Nephritis, Interstitial

肾炎, 间质性
  • 文章类型: Systematic Review
    急性间质性肾炎(AIN)是一种可能导致永久性肾损伤的药物并发症。已经报道了用整联蛋白抑制剂维多珠单抗治疗的炎症性肠病(IBD)患者中的AIN。通过对现有文献的系统回顾,我们旨在确定和描述接受维多珠单抗治疗的IBD患者中AIN的病例.
    我们搜索了Medline,Embase,科克伦,以及2009年1月1日至2023年4月25日之间的WebofScience核心合集。搜索产生了1473种出版物。标题和摘要由两名独立的审稿人筛选。全文审查了70份出版物。八个符合纳入标准。提取AIN病例的临床特征。根据两种国际药物不良反应概率评估量表进行病例因果关系评估。根据系统评价和荟萃分析(PRISMA)指南的首选报告项目报告结果。
    在6例溃疡性结肠炎和3例克罗恩病患者中报告了9例经活检证实的AIN病例。AIN发病的平均年龄为36岁(范围=19-58),大多数患者为女性(n=6/9)。从维多珠单抗治疗开始到AIN发作的时间从数小时到12个月。常见的症状是发烧和不适。所有患者的肌酐水平均升高。5例患者持续永久性肾损伤。
    我们的研究结果表明,维多珠单抗,虽然很少,可能导致IBD患者出现AIN。意识到与AIN一致的实验室发现和症状,同时监测肾功能,维多珠单抗治疗的IBD患者可能是合理的。
    UNASSIGNED: Acute interstitial nephritis (AIN) is a complication of drugs that may cause permanent kidney injury. AIN has been reported in patients with inflammatory bowel disease (IBD) treated with the integrin inhibitor vedolizumab. Through systematic review of existing literature, we aimed to identify and describe cases of AIN in patients with IBD treated with vedolizumab.
    UNASSIGNED: We searched Medline, Embase, Cochrane, and Web of Science Core Collection between 1 January 2009 and 25 April 2023. The search yielded 1473 publications. Titles and abstracts were screened by two independent reviewers. Seventy publications were reviewed in full-text. Eight met the inclusion criteria. Clinical characteristics of AIN cases were extracted. Case causality assessment was performed according to two international adverse drug reaction probability assessment scales. Results were reported in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
    UNASSIGNED: Nine biopsy-confirmed cases of AIN were reported in six patients with ulcerative colitis and three with Crohn\'s disease. Mean age at AIN onset was 36 years (range = 19-58) and the majority of patients were females (n = 6/9). Time from vedolizumab treatment initiation to AIN onset spanned from hours to 12 months. Common symptoms were fever and malaise. Creatinine levels were elevated in all patients. Five patients sustained permanent kidney injury.
    UNASSIGNED: Our findings suggest that vedolizumab, although rarely, could cause AIN in patients with IBD. Awareness of laboratory findings and symptoms consistent with AIN, along with monitoring of the kidney function, could be warranted in patients with IBD treated with vedolizumab.
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  • 文章类型: Review
    背景:最近的研究集中在免疫检查点抑制剂上。与其他免疫相关的不良事件相比,与使用免疫检查点抑制剂相关的肾脏并发症并不常见。急性间质性肾炎占这些肾脏并发症的大部分,肾病综合征相当罕见。我们在此报告一例与免疫检查点抑制剂相关的肾病综合征,比以前的病例更严重。通过将此案例与以前的报告进行比较,讨论了这种情况特别严重的可能原因。
    方法:一名75岁男子在首次使用纳武单抗和伊匹单抗联合治疗恶性胸膜间皮瘤后出现肾病综合征伴急性肾损伤。肾活检的结果显示微小病变伴轻度动脉粥样硬化,急性间质性肾炎,和几乎所有足细胞足突的融合。Nivolumab和ipilimumab治疗停止,开始使用皮质类固醇治疗。我们调查了以前报道的使用免疫检查点抑制剂的肾病综合征病例。免疫检查点抑制剂相关性肾病综合征17例,包括我们的,已被报道。17例免疫检查点抑制剂相关性肾病综合征患者中有2例需要血液透析治疗急性肾损伤。与以前报道的许多病例不同,本患者服用了两种不同的免疫检查点抑制剂,这可能是严重肾病综合征发展的原因之一。
    结论:除了先前报告的风险因素,与免疫检查点抑制剂单药治疗相比,免疫检查点抑制剂联合治疗可加重肾病综合征.
