title: Clinicopathologic characteristics and steroid response of IgM nephropathy in children presenting with idiopathic nephrotic syndrome.
abstract: There is no detailed information on clinical and immunopathologic features of immunoglobulin M nephropathy (IgMN) in children with idiopathic nephrotic syndrome (INS) in Pakistan. We reviewed our native renal biopsies over 15 years (July 1995-July 2010) and identified 135 cases of IgMN in nephrotic children (≤17 years). Their demographic, clinical and immunopathologic data were retrieved from biopsy reports and case notes. Mean age of this cohort was 7.6 ± 4.2 years. Males were 92 (68.1%) and females were 43 (31.9%). Steroid-dependent NS was seen in 88 (65.2%) cases and steroid-resistant NS in 47 (34.2%). Hematuria was found in 42 cases (31.2%) and hypertension in 27 (19.5%). The most common morphologic change was glomerular mesangial proliferation, found in 89 (65.9%) biopsies. Minor changes were seen in 46 (34.1%) cases and focal segmental glomerulosclerosis (FSGS) in 37 (27.4%). Immunofluorescence microscopy showed diffuse mesangial positivity of IgM in all cases. C3 and C1q were found in 72 (53.3%) and 40 (29.7%) cases, respectively. Our results show that IgMN is a fairly common cause of INS in children in Pakistan. It shows a spectrum of morphologic changes ranging from minor changes to FSGS.
authors.affiliation: Histopathology and Nephrology Departments, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. drmubaraksiut@yahoo.com
authors.forename: Muhammed
authors.initials: M
authors.lastname: Mubarak
authors.forename: Javed I
authors.initials: JI
authors.lastname: Kazi
authors.forename: Shaheera
authors.initials: S
authors.lastname: Shakeel
authors.forename: Ali
authors.initials: A
authors.lastname: Lanewala
authors.forename: Seema
authors.initials: S
authors.lastname: Hashmi
authors.forename: Fazal
authors.initials: F
authors.lastname: Akhter
mesh_terms: D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism*; D007668:Kidney / Q000276:immunology / Q000473:pathology; D008297:Male; D008856:Microscopy, Fluorescence; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D010154:Pakistan; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use
chemical_list: D007075:Immunoglobulin M; D013256:Steroids