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A 24-Year-Old Female Transplant Recipient with Type 2 Membranoproliferative Glomerulonephritis and Disseminated Shingles: A Cautionary Tale of Deferring to Primary Care. 24岁女性2型膜增生性肾小球肾炎和播散性带状疱疹移植受体:推迟初级保健的警示故事。
Pub Date : 2021-07-24 eCollection Date: 2021-01-01 DOI: 10.1155/2021/6663689
Benjamin T Burdorf

In this report, the case of a 24-year-old Caucasian female with type 2 membranoproliferative glomerulonephritis status-post living donor kidney transplant managed on triple regimen immunosuppressive therapy who developed shingles is discussed. With its onset, she promptly reached out to her nephrologist who deferred her to primary care. Prior to seeing her primary provider, she developed disseminated herpes zoster. She consulted emergency services where she was given inadequate care and again deferred to primary care. One day later, the dissemination included her entire torso, face, oral cavity, and all extremities. Fortunately, the patient had the insight to again reach out to her nephrologist who arranged for her to be admitted for appropriate care 6 days after her initial inquiry that carried 6 days of zoster progression. This case demonstrates how it is pertinent that specialists recognize potentially lethal complications associated with the conditions they follow. Although convenient to defer to primary care, if specialists were to take on the responsibility of providing a broader scope of care for their unique subsets of patients, it would likely result in a reduction in the 80% of serious medical errors that occur as a result of miscommunication, or lack thereof, between care providers.

在这篇报告中,我们讨论了一位24岁的白人女性,她患有2型膜增生性肾小球肾炎,在活体肾移植后接受三联免疫抑制治疗,并发带状疱疹。发病后,她立即联系了她的肾病科医生,后者将她推迟到初级保健部门。在看主治医生之前,她出现了播散性带状疱疹。她咨询了急救服务,但没有得到充分的照顾,于是再次求助于初级保健。一天后,她的整个躯干、面部、口腔和四肢都进行了扩散。幸运的是,患者有洞察力,再次联系她的肾脏科医生,安排她入院接受适当的治疗,在她最初的询问后6天,带状疱疹进展。这个病例表明,专家认识到与他们所遵循的条件相关的潜在致命并发症是如何相关的。虽然服从初级保健很方便,但如果专科医生承担起责任,为他们独特的病人群体提供更广泛的护理,这可能会减少80%的严重医疗事故,这些事故是由于护理提供者之间的沟通不端或缺乏沟通造成的。
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引用次数: 0
Atypical Antiglomerular Basement Membrane Disease in a Pediatric Patient Successfully Treated with Rituximab. 非典型抗肾小球基底膜病的儿童患者成功治疗利妥昔单抗。
Pub Date : 2021-07-17 eCollection Date: 2021-01-01 DOI: 10.1155/2021/2586693
Kuang-Yu Jen, Ari Auron

Classic antiglomerular basement membrane (anti-GBM) disease is an exceedingly rare but extremely aggressive form of glomerulonephritis, typically caused by autoantibodies directed against cryptic, conformational epitopes within the noncollagenous domain of the type IV collagen alpha-3 subunit. Pathologic diagnosis is established by the presence of strong, diffuse, linear staining for immunoglobulin on immunofluorescence microscopy. Recently, patients with atypical clinical and pathologic findings of anti-GBM disease have been described. These patients tend to have an indolent clinical course, without pulmonary involvement, and laboratory testing rarely reveals the presence of anti-GBM antibodies. Specific guidelines for the treatment and management of these patients are unclear. Here, we describe a case of atypical anti-GBM disease in a young child who presented with hematuria and prominent proteinuria. Throughout the course of his illness, creatinine remained normal. He was conservatively treated with steroids and rituximab, resulting in resolution of his clinical symptoms and normalization of laboratory findings.