    BACKGROUND: Recent studies have focused on immune checkpoint inhibitors. Renal complications associated with the use of immune checkpoint inhibitors are uncommon compared with other immune-related adverse events. Acute interstitial nephritis accounts for most of these renal complications, with nephrotic syndrome quite rare. We herein report a case of nephrotic syndrome associated with immune checkpoint inhibitors that was more severe than that in previous cases. By comparing this case with previous reports, the possible reasons for the particular severity of this case are discussed.
    METHODS: A 75-year-old man developed nephrotic syndrome with acute kidney injury after the first combination therapy of nivolumab and ipilimumab for malignant pleural mesothelioma. The results of a kidney biopsy indicated minimal change disease with mild atherosclerosis, acute interstitial nephritis, and fusion of nearly all podocyte foot processes. Nivolumab and ipilimumab therapy were stopped, and treatment with corticosteroids was initiated. We investigated previously reported cases of nephrotic syndrome using immune checkpoint inhibitors. Seventeen cases of immune checkpoint inhibitor-related nephrotic syndrome, including ours, have been reported. Two of the 17 patients with immune checkpoint inhibitor-related nephrotic syndrome required hemodialysis treatment for acute kidney injury. Unlike many previously reported cases, the present patient was administered two different immune checkpoint inhibitors, which may be one of the reasons for the development of severe nephrotic syndrome.
    CONCLUSIONS: In addition to previously reported risk factors, immune checkpoint inhibitor combination therapy can exacerbate nephrotic syndrome compared to immune checkpoint inhibitor monotherapy.
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  • 文章类型: Journal Article
    抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)通常表现为快速进行性肾小球肾炎,并伴有新月形成。在这份报告中,我们介绍了一个髓过氧化物酶(MPO)-AAV相关急性间质性肾炎(AIN)的局部病例,表现为轻微的隐蔽性肾小球肾炎和MPO-ANCA阳性。这种情况的特征是肾小球足细胞的足突消失,良好的预后,和缺乏新月形成。为了进一步了解这种情况,我们对GoogleScholar和PubMed进行了全面的文献检索,同时使用与“AAV和AIN”相关的自由文本单词和MeSH术语。“这次搜索产生了24个案例,我们分析了它们的临床特征,实验室发现,肾脏病理特征,和治疗结果。AAV相关的间质性肾炎主要影响老年患者,通常与贫血有关。蛋白尿,血尿,和非特异性表现,包括发烧,厌食症,疲劳,水肿,和减肥。我们审查中的大多数病例均为MPO-ANCA阳性,并表现出孤立的间质性炎症。这些患者通常血清肌酐水平相对较低,24小时尿蛋白水平,和MPO-ANCA滴度。我们研究的所有患者都接受了免疫抑制治疗,包括糖皮质激素,免疫抑制剂,利妥昔单抗,大多数患者达到临床缓解。在AAV的背景下,孤立的AIN是罕见的,但它显示出不同的临床,实验室,和病理特征。具有这种表现的患者对免疫抑制治疗表现出积极的反应。然而,AAV相关AIN的最终治疗指南的建立仍不确定,需要进一步研究以制定综合治疗指南.AIN,特别是当缺乏典型的肾小球病变时,可能代表MPO-AAV中的一个新亚组,需要额外的研究和临床关注。要点•这项研究通过强调MPO-AAV相关的间质性肾炎,即使没有新月形成,能表现出足细胞足过程消失,对治疗反应良好。•AIN的存在,独立于新月体肾小球肾炎,提示MPA-AAV中可能出现新的亚类。•值得注意的是,一些MPO-AAV相关AIN病例可能存在Scr水平正常的情况(表5,病例5,6和17).•这一观察结果突出了考虑肾活检的重要性,诊断,并及时治疗,以防止慢性肾脏病(CKD)的发展。
    Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) typically manifests as rapidly progressive glomerulonephritis with crescent formation. In this report, we present a local case of myeloperoxidase (MPO)-AAV-associated acute interstitial nephritis (AIN), showing slight pauci-immune glomerulonephritis and positive MPO-ANCA. This case is characterized by foot process effacement of podocytes in the glomerulus, a favorable prognosis, and an absence of crescentic formation. To further understand this condition, we conducted a comprehensive literature search on Google Scholar and PubMed, employing both free text words and MeSH terms related to \"AAV and AIN.