Nihon Shoni Jinzobyo Gakkai Zasshi最新文献_第10页

The treatment of chronic renal failure in children. 儿童慢性肾衰竭的治疗。

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1991-04-01 DOI: 10.3165/JJPN.4.8

B. J.W.

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The hemolytic uremic syndromes: Pathogenesis. 溶血性尿毒症综合征:发病机制。

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1990-04-01 DOI: 10.3165/JJPN.3.1

B. Kaplan

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A case of purpura nephritis complicated by nephrotic syndrome and showing membranoproliferative glomerulonephritis-like findings for which frequent steroid pulse therapy was effective 紫癜性肾炎并发肾病综合征1例，表现为膜增生性肾小球肾炎样表现，频繁类固醇脉冲治疗有效

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1900-01-01 DOI: 10.3165/JJPN.CR.2017.0118

Tetsuro Matsuhashi, N. Kumagai, M. Nakayama, H. Shima, Ikumi Umeki, Junko Kanno, S. Kure

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Evaluation of cytochrome c in hemolytic uremic syndrome 溶血性尿毒症患者细胞色素c的测定

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1900-01-01 DOI: 10.3165/JJPN.28.68

Etsuko Tanaka, T. Konomoto, Hideaki Imamura, M. Orita, H. Sakaguchi, Akihiko Nakahara, H. Nunoi

引用次数: 1

Clinical characteristics of relapsing idiopathic nephrotic syndrome associated with influenza virus infection or influenza virus vaccine in six pediatric patients 6例小儿与流感病毒感染或流感病毒疫苗相关的复发性特发性肾病综合征的临床特征

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1900-01-01 DOI: 10.3165/JJPN.CR.2016.0090

J. Fujimura, Shingo Ishimori, Ichiro Kamioka, S. Okita, Y. Oyazato, A. Nishiyama, M. Yonetani

引用次数: 1

Rituximab treatment for steroid-resistant nephrotic syndrome 利妥昔单抗治疗类固醇抵抗性肾病综合征

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1900-01-01 DOI: 10.3165/JJPN.24.198

Koji Sakuraya, D. Hirano, S. Fujinaga, A. Endo, Tsuneki Watanabe, T. Someya, Y. Ohtomo, Toshiaki Shimizu

{"title":"Rituximab treatment for steroid-resistant nephrotic syndrome","authors":"Koji Sakuraya, D. Hirano, S. Fujinaga, A. Endo, Tsuneki Watanabe, T. Someya, Y. Ohtomo, Toshiaki Shimizu","doi":"10.3165/JJPN.24.198","DOIUrl":"https://doi.org/10.3165/JJPN.24.198","url":null,"abstract":"特発性ネフローゼ症候群(idiopathic nephrotic syndrome:以下,INS)は,小児10万人に5人が発症すると推定される小児科領域では重要な疾患である。小児期 INSは経口ステロイド剤への反応性が良好で,約90%が反応し,いったん蛋白尿が消失する。しかし,残りの 10%はステロイド剤に反応せず,ステロイド抵抗性ネフローゼ症候群(steroid-resistant NS:以下,SRNS)と呼ばれている。SRNSは,初発時よりステロイド療法に抵抗を示す initial non-responderと,初発時はステロイド感受性だが頻回再発やステロイド依存性から再発時にステロイド療法に抵抗を示す late non-responderに分類される。組織型としては,主に微小変化群,巣状分節性糸球体硬化症,びまん性メサンギウム増殖の3つがある。 SRNSは治療に反応しなければ末期腎不全に至る疾患であり,先天性の腎疾患に次いで小児末期腎不全に陥る頻度の高い疾患である。2009年に Hamasakiらが報告した SRNSに対する MPTおよびシクロスポリン(cyclosporin A)の併用療法は,その有効率が80%以上と非常に好成績であった。しかし,それらの治療をもってしても高度蛋白尿が遷延し,末期腎不全に陥る,いわゆる難治性 SRNSが10%以上存在するため,新たな治療法の開発が望まれている。そのような症例に対する次なる治療として,近年リツキシマブが世界的に注目されている。リツキシマブは B細胞表面に発現する分化抗原 CD20 難治性ステロイド抵抗性ネフローゼ症候群に対するリツキシマブ療法","PeriodicalId":325052,"journal":{"name":"Nihon Shoni Jinzobyo Gakkai Zasshi","volume":"45 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":"115747118","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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One Case of Renal Dysfunction with Vesicoureteral Reflux and Oligomeganephronia 肾功能障碍伴膀胱输尿管反流及肾上腺素过少1例

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1900-01-01 DOI: 10.3165/JJPN.7.60

Chieko Matsumura, Tadashi Nishioka, J. Udagawa, H. Kurayama, B. Akikusa, Yuichi Osa, Hiroshi Horie

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Regulatory T Cell and Nephrotic Syndrome 调节性T细胞与肾病综合征

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1900-01-01 DOI: 10.3165/JJPN.25.137

T. Ninchoji, H. Kaito, K. Iijima

{"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}

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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. IV型胶原蛋白α的表达。x -连锁Alport综合征女性表皮基底膜5链与疾病严重程度的关系

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1900-01-01 DOI: 10.3165/JJPN.9.140

K. Nakanishi, N. Kuroda, Y. Inoue, K. Iijima, Hajime Nakamura, Y. Sado, N. Yoshikawa

{"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}

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Long-term outcomes in childhood-onset idiopathic nephrotic syndrome: experience in a single center 儿童期发病特发性肾病综合征的长期预后:单一中心的经验

Nihon Shoni Jinzobyo Gakkai Zasshi Pub Date : 1900-01-01 DOI: 10.3165/JJPN.OA.2015.0078

Taichi Hara, S. Fujinaga, A. Yamada, Yasuko Urusihara, Yoshiyuki Ootomo, Toshiaki Shimizu

引用次数: 0