典型的抗肾小球基底膜(anti-GBM)疾病是一种极为罕见但极具侵袭性的肾小球肾炎,通常由针对IV型胶原α -3亚基非胶原结构域内的隐型构象表位的自身抗体引起。病理诊断是建立在免疫荧光显微镜下免疫球蛋白强,弥漫,线性染色的存在。最近,抗gbm疾病的非典型临床和病理表现的患者已被描述。这些患者往往有一个惰性的临床过程,没有肺部累及,实验室检测很少显示抗gbm抗体的存在。治疗和管理这些患者的具体指南尚不清楚。在这里,我们描述了一个不典型的抗gbm疾病在一个年轻的孩子谁提出血尿和突出的蛋白尿。在整个病程中,肌酐保持正常。他接受了类固醇和利妥昔单抗的保守治疗,导致他的临床症状得到缓解,实验室检查结果正常化。
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引用次数: 1
A Complex Case of Emphysematous Cystitis in a Peritoneal Dialysis Patient. 腹膜透析患者并发复杂肺气肿性膀胱炎1例。
Pub Date : 2021-07-07 eCollection Date: 2021-01-01 DOI: 10.1155/2021/8343022
Christina Okello, Rajesh Raj

Emphysematous cystitis (EC) is a relatively rare condition characterized by gas formation in the bladder wall and/or lumen. We report a case of emphysematous cystitis with a bladder perforation in an 84-year-old male on peritoneal dialysis who presented with fever, dysuria, hematuria, and hypotension. Gas in the bladder wall, as well as a small perforation in the roof of the urinary bladder, was seen on the abdominal CT scan. The causative organism identified was Escherichia coli. The patient recovered with broad-spectrum antibiotics along with bladder irrigation and drainage. After initial bladder washouts, peritoneal dialysis was continued with close monitoring. Early antibiotic therapy and a conservative approach to the management of small intraperitoneal bladder perforations were effective in this patient. Peritoneal dialysis was uninterrupted for the duration of the admission and after discharge.

肺气肿性膀胱炎(EC)是一种相对罕见的疾病,其特征是膀胱壁和/或腔内形成气体。我们报告一例肺气肿性膀胱炎伴膀胱穿孔,患者为84岁男性,接受腹膜透析,表现为发热、排尿困难、血尿和低血压。腹部CT扫描发现膀胱壁上有气体,膀胱顶部有小穿孔。病原菌为大肠杆菌。患者经广谱抗生素治疗及膀胱冲洗引流后恢复。在最初的膀胱冲洗后,继续进行腹膜透析并密切监测。早期抗生素治疗和保守的方法来管理小腹膜膀胱穿孔是有效的在这个病人。入院期间及出院后均不间断腹膜透析。
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引用次数: 0
Bartonella-Associated Endocarditis with Severe Active Crescentic Glomerulonephritis and Acute Renal Failure. 巴尔通体相关心内膜炎合并严重活动性月牙状肾小球肾炎和急性肾功能衰竭。
Pub Date : 2021-05-29 eCollection Date: 2021-01-01 DOI: 10.1155/2021/9951264
Ramanath Dukkipati, Benjamin Lawson, Cynthia C Nast, Anuja Shah

We report a case of severe acute kidney failure due to crescentic glomerulonephritis who presented initially with culture-negative endocarditis with vegetations on the aortic valve. Anti-nuclear and anti-phospholipid antibodies were positive with initially negative anti-neutrophil cytoplasmic antibodies (ANCAs). Kidney biopsy revealed severe acute crescentic glomerulonephritis with mesangial immune complex deposition. PR3-ANCA subsequently become positive, and the patient developed worsening kidney failure requiring hemodialysis. This case illustrates that Bartonella can present as culture-negative endocarditis with severe crescentic glomerulonephritis with positive PR-3 ANCAs and can mimic ANCA-associated crescentic glomerulonephritis.

我们报告一例严重急性肾衰竭,由于月牙状肾小球肾炎,最初表现为培养阴性心内膜炎和主动脉瓣上的植被。抗核抗体和抗磷脂抗体阳性,抗中性粒细胞胞浆抗体(ANCAs)初始阴性。肾活检显示严重急性新月形肾小球肾炎伴系膜免疫复合物沉积。PR3-ANCA随后呈阳性,患者肾功能衰竭加重,需要血液透析。本病例表明巴尔通体可表现为培养阴性心内膜炎合并严重新月体肾小球肾炎,伴PR-3 anca阳性,并可模拟anca相关的新月体肾小球肾炎。
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引用次数: 3
Profound Metabolic Acidosis due to Metformin Intoxication Requiring Dialysis. 二甲双胍中毒引起的需要透析的深度代谢性酸中毒。
Pub Date : 2021-05-24 eCollection Date: 2021-01-01 DOI: 10.1155/2021/9914982
Klodia Hermez, Carla Dudash-Mion

