Clinical nephrology. Case studies最新文献_第3页

Monoclonal gammopathy of renal significance: An atypical presentation of Waldenström's disease. 具有肾脏意义的单克隆丙种球蛋白病：瓦尔登斯特伦病的一种非典型表现。

Clinical nephrology. Case studies Pub Date : 2024-02-08 eCollection Date: 2024-01-01 DOI: 10.5414/CNCS111200

Pablo Rodríguez-Doyágüez, Motornaya-Morozova, Patricia Martínez-Miguel, Carolina Castillo-Torres, Óscar Toldos-González, Juan José Gil-Fernández

{"title":"Monoclonal gammopathy of renal significance: An atypical presentation of Waldenström's disease.","authors":"Pablo Rodríguez-Doyágüez, Motornaya-Morozova, Patricia Martínez-Miguel, Carolina Castillo-Torres, Óscar Toldos-González, Juan José Gil-Fernández","doi":"10.5414/CNCS111200","DOIUrl":"10.5414/CNCS111200","url":null,"abstract":"Waldenström's disease is a rare lymphoproliferative syndrome in the bone marrow and sometimes in lymphoid organs which secretes high amounts of monoclonal immunoglobulin M into serum. It can remain indolent for years and rarely affects the kidney, with intraglomerular rather than intratubular damage being predominant, in contrast to multiple myeloma. Different studies identified AL amyloidosis as the most frequent renal lesion, followed by cryoglobulinemic glomerulonephritis. Signs and symptoms may be unspecific, as well as renal manifestations, so collaboration between nephrologists, hematologists, and pathologists is crucial to establish the role of paraprotein in the development of renal damage. We present an atypical case of Waldenström's disease that had a minimal monoclonal peak and clinically debuted with nephritic and nephrotic syndromes. The diagnosis was cryoglobulinemic glomerulonephritis. Currently, there are numerous treatment options, without enough evidence yet to establish a standardised treatment.","PeriodicalId":510898,"journal":{"name":"Clinical nephrology. Case studies","volume":"12 ","pages":"17-21"},"PeriodicalIF":0.0,"publicationDate":"2024-02-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10862989/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139731451","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}

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A case report of atypical anti-glomerular basement membrane nephritis associated with Mycobacterium Avium. 与禽分枝杆菌相关的非典型抗肾小球基底膜肾炎病例报告。

Clinical nephrology. Case studies Pub Date : 2024-01-12 eCollection Date: 2024-01-01 DOI: 10.5414/CNCS111254

Julie Bech Jensen, Eva Gravesen, Sidse Graff Jensen, Iain Bressendorff

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Calciphylaxis, beware the ophthalmic mimic: A case series. 钙化性紫癜，小心眼科疑似病例：病例系列。

Clinical nephrology. Case studies Pub Date : 2023-12-12 eCollection Date: 2023-01-01 DOI: 10.5414/CNCS111088

Chelsea Guymer, Sadia Jahan, Bobak Bahrami, David Sia, Bee Qung Tan, Stephen McDonald, Sumu Simon

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Concomitant presentation of thrombotic thrombocytopenic purpura, immune thrombocytopenia, and autoimmune hemolytic anemia in a patient with newly diagnosed systemic lupus erythematosus. 一名新确诊的系统性红斑狼疮患者同时出现血栓性血小板减少性紫癜、免疫性血小板减少症和自身免疫性溶血性贫血。

Clinical nephrology. Case studies Pub Date : 2023-12-12 eCollection Date: 2023-01-01 DOI: 10.5414/CNCS111193

Lina Bruns, Linus Völker, Robert Klamroth, Martin K Kuhlmann, Wolfram J Jabs

{"title":"Concomitant presentation of thrombotic thrombocytopenic purpura, immune thrombocytopenia, and autoimmune hemolytic anemia in a patient with newly diagnosed systemic lupus erythematosus.","authors":"Lina Bruns, Linus Völker, Robert Klamroth, Martin K Kuhlmann, Wolfram J Jabs","doi":"10.5414/CNCS111193","DOIUrl":"10.5414/CNCS111193","url":null,"abstract":"Thrombocytopenia is always of concern when encountered in emergency settings. We report a case of a 29-year-old women in whom a unique constellation of hematological disorders occurred. The patient had been diagnosed with idiopathic immune thrombocytopenia (ITP) in 2007, with a history of several thrombocytopenic flares. She now presented with homonymous hemianopia accompanied by thrombocytopenia and microangiopathic hemolytic anemia (MAHA) and was soon after diagnosed with a posterior stroke. Symptoms were more reminiscent of acquired thrombotic thrombocytopenic purpura (aTTP) rather than ITP. Immediate treatment with plasma exchange and caplacizumab curtailed MAHA, and progressive ischemic disease was averted. ADAMTS-13 testing confirmed the diagnosis of immune-mediated aTTP. Repeated testing for ITP, however, also showed IgG-loaded thrombocytes with the former known anti-GPIIb/IIIa specificity. Furthermore, autoimmune hemolytic anemia (AIHA) could be detected by direct antiglobulin test showing IgG and complement loading of the patient's erythrocytes. The autoimmune background of all three entities suggested an underlying systemic disease. Indeed, systemic lupus erythematosus (SLE) serology was strongly positive allowing for the diagnosis of SLE. ITP and AIHA as well as aTTP can be secondary to SLE, but emergence of all three disorders has not been reported at the same time.","PeriodicalId":510898,"journal":{"name":"Clinical nephrology. Case studies","volume":"11 ","pages":"147-153"},"PeriodicalIF":0.0,"publicationDate":"2023-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10759207/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139089897","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: 0