title: Primary membranous nephropathy in adolescence: A prospective study.
abstract: OBJECTIVE
  Primary membranous nephropathy (PMN) accounts for only 1-2% of nephrotic syndrome in children. Antibodies to m-type phospholipase A2 receptor (aPLA2R) is seen in 70% of adult PMN cases. The present study was undertaken to study m-type phospholipase A2receptor (PLA2R) status and clinical behavior in adolescent PMN cases.
  
  
  METHODS
  The present prospective observational study included adolescent (10-19 years) onset biopsy proved PMN. Patients were followed on a monthly basis with urine protein, serum albumin and creatinine. Serum aPLA2R was done at baseline and at 6 and 12 months of starting treatment. Patients were treated as per unit's protocol.
  
  
  RESULTS
  During the study period a total of 18 patients were enrolled. The mean age of the cases was 16.27 ± 2.39 (11-19) years. Seventeen (94.44%) patients presented with nephrotic syndrome. The mean proteinuria and serum albumin was 4.52 ± 1.93 (2.43-9.20) g/day and 2.1 ± 0.6 (1.1-3.4) g/dL respectively. PMN was PLA2R related in 83%. aPLA2R and enhanced staining for PLA2R in glomeruli was seen in 14 (77.78%) and 13 (72.22%), respectively. Clinical remission at the end of 6 and 12 months of therapy was seen in 11 (61.11%) and 9 (50%) subjects respectively. There was a significant association of aPLA2R to clinical remission/ resistance.
  
  
  CONCLUSIONS
  Primary membranous nephropathy in adolescent population is aPLA2R related in over three-quarters of the cases and the response to therapy is seen in only half of them. aPLA2R monitoring is clinically relevant and should be incorporated in the management of adolescent onset PMN.
authors.affiliation: Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Vinod
authors.initials: V
authors.lastname: Kumar
authors.affiliation: Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Ashwani Kumar
authors.initials: AK
authors.lastname: Varma
authors.affiliation: Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Ritambhra
authors.initials: R
authors.lastname: Nada
authors.affiliation: Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Ratan
authors.initials: R
authors.lastname: Ghosh
authors.affiliation: Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Deepti
authors.initials: D
authors.lastname: Suri
authors.affiliation: Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Anju
authors.initials: A
authors.lastname: Gupta
authors.affiliation: Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Vivek
authors.initials: V
authors.lastname: Kumar
authors.affiliation: Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Manish
authors.initials: M
authors.lastname: Rathi
authors.affiliation: Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Harbir
authors.initials: H
authors.lastname: Kohli
authors.affiliation: Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Vivekanand
authors.initials: V
authors.lastname: Jha
authors.affiliation: Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Krishan
authors.initials: K
authors.lastname: Gupta
authors.affiliation: Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Raja
authors.initials: R
authors.lastname: Ramachandran
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001323:Autoantibodies / Q000097:blood; D001706:Biopsy; D002648:Child; D003404:Creatinine / Q000097:blood; D005260:Female; D015433:Glomerulonephritis, Membranous / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007678:Kidney Glomerulus / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000097:blood / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D011446:Prospective Studies; D011507:Proteinuria / Q000097:blood / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D054507:Receptors, Phospholipase A2 / Q000276:immunology; D012074:Remission Induction; D000075462:Serum Albumin, Human / Q000378:metabolism; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: C422455:ALB protein, human; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D054507:Receptors, Phospholipase A2; D003404:Creatinine; D000075462:Serum Albumin, Human
keywords: PLA2R; adolescent; childhood; membranous nephropathy; pediatric; tacrolimus