Metformin-associated lactic acidosis (MALA) is a rare but life-threatening condition with often high mortality rates. Despite this, metformin continues to be one of the most commonly prescribed antihyperglycemic agents in the market. We present a unique case of a 61-year-old female with severe acidosis of pH = 6.72 and lactic acid of 26 mmol/L who presented obtunded after ingestion of an unknown amount of metformin. She was subsequently intubated, became hypotensive, and was initiated on vasopressors. She was swiftly started on a combination of intermittent hemodialysis (IHD) and bicarbonate therapy 7 hours after admission followed by continuous renal replacement therapy (CRRT) as she became more hemodynamically unstable. The patient's renal function improved, and she was discharged 7 days after admission with favorable sequelae. Dialysis is often reported in cases of severe MALA; however, it remains unclear how quickly dialysis should be initiated. This case aims to explore the benefits of quick initiation of extracorporeal measures in the forms of IHD and CRRT with concurrent bicarbonate supplementation. Furthermore, this case demonstrates the importance of clinical suspicion in metabolic acidosis in a patient on metformin therapy.

二甲双胍相关性乳酸酸中毒(MALA)是一种罕见但危及生命的疾病,死亡率通常很高。尽管如此,二甲双胍仍然是市场上最常用的抗高血糖药物之一。我们提出了一个独特的情况下,61岁的女性严重酸中毒pH = 6.72和乳酸26毫摩尔/升谁在摄入不明数量的二甲双胍后出现昏倒。她随后插管,出现低血压,并开始使用血管加压药物。入院后7小时,她迅速开始间歇性血液透析(IHD)和碳酸氢盐联合治疗,随后持续肾替代治疗(CRRT),因为她的血流动力学变得更加不稳定。患者肾功能改善,入院7天后出院,后遗症良好。在严重MALA病例中经常报告透析;然而,目前尚不清楚透析应该多快开始。本病例旨在探讨快速启动IHD和CRRT形式的体外措施并同时补充碳酸氢盐的益处。此外,该病例表明,在接受二甲双胍治疗的患者中,临床怀疑代谢性酸中毒的重要性。
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引用次数: 5
A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid. 严重难治性膜性肾病伴黏膜类天疱疮1例。
Pub Date : 2021-05-11 eCollection Date: 2021-01-01 DOI: 10.1155/2021/9940293
Eric Mereniuk, Laura Sabbah, Jean-Paul Makhzoum

Primary membranous nephropathy (MN) and mucous membrane pemphigoid (MMP) are two autoimmune conditions with well-defined diagnostic and treatment guidelines. MN has been linked to bullous pemphigoid (BP) in certain case reports, though little is known regarding the association of MN and other bullous diseases. The association of MN and MMP has rarely been described, and very little data exist regarding the treatment of this association. We report a case of severe refractory membranous nephropathy secondary to mucous membrane pemphigoid successfully treated with rituximab. A 35-year-old woman with known MMP was referred to our clinic for new-onset generalized edema and proteinuria. MN was confirmed on renal biopsy. Despite therapy with high-dose systemic glucocorticoids, combined with mycophenolate mofetil, and later azathioprine, nephrotic-range proteinuria persisted even at a daily dose of prednisone of 40 mg. The patient was then started on rituximab infusions, which induced remission of both mucous membrane pemphigoid and membranous glomerulonephritis. This suggests that MN can be secondary to MMP, and rituximab may be useful induce remission in cases that are refractory to standard therapy.

