出典: meddic


focal segmental glomerulosclerosis
focal segmental glomerulosclerosis
focal and segmental glomerulosclerosis

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  • Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis.
  • Kumar J, Shatat IF, Skversky AL, Woroniecki RP, Del Rio M, Perelstein EM, Johnson VL, Mahesh S.SourceWeill Cornell Medical College, Helmsley Towers 3, Box 176, New York, NY, 10021, USA, juk2013@med.cornell.edu.
  • Pediatric nephrology (Berlin, Germany).Pediatr Nephrol.2013 Feb;28(2):333-8. doi: 10.1007/s00467-012-2314-6. Epub 2012 Oct 4.
  • BACKGROUND: Focal segmental glomerulosclerosis (FSGS) recurs in 20-40 % of allografts. Plasmapheresis (TPE) has been one of the mainstays of treatment with variable results. Rituximab (RTX), a monoclonal antibody to the protein CD20, is being used for treatment of recurrent FSGS (recFSGS) but pedia
  • PMID 23052653
  • The changing pattern of glomerular disease in HIV and Hepatitis C co-infected patients in the era of HAART.
  • Mohan S, Herlitz LC, Tan J, Cheng JT, Anderson HL, Stokes MB, Markowitz GS, D'Agati VD, Radhakrishnan J.AbstractPrevious reports have suggested a poor renal prognosis in patients with HIV and HCV co-infection with a preponderance of immune complex mediated glomerular disease on biopsy. Although the benefits of HAART on HIVAN are known, its impact on co-infected patients is unclear. We describe the renal biopsy findings and renal outcome in 29 co-infected patients in the HAART era and compare them to findings in 14 historical controls reported from our institution in the pre-HAART era. Our present cohort was predominantly male and Black with the majority reporting a history of intravenous (i.v.) drug use. Renal biopsy findings included 16 patients with immune complex mediated glomerular disease and 14 patients with FSGS, of which only 3 had collapsing features and/or tubular microcysts typical of HIVAN. Five patients had other biopsy diagnoses not directly related to viral infection. Median renal survival in our cohort was 15.6 months - significantly better than the 1.7 months seen our pre-HAART cohort. The modern cohort's improved renal outcome occurred despite older patients, longer HIV infection and similar levels of renal insufficiency. Our data indicate a changing epidemiology and natural history of renal disease in the HAART era with less immune complex mediated glomerular disease and more non-collapsing FSGS of the usual type. The marked improvement is likely to be multifactorial, including use of antiretroviral and anti-HCV therapies, RAAS antagonists, earlier nephrology referral and generally improved medical care.
  • Clinical nephrology.Clin Nephrol.2013 Jan 15. [Epub ahead of print]
  • Previous reports have suggested a poor renal prognosis in patients with HIV and HCV co-infection with a preponderance of immune complex mediated glomerular disease on biopsy. Although the benefits of HAART on HIVAN are known, its impact on co-infected patients is unclear. We describe the renal biops
  • PMID 23320974
  • Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis.
  • Hamasaki Y, Yoshikawa N, Nakazato H, Sasaki S, Iijima K, Nakanishi K, Matsuyama T, Ishikura K, Ito S, Kaneko T, Honda M; for Japanese Study Group of Renal Disease in Children.SourceDepartment of Nephrology, Tokyo Metropolitan Children's Medical Center, Fuchu, Japan, yuhamasaki@med.toho-u.ac.jp.
  • Pediatric nephrology (Berlin, Germany).Pediatr Nephrol.2013 Jan 13. [Epub ahead of print]
  • BACKGROUND: Cyclosporine has improved remission rates in children with steroid-resistant nephrotic syndrome (SRNS). However, little prospective long-term follow-up data is available.METHODS: We prospectively followed and analyzed 5-year outcomes of all 35 patients enrolled in our previous prospectiv
  • PMID 23314441


  • 腎移植領域におけるアフェレシス(<特集>腎疾患とアフェレシス-最近の知見-)
  • 原田 浩,堀田 記世彦,三浦 正義
  • 日本アフェレシス学会雑誌 30(2), 116-123, 2011-05-31
  • … Herein, we discuss the role and the practical management of TA in successfully reducing preexisting antibodies and ill-defined but possible permeability factors, which are believed to be important in the development of FSGS. …
  • NAID 110008661251
  • 小児腎疾患におけるアフェレシス(<特集>腎疾患とアフェレシス-最近の知見-)
  • 服部 元史
  • 日本アフェレシス学会雑誌 30(2), 90-96, 2011-05-31
  • … In this article, the efficacy of plasmapheresis and possible mechanisms of action of plasmapheresis in renal diseases, especially focusing on 1) a rapidly progressive type of Henoch-Schonlein purpura nephritis (HSPN), 2) steroid-resistant primary focal segmental glomerulosclerosis (FSGS) and 3) post-transplant recurrence of FSGS are reviewed. …
  • NAID 110008661247


... ・ご家族の皆さま > 私たちが携わる疾患 > 巣状糸球体硬化症 (FGS) (focal glomerular sclerosis)/ (FSGS) (focal segmental glomerular sclerosis) 血液と治療の話 血液のしくみ 血液の機能 血液から病因物質を除去する治療法 私たち ...
FSGS Facts More than 5400 patients are diagnosed with FSGS every year, however, this is considered an underestimate because: a limited number of biopsies are performed the number of FSGS cases are rising more than any other ...



リンク元巣状分節性糸球体硬化症」「focal segmental glomerulosclerosis



focal segmental glomerulosclerosis, FSGS, focal and segmental glomerulosclerosis
巣状硬化性糸球体腎炎 focal sclerosing glomerulonephritis
巣状糸球体硬化症 focal glomerulosclerosis, FGS


  • 本症は糸球体が巣状かつ分節状に硬化した疾患。
  • ネフローゼの20-30%を占める



  • 血尿、高血圧
  • 蛋白尿はnonselective


  • 1. 一次性
  • 2. 二次性
  • 1. HIV感染、ヘロイン
  • 2. 他の糸球体腎炎に続発
  • 3. ネフロン喪失に続発するmaladaptation
  • 4. 足細胞に発現する細胞骨格に関連する蛋白質(ネフリン)


  • 光学顕微鏡
  • 巣状かつ分節性の硬化病変
  • メサンギウム基質、毛細管腔の閉塞、ヒアリン・脂質滴の沈着
  • 蛍光抗体法…IgMと補体成分(特にC3)の沈着が認められる
  • 電子顕微鏡…上皮細胞に足突起の融合が見られ、ところどころで上皮細胞そのものが脱落し、基底膜が丸出しになっている所見も認められる


  • 予後不良
  • ステロイドが効かない
  • 腎移植後の再発が多い

focal segmental glomerulosclerosis」

  [★] 巣状分節性糸球体硬化症 FSGS



"http://meddic.jp/FSGS" より作成