\" This search yielded 24 cases, which we analyzed for their clinical features, laboratory findings, renal pathological characteristics, and therapeutic outcomes. AAV-associated interstitial nephritis predominantly affects elderly patients and is often associated with anemia, proteinuria, hematuria, and nonspecific manifestations, including fever, anorexia, fatigue, edema, and weight loss. Most of the cases in our review were MPO-ANCA-positive and exhibited isolated interstitial inflammation. These patients typically presented with relatively lower levels of serum creatinine, 24-h urine protein levels, and MPO-ANCA titers. All patients in our study received immunosuppressive therapy, including glucocorticoids, immunosuppressants, and rituximab, with the majority achieving clinical remission. Isolated AIN in the context of AAV is a rare occurrence, but it displays distinct clinical, laboratory, and pathological features. Patients with this presentation show a positive response to immunosuppressive treatment. Nevertheless, the establishment of definitive therapy guidelines for AAV-associated AIN remains uncertain and necessitates further investigation to develop comprehensive treatment guidelines. AIN, particularly when lacking typical glomerulus lesions, may represent a novel subgroup within MPO-AAV warranting additional research and clinical attention. Key Points • This study contributes valuable scientific insights by highlighting that MPO-AAV-associated interstitial nephritis, even without crescentic formation, can exhibit podocyte foot process effacement and respond well to treatment. • The presence of AIN, independent of crescentic glomerulonephritis, suggests the potential emergence of a new subclass within MPA-AAV. • Notably, some cases of MPO-AAV-associated AIN may present with normal levels of Scr (Table 5, cases 5, 6, and 17). • This observation highlights the importance of considering renal biopsy, diagnosis, and therapy in a timely manner to prevent the development of chronic kidney disease (CKD).
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  • 文章类型: Journal Article
    由严重急性呼吸道综合症冠状病毒2型(SARS-CoV-2)引起的2019年冠状病毒病(COVID-19)大流行对全球医疗保健构成了相当大的挑战。已报道SARS-CoV-2感染和疫苗接种后的急性间质性肾炎(AIN),但其临床特征和发病机制仍不清楚。我们回顾了迄今为止最大的22例SARS-CoV-2感染后的AIN和36例COVID-19疫苗接种后的AIN。AIN的发病主要与信使RNA疫苗有关(52.8%)。除了发烧,蛋白尿(45.5%)是SARS-CoV-2感染后AIN的主要表现,左急性肾损伤(AKI,63.9%)在COVID-19疫苗接种后的患者中。疫苗接种诱导AIN的潜在机制是将疫苗与蛋白质结合形成半抗原,激活树突状细胞并促进导致AIN的级联免疫反应。
    The 2019 coronavirus disease (COVID-19) pandemic caused by severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) has posed a considerable challenge to global healthcare. Acute interstitial nephritis (AIN) post SARS-CoV-2 infection and vaccination has been reported, but its clinical features and pathogenesis remained unclear. We reviewed so far the largest 22 cases of AIN post SARS-CoV-2 infection and 36 cases of AIN following COVID-19 vaccination. The onset of AIN was mainly related to messenger RNA vaccines (52.8%). Apart from fever, proteinuria (45.5%) was the main manifestation of AIN post SARS-CoV-2 infection, left acute kidney injury (AKI, 63.9%) in patients post COVID-19 vaccination. The potential mechanism of vaccination induced AIN was conjugating vaccines with proteins to form a hapten, which activated dendritic cells and promoted a cascade immunological reaction leading to AIN.
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  • 文章类型: Systematic Review
    目的:肾小管间质性肾炎和葡萄膜炎(TINU)综合征是一种罕见的疾病。我们提出了一个确诊的TINU综合征,以及对流行病学特征的系统评价,临床表现,管理,以及中国患者的预后。
    方法:使用定义的术语进行了系统搜索,并在PubMed上更新至2022年9月,WebofScience,万方,CNKI,VIP,为了确定中国报告的TINU病例,根据PRISMA指南。
    结果:一名18岁男孩,血清肌酐升高,24小时尿蛋白水平>2g。检查结果显示为急性肾小管间质性肾炎,和双侧葡萄膜炎。患者被诊断为TINU综合征,并接受甲基强的松龙琥珀酸钠治疗,导致肌酐和尿蛋白水平显着下降。系统审查确定了35种符合纳入标准的出版物。本文共纳入71例,其中70来自出版物,1来自我们医院。发病年龄中位数为42岁,男性显著低于女性(P<0.05)。葡萄膜炎的症状通常发生在肾损伤后(54%),大多数葡萄膜炎发生在前部(55%)和双侧(75%)。在随访6个月以上的51例患者中,24例反复出现眼部症状或进展为慢性葡萄膜炎。20名患者经历了慢性或进行性肾脏疾病。
    结论:TINU综合征容易误诊,因为肾脏损害可能不会与葡萄膜炎同时发生。儿童肾脏后遗症的发生率低于成人,糖皮质激素是首选治疗方法。
    INPLASY202350050。
    OBJECTIVE: Tubulointerstitial nephritis and uveitis (TINU) syndrome is an uncommon disease. We present a confirmed case of TINU syndrome, and a systematic review of epidemiological characteristics, clinical manifestations, management, and outcomes in Chinese patients.
