There are several conditions that have the clinical phenotype of the hemolytic uremic syndrome (HUS). This phenotype consists of acute hemolytic anemia with fragmented erythrocytes, thrombocytopenia, and acute renal injury. The Typical or classical from of HUS tends to occur in infants and children, usually in association with an acute episode of bloody diarrhea, may be mild or severe, and frequently has a good prognosis. The commonest cause of this form of HUS is a verotoxin-producing bacterium such as E. coli O157: H7, or a shiga toxin-producing shigella bacterium. This form of HUS cannot be confused easily with thrombotic thrombocytopenic purpura. Recent studies have shown that these patients have very high levels of elastase, and that most of them have a typical pattern of circulating VIII: vwf multimers. There are two groups of Inherited forms of HUS: autosomal recessive and autosomal dominant. These patients tend to have an insidious onset of HUS with either no predisposing cause, or they may have a preceding “viral” infection. The clinical condition is often progressive, and death or end stage renal failure often occur.
{"title":"The hemolytic uremic syndromes: Pathogenesis.","authors":"B. Kaplan","doi":"10.3165/JJPN.3.1","DOIUrl":"https://doi.org/10.3165/JJPN.3.1","url":null,"abstract":"There are several conditions that have the clinical phenotype of the hemolytic uremic syndrome (HUS). This phenotype consists of acute hemolytic anemia with fragmented erythrocytes, thrombocytopenia, and acute renal injury. The Typical or classical from of HUS tends to occur in infants and children, usually in association with an acute episode of bloody diarrhea, may be mild or severe, and frequently has a good prognosis. The commonest cause of this form of HUS is a verotoxin-producing bacterium such as E. coli O157: H7, or a shiga toxin-producing shigella bacterium. This form of HUS cannot be confused easily with thrombotic thrombocytopenic purpura. Recent studies have shown that these patients have very high levels of elastase, and that most of them have a typical pattern of circulating VIII: vwf multimers. There are two groups of Inherited forms of HUS: autosomal recessive and autosomal dominant. These patients tend to have an insidious onset of HUS with either no predisposing cause, or they may have a preceding “viral” infection. The clinical condition is often progressive, and death or end stage renal failure often occur.","PeriodicalId":325052,"journal":{"name":"Nihon Shoni Jinzobyo Gakkai Zasshi","volume":"34 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1990-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121147457","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.3165/JJPN.CR.2017.0118
Tetsuro Matsuhashi, N. Kumagai, M. Nakayama, H. Shima, Ikumi Umeki, Junko Kanno, S. Kure
{"title":"A case of purpura nephritis complicated by nephrotic syndrome and showing membranoproliferative glomerulonephritis-like findings for which frequent steroid pulse therapy was effective","authors":"Tetsuro Matsuhashi, N. Kumagai, M. Nakayama, H. Shima, Ikumi Umeki, Junko Kanno, S. Kure","doi":"10.3165/JJPN.CR.2017.0118","DOIUrl":"https://doi.org/10.3165/JJPN.CR.2017.0118","url":null,"abstract":"","PeriodicalId":325052,"journal":{"name":"Nihon Shoni Jinzobyo Gakkai Zasshi","volume":"8 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115240710","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Etsuko Tanaka, T. Konomoto, Hideaki Imamura, M. Orita, H. Sakaguchi, Akihiko Nakahara, H. Nunoi
{"title":"Evaluation of cytochrome c in hemolytic uremic syndrome","authors":"Etsuko Tanaka, T. Konomoto, Hideaki Imamura, M. Orita, H. Sakaguchi, Akihiko Nakahara, H. Nunoi","doi":"10.3165/JJPN.28.68","DOIUrl":"https://doi.org/10.3165/JJPN.28.68","url":null,"abstract":"","PeriodicalId":325052,"journal":{"name":"Nihon Shoni Jinzobyo Gakkai Zasshi","volume":"88 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115620396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.3165/JJPN.CR.2016.0090