原发性膜性肾病(MN)和粘膜类天疱疮(MMP)是两种自身免疫性疾病,具有明确的诊断和治疗指南。在某些病例报告中,MN与大疱性类天疱疮(BP)有关,尽管MN与其他大疱性疾病的关系尚不清楚。MN和MMP的关联很少被描述,关于这种关联的治疗也很少有数据存在。我们报告一例严重难治性膜性肾病继发于粘膜类天疱疮成功治疗利妥昔单抗。一名35岁女性已知MMP被转介到我们的诊所新发全身性水肿和蛋白尿。肾活检证实MN。尽管使用大剂量全身糖皮质激素,联合霉酚酸酯和后来的硫唑嘌呤治疗,肾范围蛋白尿即使在每日40mg强的松剂量下仍持续存在。然后患者开始输注利妥昔单抗,引起粘膜类天疱疮和膜性肾小球肾炎的缓解。这表明MN可能继发于MMP,利妥昔单抗可能有助于在标准治疗难治性病例中诱导缓解。
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引用次数: 3
A Rare Case of Immunotactoid Glomerulopathy Associated with Hodgkin Lymphoma. 免疫因子样肾小球病变合并霍奇金淋巴瘤1例。
Pub Date : 2021-05-06 eCollection Date: 2021-01-01 DOI: 10.1155/2021/5527966
Keiki Nagaharu, Yuka Sugimoto, Keiki Kawakami

Immunotactoid glomerulopathy (ITG) is characterized by Congo red-negative microtubular deposits, and it has been reported as a rare paraneoplastic syndrome due to hematologic malignancies, viral infections, or autoimmune diseases. In hematologic malignancies, multiple myeloma and other mature B-cell malignancies are the most common hematologic malignancies, and Hodgkin lymphoma (HL) is extremely rare. A 59-year-old woman was admitted to our hospital because of a pulmonary mass and proteinuria. Computed tomography-guided lung biopsy confirmed the presence of HL stage IIA. Immunofixation of peripheral blood was positive for immunoglobulin G (IgG) kappa. Renal biopsy showed mesangial proliferation with deposits in the subendothelial lesion and no invasion of the HL. These deposits were positive for IgG3, C3, and kappa light chain but negative for C1q and lambda light chain. Electron microscopy showed randomly aligned tubular structures with a diameter of approximately 50 nm. We diagnosed the patient with immunotactoid nephropathy and HL. After systemic chemotherapy, the patient achieved a complete response and loss of proteinuria. On the contrary, her serum monoclonal gammopathy was observed after chemotherapy. The existence of a monoclonal antibody itself might not be a sufficient factor for ITG in some cases, and an additive trigger is necessary for development.

免疫因子样肾小球病(ITG)以刚果红阴性微管沉积为特征,是一种罕见的副肿瘤综合征,由血液系统恶性肿瘤、病毒感染或自身免疫性疾病引起。在血液恶性肿瘤中,多发性骨髓瘤和其他成熟b细胞恶性肿瘤是最常见的血液恶性肿瘤,霍奇金淋巴瘤(HL)极为罕见。一位59岁的女性因肺部肿块和蛋白尿而入院。计算机断层引导下的肺活检证实了HL IIA期的存在。外周血免疫固定免疫球蛋白G (IgG) kappa阳性。肾活检显示肾小球系膜增生,内皮下病变有沉积物,未侵犯HL。这些沉积物中IgG3、C3和kappa轻链呈阳性,而C1q和lambda轻链呈阴性。电镜显示随机排列的管状结构,直径约50纳米。我们诊断患者为类免疫球蛋白肾病和HL。全身化疗后,患者完全缓解,蛋白尿消失。化疗后血清单克隆γ病变。在某些情况下,单克隆抗体本身的存在可能不是ITG的充分因素,并且需要一个附加的触发因素才能发展。
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引用次数: 1
Rifampicin-Associated Secondary Minimal Change Disease Presenting with Nephrotic Syndrome in a Pulmonary Tuberculosis Patient. 以肾病综合征为表现的肺结核患者利福平相关继发性微小改变疾病
Pub Date : 2021-04-14 eCollection Date: 2021-01-01 DOI: 10.1155/2021/5546942
Satyanand Sathi, Anil Kumar Garg, Manoj Kumar Singh, Virendra Singh Saini, Devinder Vohra

