title: A combined low-density lipoprotein apheresis and prednisone therapy for steroid-resistant primary focal segmental glomerulosclerosis in children.
abstract: BACKGROUND
  Treatment of steroid-resistant (SR) primary focal segmental glomerulosclerosis (FSGS) remains a major challenge in nephrology. A prospective study was conducted to clarify the therapeutic role of low-density lipoprotein apheresis (LDL-A) in 11 nephrotic children with SR and cyclosporine A (CsA)-resistant primary FSGS.
  
  
  METHODS
  Based on entry criteria, all 11 eligible patients had biopsy-proven primary FSGS presenting with nephrotic syndrome (NS) and were resistant to steroid and conventional-dose CsA therapy. LDL-A was performed twice a week for 3 weeks (first course), then weekly for 6 weeks (second course). Beginning from the second LDL-A course, a dosage of 1 mg/kg/d of prednisone was administered for 6 weeks, then tapered.
  
  
  RESULTS
  Seven patients experienced remission of NS, 5 of whom achieved complete remission within 4 weeks after initiating prednisone therapy with LDL-A. These 5 patients maintained normal renal function during follow-up (median, 4.4 years). Of 2 patients with partial remission, 1 patient maintained stable renal function during follow-up (4.5 years), whereas the other patient showed a gradual decline in renal function and progressed to end-stage renal failure (ESRF) 7.8 years after LDL-A therapy. Four patients who were considered to experience treatment failure had persistent NS and progressed to ESRF in 1.3 years (median) after LDL-A therapy. Complete remission (n = 5) was associated with significantly more highly selective proteinuria compared with treatment failure (n = 4).
  
  
  CONCLUSIONS
  This study suggests that combined LDL-A and prednisone therapy can be a valuable addition to therapeutic options for treating patients with SR-FSGS. The role of LDL-A in treating these patients deserves to be assessed further in larger randomized controlled trials.
authors.affiliation: Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine, Tokyo, Japan. hattori@kc.twmu.ac.jp
authors.forename: Motoshi
authors.initials: M
authors.lastname: Hattori
authors.forename: Hiroko
authors.initials: H
authors.lastname: Chikamoto
authors.forename: Yuko
authors.initials: Y
authors.lastname: Akioka
authors.forename: Hyogo
authors.initials: H
authors.lastname: Nakakura
authors.forename: Daisuke
authors.initials: D
authors.lastname: Ogino
authors.forename: Akira
authors.initials: A
authors.lastname: Matsunaga
authors.forename: Akira
authors.initials: A
authors.lastname: Fukazawa
authors.forename: Sanpei
authors.initials: S
authors.lastname: Miyakawa
authors.forename: Miyuki
authors.initials: M
authors.lastname: Khono
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kawaguchi
authors.forename: Katsumi
authors.initials: K
authors.lastname: Ito
mesh_terms: D000293:Adolescent; D001781:Blood Component Removal; D002482:Cellulose; D002648:Child; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000494:pharmacology / Q000627:therapeutic use; D016264:Dextran Sulfate; D004351:Drug Resistance; D005260:Female; D005500:Follow-Up Studies; D005923:Glomerulosclerosis, Focal Segmental / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D006801:Humans; D006949:Hyperlipidemias / Q000188:drug therapy / Q000209:etiology / Q000628:therapy; D008077:Lipoproteins, LDL / Q000097:blood*; D008297:Male; D009404:Nephrotic Syndrome / Q000097:blood / Q000188:drug therapy / Q000209:etiology / Q000628:therapy; D011241:Prednisone / Q000627:therapeutic use*; D011446:Prospective Studies; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology / Q000628:therapy; D012074:Remission Induction; D016896:Treatment Outcome
chemical_list: D008077:Lipoproteins, LDL; D016572:Cyclosporine; D002482:Cellulose; D016264:Dextran Sulfate; D011241:Prednisone