J. Fujimura, Shingo Ishimori, Ichiro Kamioka, S. Okita, Y. Oyazato, A. Nishiyama, M. Yonetani
{"title":"Clinical characteristics of relapsing idiopathic nephrotic syndrome associated with influenza virus infection or influenza virus vaccine in six pediatric patients","authors":"J. Fujimura, Shingo Ishimori, Ichiro Kamioka, S. Okita, Y. Oyazato, A. Nishiyama, M. Yonetani","doi":"10.3165/JJPN.CR.2016.0090","DOIUrl":"https://doi.org/10.3165/JJPN.CR.2016.0090","url":null,"abstract":"","PeriodicalId":325052,"journal":{"name":"Nihon Shoni Jinzobyo Gakkai Zasshi","volume":"71 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121812480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"One Case of Renal Dysfunction with Vesicoureteral Reflux and Oligomeganephronia","authors":"Chieko Matsumura, Tadashi Nishioka, J. Udagawa, H. Kurayama, B. Akikusa, Yuichi Osa, Hiroshi Horie","doi":"10.3165/JJPN.7.60","DOIUrl":"https://doi.org/10.3165/JJPN.7.60","url":null,"abstract":"症例は精神発達遅滞を合併した15歳男児で，7歳時学校検尿で血尿・蛋白尿・軽度高窒素血症を指摘され，以後腎不全が進行した。DIP・腎シンチにて左small kidneyを呈するが，左右共腎瘢痕所見は軽度であった。膀胱造影では右VURI度 (国際分類)，膀胱鏡では両側尿管開口部の馬蹄型変形を認めた。右開放腎生検ではFGS病変と共に著明な糸球体肥大と糸球体populationの低下を認めた。本症例はVURのみで腎不全を説明するにはDIP・腎シンチにおける腎瘢痕所見に乏しく， Oligomeganephronia様の腎低形成を合併していると考えられた。","PeriodicalId":325052,"journal":{"name":"Nihon Shoni Jinzobyo Gakkai Zasshi","volume":"7 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116636465","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The pathogenesis of idiopathic nephrotic syndrome (INS) has not been clearly understood. Most of the pediatric nephrologists currently considered that immune mediated factor derived from T cell is related in some way to INS. Some clinical findings supporting this hypothesis were shown as follows: the response of the disease to corticosteroids and to alkylating agents, the remission which is occurring in association with measles, and occurance of minimal change nephrotic syndrome (MCNS) in patients with Hodgkin’s disease. Reguratory T cell (Treg) is one of T cell component, which function to maintain the balance between self-tolerance and autoimmunity. It is known that Treg is responsible for the pathogenesis of many autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, and myasthenia gravis. Recent reports also suggested the relationship between Treg and INS. We experienced two cases of INS which were thought to be due to reduction of Treg or impaired Treg function. First case was immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome complicated by MCNS. IPEX syndrome is caused by mutations in the FOXP3 gene that result in the defective development of Treg. After bone marrow transplantation, INS in this patients were achieved complete remission. This case imply that reduction of Treg may be cruicial for development of MCNS. Second case was polyglandular autoimmune syndrome (PGA) complicated by INS. The etiology of PGA is currently unknown, however, one of the most leading hypothesis is dysfunction of Treg. This case also imply that impaired Treg may be cruicial for development of INS. Together with previous reports, it is of much interest to consider Treg as one of the pathogenesis of INS. Further studies are needed to define a precise relationship between INS and Treg.
特发性肾病综合征(INS)的发病机制尚不清楚。目前大多数儿科肾病学家认为T细胞衍生的免疫介导因子与INS有一定的关系。支持这一假设的一些临床发现如下:疾病对皮质类固醇和烷基化剂的反应，与麻疹相关的缓解，以及霍奇金病患者的微小变化肾病综合征(MCNS)的发生。调节性T细胞(regulatory T cell, Treg)是T细胞的组成部分之一，具有维持机体自身耐受和自身免疫平衡的功能。众所周知，Treg与许多自身免疫性疾病的发病机制有关，如类风湿关节炎、系统性红斑狼疮和重症肌无力。最近的报告也提出Treg和INS之间的关系。我们经历了两个被认为是由于Treg减少或Treg功能受损的INS病例。第一例为免疫失调、多内分泌病变、肠病、x连锁综合征合并MCNS。IPEX综合征是由FOXP3基因突变导致Treg发育缺陷引起的。骨髓移植后，患者INS完全缓解。该病例提示Treg的减少可能对MCNS的发展至关重要。第二例为多腺体自身免疫综合征(PGA)合并INS。PGA的病因目前尚不清楚，但最主要的假说之一是Treg功能障碍。本病例也暗示Treg受损可能对INS的发展至关重要。结合以往的报道，认为Treg是INS的发病机制之一是很有意义的。需要进一步的研究来确定INS和Treg之间的确切关系。
