title: Membranoproliferative glomerulonephritis and C3 glomerulonephritis: frequency, clinical features, and outcome in children.
abstract: OBJECTIVE
  C3 glomerulonephritis (C3GN) is a recently described disease that is related to membranoproliferative glomerulonephritis (MPGN). We retrospectively compared the frequencies, clinical characteristics, treatment modalities, and outcomes of C3GN and MPGN in a cohort of Japanese children.
  
  
  METHODS
  Children who were pathologically diagnosed with MPGN (type I or III) in our hospital were divided into two groups based on immunofluorescence imaging of renal biopsies: children with MPGN induced by classical complement pathway activation (classical MPGN) and children with C3GN.
  
  
  RESULTS
  Of 14 children with MPGN (five boys), four had classical MPGN, eight had C3GN, and two had unclassifiable glomerulonephritis. Four children with classical MPGN and seven with C3GN received methylprednisolone pulse therapy followed by oral prednisolone for 2 years (MPT+PSL therapy). Subsequently, six of seven children with C3GN received combined therapy (prednisolone, azathioprine, and anticoagulants) for 2 years because they responded poorly to MPT+PSL therapy. At the last follow-up visit, two children with classical MPGN and seven with C3GN had not achieved remission. One child with classical MPGN and five with C3GN had hypocomplementaemia at the last follow-up. None of the children had renal impairment.
  
  
  CONCLUSIONS
  More than half of the patients previously diagnosed with MPGN fulfilled the criteria for C3GN in children. C3GN may be more refractory than classical MPGN to immunosuppressant therapy.
authors.affiliation: Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Okuda
authors.forename: Kenji
authors.initials: K
authors.lastname: Ishikura
authors.forename: Riku
authors.initials: R
authors.lastname: Hamada
authors.forename: Ryoko
authors.initials: R
authors.lastname: Harada
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Sakai
authors.forename: Yuko
authors.initials: Y
authors.lastname: Hamasaki
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hataya
authors.forename: Ryuji
authors.initials: R
authors.lastname: Fukuzawa
authors.forename: Kentaro
authors.initials: K
authors.lastname: Ogata
authors.forename: Masataka
authors.initials: M
authors.lastname: Honda
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000925:Anticoagulants / Q000627:therapeutic use; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003176:Complement C3 / Q000032:analysis*; D003170:Complement Pathway, Alternative; D003171:Complement Pathway, Classical; D004359:Drug Therapy, Combination; D005260:Female; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D015432:Glomerulonephritis, Membranoproliferative / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan; D007678:Kidney Glomerulus / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D008297:Male; D011237:Predictive Value of Tests; D012074:Remission Induction; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D015415:Biomarkers; C038245:C3 protein, human; D003176:Complement C3; D007166:Immunosuppressive Agents; D013256:Steroids
keywords: C3 glomerulopathy; immunofluorescence; immunosuppressant; membranoproliferative glomerulonephritis; methylprednisolone pulse therapy