Various extraglomerular disease processes have been associated with drug-induced secondary minimal change disease (MCD). In a majority of cases, preferably, a hypersensitivity reaction appears to be involved, and in some cases, there is direct toxic effect over glomerular capillaries. There are several reports to demonstrate that rifampicin has been associated with various nephrotoxic adverse effects, but rifampicin-induced secondary minimal change disease (MCD) is very rare. Here, we report the case of a young adult male who presented with nephrotic proteinuria with bland urine sediment after one month of initiation of rifampicin treatment for pulmonary tuberculosis. The patient had no proteinuria before the start of antituberculosis treatment. Renal biopsy showed nonproliferative glomerulopathy and immunofluorescence did not show significant glomerular immune deposits. Electron microscopy showed diffuse effacement of visceral epithelial cell foot processes and did not show any presence of glomerular immune complexes and thickening of glomerular basement membrane, promoting the diagnosis of minimal change nephrotic syndrome. The patient got complete remission after discontinuation of rifampicin.

各种肾小球外疾病过程与药物诱导的继发性最小改变病(MCD)有关。在大多数情况下,最好是超敏反应,在某些情况下,对肾小球毛细血管有直接的毒性作用。有几份报告表明,利福平与各种肾毒性不良反应有关,但利福平诱导的继发性最小改变病(MCD)非常罕见。在这里,我们报告一例年轻的成年男性,在开始利福平治疗肺结核一个月后,出现了肾病性蛋白尿和淡性尿沉淀。患者在开始抗结核治疗前无蛋白尿。肾活检显示非增生性肾小球病变,免疫荧光未显示明显的肾小球免疫沉积。电镜显示内脏上皮细胞足突弥漫性消失,未见肾小球免疫复合物和肾小球基底膜增厚,提示微小改变肾病综合征的诊断。患者停用利福平后病情完全缓解。
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引用次数: 2
A Case of De Novo Antiglomerular Basement Membrane Disease Presenting during Pregnancy. 妊娠期新发抗肾小球基底膜病1例。
Pub Date : 2021-03-17 eCollection Date: 2021-01-01 DOI: 10.1155/2021/5539205
F A K Lodhi, T Akcan, J N Mojarrab, S Sajjad, R Blonsky

Background. Acute kidney injury (AKI) requiring dialysis during pregnancy is uncommon. We present a case of a young female diagnosed with antiglomerular basement membrane (anti-GBM) disease during pregnancy. Case Presentation. A 23-year-old woman approximately 15 weeks pregnant experienced weakness, nausea, vomiting, and anorexia for one week and anuria for 48 hours. No known drug allergies and no significant social or family history for kidney or genitourinary disease were reported. Laboratory analysis revealed anemia, life-threatening hyperkalemia, AKI, and elevated antiglomerular basement membrane (GBM) antibodies. Renal biopsy revealed 100% cellular crescents, confirming the diagnosis. The patient was treated using plasmapheresis and methylprednisolone followed by oral steroids, azathioprine, and tacrolimus. At 24 weeks and 4 days of gestation, the patient had hypoxic respiratory failure as well as preterm premature rupture of membranes. Due to the development of infection and lack of renal recovery, immunosuppression was discontinued. At 28 weeks and 0 days of gestation, the patient developed uncontrollable hypertension requiring emergent delivery. Postpartum, her hypertension improved without signs of preeclampsia though still requires dialysis. Discussion. Pregnancy presents a unique challenge for providers treating patients with anti-GBM disease. Fetal safety should be considered and risks thoroughly discussed with the patient when choosing an immunosuppressive regimen for this condition.