    METHODS: A systematic search was carried out using defined terms and updated up to September 2022, in PubMed, Web of Science, Wanfang, CNKI, and VIP, to identify reported cases of TINU in China, according to PRISMA guidelines.
    RESULTS: An 18-year-old boy presented with elevated serum creatinine and 24-h urine protein level of > 2 g. Inspection result revealed acute tubulointerstitial nephritis, and bilateral uveitis. The patient was diagnosed with TINU syndrome and received treatment with methylprednisolone sodium succinate, which resulted in a significant decrease in creatinine and urinary protein levels. Systematic review identified 35 publications that met the inclusion criteria. A total of 71 cases were included in this article, of which 70 were from publications and 1 was from our hospital. The median age at onset was 42 years and was significantly lower in males than females (P < 0.05). The symptoms of uveitis often occurred after kidney injury (54%) and most uveitis was anterior (55%) and bilateral (75%). Among the 51 patients who were followed up for more than 6 months, 24 had recurrent ocular symptoms or progression to chronic uveitis. Twenty patients experienced chronic or progressive kidney disease.
    CONCLUSIONS: TINU syndrome is prone to misdiagnosis because kidney damage may not occur simultaneously with uveitis. The incidence of kidney sequelae in children is lower than that in adults, and glucocorticoids are the preferred treatment.
    UNASSIGNED: INPLASY202350050.
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  • 文章类型: Review
    背景:肾小管酸中毒是原发性干燥综合征患者肾小管间质性肾炎的主要临床特征。间质性肾炎引起的肾小管功能障碍被认为是连接肾小管酸中毒和原发性干燥综合征的潜在病理生理学。然而,原发性干燥综合征肾小管酸中毒病理生理学的详细机制尚不完全清楚.
    方法:一名30岁女性因四肢无力而入院。该患者13年前因类似问题住院,并因远端肾小管酸中毒伴原发性干燥综合征而被诊断为低钾性瘫痪。该诊断是基于Schirmer阳性测试。此外,还检测到抗舍格伦综合征相关抗原A。实验室测试表明远端RTA;然而,肾活检未见明显的间质性肾炎。第二次入院期间进行的实验室检查显示远端肾小管酸中毒。因此,再次进行了肾活检,显示间质性肾炎.酸碱转运蛋白的组织学分析显示,收集管中不存在液泡型H-ATPases。在过去的肾活检中也没有空泡型H-ATP酶,提示酸碱转运蛋白的改变与间质性肾炎无关。
    结论:本病例研究表明,空泡型H+-ATP酶与远端肾小管酸中毒有关,在原发性干燥综合征患者中,远端肾小管酸中毒先于间质性肾炎。
    Renal tubular acidosis is the principal clinical feature associated with tubulointerstitial nephritis in patients with primary Sjögren\'s syndrome. Renal tubular dysfunction due to interstitial nephritis has been considered the underlying pathophysiology connecting renal tubular acidosis and primary Sjögren\'s syndrome. However, the detailed mechanisms underlying the pathophysiology of renal tubular acidosis in primary Sjögren\'s syndrome is not fully understood.
    A 30-year-old woman was admitted with complaints of weakness in the extremities. The patient was hospitalized thirteen years earlier for similar issues and was diagnosed with hypokalemic paralysis due to distal renal tubular acidosis with primary Sjögren\'s syndrome. This diagnosis was based on a positive Schirmer\'s test. Besides, anti-Sjögren\'s syndrome-related antigen A was also detected. Laboratory tests indicated distal RTA; however, a renal biopsy showed no obvious interstitial nephritis. Laboratory tests conducted during the second admission indicated distal renal tubular acidosis. Therefore, a renal biopsy was performed again, which revealed interstitial nephritis. Histological analysis of acid-base transporters revealed the absence of vacuolar type H+-ATPases in the collecting duct. The vacuolar type H+-ATPase was also absent in the past renal biopsy, suggesting that the alteration in acid-base transporters is independent of interstitial nephritis.
