakamura, Kaoru Tada, Masashi Matsuta, Atsuro Murai, Hiroyuki Tsuchiya

Nafamostat mesylate (NM) has been used to treat pancreatitis and disseminated intravascular coagulation during hemodialysis (HD). However, there have been some reports of adverse effects related to anaphylactic reactions. We present a case in which anaphylactic reactions caused by NM during preoperative HD caused repeated postponement of surgery for carpal tunnel syndrome. Symptoms including fever, shivering, chills, low blood pressure, tachycardia, nausea, and vomiting appeared during preoperative HD, and surgery was postponed thrice. Initially, the patient was misdiagnosed with sepsis because of elevated C-reactive protein and procalcitonin levels. However, since the symptoms appeared only when NM was administered and disappeared quickly after the administration of NM was terminated, the condition was diagnosed as anaphylactic reactions caused by NM. Therefore, it is essential to consider anaphylactic reactions caused by NM as differential diagnoses, when symptoms, such as fever, are observed during perioperative HD.

甲磺酸纳莫他酯(NM)已被用于治疗血液透析(HD)期间的胰腺炎和弥散性血管内凝血。然而,也有一些与过敏反应有关的不良反应的报道。我们提出了一个病例,在术前HD期间由NM引起的过敏反应导致腕管综合征的手术一再推迟。HD术前出现发热、寒战、低血压、心动过速、恶心、呕吐等症状,手术推迟三次。最初,由于c反应蛋白和降钙素原水平升高,患者被误诊为败血症。但由于症状仅在服用NM时出现,停药后迅速消失,故诊断为NM引起的过敏反应。因此,当围手术期观察到发热等症状时,将NM引起的过敏反应作为鉴别诊断是必要的。
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引用次数: 2
An Interesting Case of Nonlupus Full-House Nephropathy. 一例有趣的非狼疮性全屋肾病。
Pub Date : 2021-12-28 eCollection Date: 2021-01-01 DOI: 10.1155/2021/9043003
Satyanand Sathi, Alok Sharma, Anil Kumar Garg, Virendra Singh Saini, Manoj Kumar Singh, Devinder Vohra, Arvind Trivedi

Full-house immunofluorescence and endothelial tubuloreticular inclusions are known as characteristic features of lupus nephritis. However, both features are not pathognomonic for lupus nephritis. A kidney biopsy specimen showing full-house immunofluorescence pattern in the absence of autoantibodies and classical clinical features of Systemic Lupus Erythematosus (SLE) is now considered as nonlupus full-house nephropathy (FHN). Nonlupus FHN may be idiopathic or due to other disease processes known as secondary nonlupus FHN. Here, we report the case of a 36-year-old female who presented with nephrotic proteinuria with bland urine sediment. Additional analyses revealed normal serum antinuclear antibody (ANA), normal anti-double-stranded DNA (anti-dsDNA) antibodies, and normal serum C3 and C4 levels. A renal biopsy showed a normal-appearing glomerulus without any proliferation or capillary wall thickening and widespread glomerular immune deposits (full-house effect; IgA, IgG, IgM, C3, and C1Q) on direct immunofluorescence. Renal electron microscopy showed diffuse effacement of visceral epithelial cell foot processes and mesangial electron dense deposits. The patient was diagnosed as nonlupus FHN. There is a controversial role of steroids and other immunosuppressive drugs in the treatment of nonlupus FHN patients, but our case patient responded favourably to steroid therapy. The term nonlupus FHN can be used as an umbrella term for patients who do not satisfy the clinical and serological criteria of SLE.

全身免疫荧光和内皮管网状包涵体是狼疮肾炎的特征性特征。然而,这两个特征都不是狼疮性肾炎的典型症状。肾活检标本在没有自身抗体和系统性红斑狼疮(SLE)的典型临床特征的情况下显示全屋免疫荧光模式,现在被认为是非狼疮性全屋肾病(FHN)。非狼疮性FHN可能是特发性的或由于其他疾病过程称为继发性非狼疮性FHN。在这里,我们报告一个36岁的女性谁提出了肾病蛋白尿淡性尿沉淀。进一步分析显示血清抗核抗体(ANA)、抗双链DNA(抗dsdna)抗体正常,血清C3和C4水平正常。肾活检显示肾小球外观正常,未见增生或毛细血管壁增厚,肾小球免疫沉积广泛存在(全室效应;IgA, IgG, IgM, C3和C1Q)的直接免疫荧光。肾脏电镜显示内脏上皮细胞足突弥漫性消失及系膜电子致密沉积。患者被诊断为非狼疮性FHN。类固醇和其他免疫抑制药物在治疗非狼疮性FHN患者中的作用存在争议,但本病例患者对类固醇治疗反应良好。术语非狼疮性FHN可以作为不满足SLE临床和血清学标准的患者的总称。
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引用次数: 1
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Case Reports in Nephrology
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