背景。妊娠期需要透析的急性肾损伤(AKI)并不常见。我们提出一个病例的年轻女性诊断为抗肾小球基底膜(抗gbm)疾病在怀孕期间。案例演示。一名怀孕约15周的23岁妇女出现虚弱、恶心、呕吐和厌食一周,无尿48小时。没有已知的药物过敏,也没有肾脏或泌尿生殖系统疾病的显著社会或家族史。实验室分析显示贫血、危及生命的高钾血症、AKI和抗肾小球基底膜(GBM)抗体升高。肾活检显示100%细胞新月形,证实了诊断。患者接受血浆置换和甲基强的松龙治疗,随后口服类固醇、硫唑嘌呤和他克莫司。在妊娠24周零4天,患者出现了缺氧呼吸衰竭和早产胎膜早破。由于感染的发展和肾脏缺乏恢复,停止免疫抑制。在妊娠28周零天时,患者出现无法控制的高血压,需要紧急分娩。产后,她的高血压有所改善,没有先兆子痫的迹象,但仍需要透析。讨论。妊娠是治疗抗gbm疾病患者的独特挑战。在选择免疫抑制方案时,应考虑胎儿安全,并与患者充分讨论风险。
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引用次数: 1
Targeting Cytokine Storm in COVID-19: A Role of Online Hemodiafiltration with Asymmetric Cellulose Triacetate in Maintenance Hemodialysis Patients-A Report of 10 Cases. 针对 COVID-19 中的细胞因子风暴:使用三醋酸非对称纤维素进行在线血液滤过在维持性血液透析患者中的作用--10 个病例的报告。
Pub Date : 2021-03-12 eCollection Date: 2021-01-01 DOI: 10.1155/2021/5575928
José C De La Flor, Francisco Valga, Alexander Marschall, Tania Monzon, Cristina Albarracín, Elisa Ruiz, Miguel Rodeles

Early reports have suggested that maintenance hemodialysis (MHD) patients could be more susceptible to a severe course of COVID-19. Among the therapeutic approaches, the use of drugs that reduce the cytokine storm characteristic of this disease has been proposed. Some dialyzers, such as the new generation of asymmetric cellulose triacetate (ATA) membranes, could favor the effective elimination of medium-sized molecules and other inflammatory mediators. In this case series, we describe in depth the clinical, analytical, and radiological details, therapeutic aspects, and outcomes of the case series of 10 MHD patients of our dialysis unit, who tested positive for SARS-CoV-2 from 5 October to 30 November 2020. Furthermore, we evaluate the removal of hyperinflammatory parameters with the ATA membrane in postdilution online hemodiafiltration (OL-HDF) in these patients through a variety of biomarkers of systemic inflammation from the diagnosis until stripping. Biochemical blood analysis was carried out at baseline and at days 7 and 14 after diagnosis, respectively. 50% of the patients presented COVID-19 pneumonia and required hospital admission. Median hospitalization time was 21 days. A total of 4 patients developed severe pneumonia (3 of them died) and 1 patient developed moderate pneumonia. Patients who died (n = 3) were more likely to present bilateral pneumonia (100% vs 14.3%) at diagnosis and less reduction in interleukin 6 (IL-6) at day 14, as compared to those who survived. The use of the ATA membrane could be considered a therapeutic option, due to its immunomodulatory effect in MHD patients with SARS-CoV-2 infection, especially at the beginning of the disease, where the inflammatory component is predominant.

早期报告显示,维持性血液透析(MHD)患者更容易出现严重的 COVID-19 病程。在治疗方法中,有人建议使用能减少这种疾病特有的细胞因子风暴的药物。一些透析器,如新一代非对称三醋酸纤维素(ATA)膜,可以有效清除中型分子和其他炎症介质。在本病例系列中,我们深入介绍了 2020 年 10 月 5 日至 11 月 30 日期间,本透析室 10 例对 SARS-CoV-2 检测呈阳性的 MHD 患者的临床、分析和放射学细节、治疗方面和结果。此外,我们还通过从诊断到剥离的各种全身炎症生物标志物,评估了这些患者在稀释后在线血液透析(OL-HDF)中使用 ATA 膜去除高炎症参数的情况。分别在基线和诊断后第 7 天和第 14 天进行了血液生化分析。50%的患者出现 COVID-19 肺炎,需要入院治疗。住院时间中位数为 21 天。共有 4 名患者发展为重症肺炎(其中 3 人死亡),1 名患者发展为中度肺炎。与存活的患者相比,死亡患者(n = 3)在诊断时更有可能出现双侧肺炎(100% vs 14.3%),且在第 14 天时白细胞介素 6(IL-6)的下降幅度较小。由于 ATA 膜对感染 SARS-CoV-2 的 MHD 患者具有免疫调节作用,尤其是在疾病初期,炎症成分占主导地位,因此可以将其作为一种治疗选择。
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引用次数: 0
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Case Reports in Nephrology
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