    This case study demonstrates that vacuolar-type H+-ATPases are associated with distal renal tubular acidosis, and distal renal tubular acidosis precedes interstitial nephritis in patients with primary Sjögren\'s syndrome.
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  • 文章类型: Journal Article
    由于其广谱杀菌活性,阿莫西林(AMX)和第三代头孢菌素(TGC)广泛用于预防和治疗已确定的感染。它们被认为是相对安全的,但是最近的一些报道表明有很大的肾毒性,尤其是使用AMX。考虑到AMX和TGC在临床实践中的重要性,我们进行了最新的审查,使用PubMed数据库,特别关注这些分子的肾毒性。我们还简要回顾了AMX和TGC的药理学。AMX的肾毒性可能由几种病理生理机制驱动,如IV型超敏反应,过敏反应,或肾小管内和/或尿路药物沉淀。在这次审查中,我们关注AMX的两个主要肾脏不良反应,即急性间质性肾炎和晶体肾病。我们在发病率方面总结了当前的知识,发病机制,因素,临床特征,和诊断。这篇综述的目的也是强调AMX肾毒性的可能低估,并教育临床医生最近与晶体肾病相关的发病率增加和严重的肾脏预后。我们还提出了处理这些并发症的一些关键要素,以避免不当使用并限制肾毒性的风险。虽然TGC的肾损伤似乎更罕见,文献中已经报道了几种类型的肾毒性,比如肾结石,免疫介导的溶血性贫血,或者急性间质性肾病,我们在这篇综述的第二部分详细介绍了这一点。
    Because of their broad-spectrum bactericidal activity, amoxicillin (AMX) and third-generation cephalosporins (TGC) are widely used for the prophylaxis and treatment of established infections. They are considered relatively safe, but several recent reports have suggested substantial nephrotoxicity, especially with AMX use. Considering the importance of AMX and TGC for clinical practice, we conducted this up-to-date review, using the PubMed database, which focuses specifically on the nephrotoxicity of these molecules. We also briefly review the pharmacology of AMX and TGC. Nephrotoxicity of AMX may be driven by several pathophysiological mechanisms, such as a type IV hypersensitivity reaction, anaphylaxis, or intratubular and/or urinary tract drug precipitation. In this review, we focused on the two main renal adverse effects of AMX, namely acute interstitial nephritis and crystal nephropathy. We summarize the current knowledge in terms of incidence, pathogenesis, factors, clinical features, and diagnosis. The purpose of this review is also to underline the probable underestimation of AMX nephrotoxicity and to educate clinicians about the recent increased incidence and severe renal prognosis associated with crystal nephropathy. We also suggest some key elements on the management of these complications to avoid inappropriate use and to limit the risk of nephrotoxicity. While renal injury appears to be rarer with TGC, several patterns of nephrotoxicity have been reported in the literature, such as nephrolithiasis, immune-mediated hemolytic anemia, or acute interstitial nephropathy, which we detail in the second part of this review.
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  • 文章类型: Review
    核型间质性肾炎(KIN)是以肾小管上皮核增大为特征的慢性间质性肾炎的罕见原因。在肾移植中报告的第一例KIN是在2019年。这里,我们报告了2名兄弟中的首例KIN病例,他们接受了来自2名不同无关活体供者的肾脏。一名男性肾脏移植受者,以局灶性节段性肾小球硬化为原始肾脏疾病,表现为移植物受损和蛋白尿,移植活检显示KIN.该患者有一个兄弟,他也是肾移植受者,有一次移植物受损,也被诊断为KIN。
    Karyomegalic interstitial nephritis (KIN) is a rare cause of chronic interstitial nephritis characterized by enlarged renal tubular epithelial nuclei. The first case of KIN reported in a kidney graft was in 2019. Here, we report the first case of KIN in 2 brothers receiving kidneys from 2 different unrelated living donors. A male kidney transplant recipient with focal segmental glomerulosclerosis as the original kidney disease presented with graft impairment and proteinuria, and graft biopsy revealed KIN. This patient had a brother who was also a kidney transplant recipient and had one episode of graft impairment and was diagnosed with KIN as well.