{"title":"Regulatory T Cell and Nephrotic Syndrome","authors":"T. Ninchoji, H. Kaito, K. Iijima","doi":"10.3165/JJPN.25.137","DOIUrl":"https://doi.org/10.3165/JJPN.25.137","url":null,"abstract":"The pathogenesis of idiopathic nephrotic syndrome (INS) has not been clearly understood. Most of the pediatric nephrologists currently considered that immune mediated factor derived from T cell is related in some way to INS. Some clinical findings supporting this hypothesis were shown as follows: the response of the disease to corticosteroids and to alkylating agents, the remission which is occurring in association with measles, and occurance of minimal change nephrotic syndrome (MCNS) in patients with Hodgkin’s disease. Reguratory T cell (Treg) is one of T cell component, which function to maintain the balance between self-tolerance and autoimmunity. It is known that Treg is responsible for the pathogenesis of many autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, and myasthenia gravis. Recent reports also suggested the relationship between Treg and INS. We experienced two cases of INS which were thought to be due to reduction of Treg or impaired Treg function. First case was immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome complicated by MCNS. IPEX syndrome is caused by mutations in the FOXP3 gene that result in the defective development of Treg. After bone marrow transplantation, INS in this patients were achieved complete remission. This case imply that reduction of Treg may be cruicial for development of MCNS. Second case was polyglandular autoimmune syndrome (PGA) complicated by INS. The etiology of PGA is currently unknown, however, one of the most leading hypothesis is dysfunction of Treg. This case also imply that impaired Treg may be cruicial for development of INS. Together with previous reports, it is of much interest to consider Treg as one of the pathogenesis of INS. Further studies are needed to define a precise relationship between INS and Treg.","PeriodicalId":325052,"journal":{"name":"Nihon Shoni Jinzobyo Gakkai Zasshi","volume":"12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117353064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Expression of the type IV collagen .ALPHA.5 chain in the epidermal basement membrane and the severity of the disease in females with X-linked Alport syndrome.","authors":"K. Nakanishi, N. Kuroda, Y. Inoue, K. Iijima, Hajime Nakamura, Y. Sado, N. Yoshikawa","doi":"10.3165/JJPN.9.140","DOIUrl":"https://doi.org/10.3165/JJPN.9.140","url":null,"abstract":"X連鎖型アルポート症候群はIV型コラーゲンα5鎖遺伝子の異常が原因で，女性患者ではα5鎖は皮膚基底膜・糸球体基底膜にモザイク状に存在する。X連鎖型アルポート症候群女性患者の重症度は様々であり，正常α5鎖遺伝子を有するX染色体の不活化の程度，つまり正常α5鎖の発現程度により決定されると考えられる。そこで，X連鎖型アルポート症候群女性患者の重症度と皮膚基底膜・糸球体基底膜におけるα5鎖発現率の関係を検討した。皮膚基底膜と糸球体基底膜におけるα5鎖発現率には有意の正の相関を認めた。皮膚基底膜における各群のα5鎖発現率は重症群ほど有意に低く，皮膚基底膜のα5鎖発現率は腎炎の重症度をよく反映していた。X連鎖型アルポート症候群女性患者の腎炎重症度は正常α5鎖遺伝子を有するX染色体め不活化の程度，つまり正常α5鎖の発現程度により決定される。皮膚生検による皮膚基底膜のα5鎖発現率の検索は，X連鎖型アルポート症候群女性患者の予後判定に極めて有用である。","PeriodicalId":325052,"journal":{"name":"Nihon Shoni Jinzobyo Gakkai Zasshi","volume":"102 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121129407","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.3165/JJPN.OA.2015.0078
Taichi Hara, S. Fujinaga, A. Yamada, Yasuko Urusihara, Yoshiyuki Ootomo, Toshiaki Shimizu
{"title":"Long-term outcomes in childhood-onset idiopathic nephrotic syndrome: experience in a single center","authors":"Taichi Hara, S. Fujinaga, A. Yamada, Yasuko Urusihara, Yoshiyuki Ootomo, Toshiaki Shimizu","doi":"10.3165/JJPN.OA.2015.0078","DOIUrl":"https://doi.org/10.3165/JJPN.OA.2015.0078","url":null,"abstract":"","PeriodicalId":325052,"journal":{"name":"Nihon Shoni Jinzobyo Gakkai Zasshi","volume":"76 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127078305","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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