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  • 文章类型: Review
    背景:IgG4相关疾病(IgG4-RD)是一种新发现的全身性疾病,可影响体内任何器官或组织。IgG4相关的肾脏疾病(IgG4-RKD)相对罕见,但对IgG4-RD至关重要。然而,IgG4-RD模拟输尿管恶性肿瘤导致肾积水的报道很少.我们在此报告一例罕见的IgG4-RD累及输尿管。
    方法:一名87岁的男子因厌食症出现在我们的肾脏科,恶心,以及2020年11月的急性肾损伤。尿液计算机断层扫描(CT)检查显示右下输尿管肿块伴右肾和输尿管肾积水。血清IgG4水平为1890mg/dL,同时肾活检显示肾间质中IgG4阳性浆细胞广泛浸润,被诊断为IgG4相关的小管间质性肾炎(IgG4-TIN)。右输尿管双J管植入和中等剂量激素治疗后肾功能明显改善。血清IgG4降到正常范围,低剂量激素维持治疗一年后,右下输尿管肿块几乎消失。
    结论:IgG4-RD可以表现为肾盂和(或)输尿管中的肿块,导致肾积水.因此,对这种疾病的早期识别意义重大。大多数患者对激素治疗反应良好,以避免因误诊为恶性肿瘤而进行手术治疗,对患者造成二次伤害。
    BACKGROUND: IgG4-related disease (IgG4-RD) is a newly discovered systemic disease that can affect any organ or tissue in the body. IgG4-related kidney disease (IgG4-RKD) is relatively rare but essential to IgG4-RD. However, there are few reports of IgG4-RD mimicking malignant ureteral tumors leading to hydronephrosis. We report here a rare case of IgG4-RD involving the ureter.
    METHODS: An 87-year-old man presented to our nephrology department with anorexia, nausea, and acute kidney injury in November 2020. Urinary computed tomography (CT) examination revealed a right lower ureter mass with right renal and ureter hydronephrosis. The serum level of IgG4 was 1890 mg/dL, and the concurrently renal biopsy revealed extensive infiltration of IgG4-positive plasma cells in renal interstitium, which was diagnosed as IgG4-associated tubule-interstitial nephritis(IgG4-TIN). The renal function improved significantly after double-J tube implantation of the right ureter and moderate-dose hormone therapy. The serum IgG4 decreased to the normal range, and the right lower ureter mass almost disappeared after one year of low-dose hormone maintenance therapy.
    CONCLUSIONS: IgG4-RD can present as a mass in the renal pelvis and (or) ureter, leading to hydronephrosis. Therefore, early recognition of this disease is significant. Most patients respond well to hormonal therapy to avoid surgical treatment due to misdiagnosis as malignant tumors, causing secondary harm to patients.
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  • 文章类型: Case Reports
    结节病是一种病因不明的非坏死性肉芽肿性炎症性多系统疾病。在儿童中,就像成年人一样,它可以在不同程度和程度上涉及几个或所有器官系统,涉及多系统表现。小儿起病的成人结节病的肾脏受累很少见,广泛的肾脏表现,其中大部分与钙代谢有关。儿童肾结节病往往比成人更有症状,虽然男性患者的患病率较高。我们介绍了一个10岁男孩的病例,该男孩患有晚期肾衰竭并伴有肾钙质沉着和重要的肝脾肿大。诊断是通过组织病理学检查确定的,随之而来的可的松治疗和血液透析。这篇综述强调,结节病在急性肾功能不全或病因不明的慢性肾脏疾病的儿科患者的鉴别诊断中应考虑。据我们所知,这是关于罗马尼亚儿童肺外结节病的第一项研究.
    Sarcoidosis is a non-necrotizing granulomatous inflammatory multisystemic disorder of unknown etiology. In children, as in adults, it can involve a few or all organ systems to a varying extent and degree, entailing multisystemic manifestations. Kidney involvement in pediatric-onset adult-type sarcoidosis is rare, with a wide range of renal manifestations, most of them related to calcium metabolism. Children with renal sarcoidosis tend to be more symptomatic than adults, although male patients have a higher prevalence. We present the case of a 10-year-old boy who presented with advanced renal failure with nephrocalcinosis and important hepatosplenomegaly. The diagnosis was established by histopathological examination, with consequent cortisone therapy and hemodialysis. This review emphasizes that sarcoidosis should be considered in the differential diagnosis of pediatric patients with acute kidney insufficiency or chronic kidney disease of an unknown etiology. As far as we know, this is the first study regarding extrapulmonary sarcoidosis in children from Romania.
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