title: A clinicopathologic study of primary focal segmental glomerulosclerosis in children.
abstract: There is very little information in the literature on the treatment and prognosis of primary focal segmental glomerulosclerosis (FSGS) among children in Pakistan. This is a review of 94 children (≤16 years) with a diagnosis of primary FSGS who presented to the Sindh Institute of Urology and Transplantation between 1995 and 2008. The clinical records and original renal biopsy reports were reviewed to determine demographic, clinical, laboratory and pathologic features. Renal biopsies were studied by light microscopy, immunofluoroscence and electron microscopy. Thera-peutic regimens and response to therapy were analyzed. Majority of the children (60, 63.8%) had steroid-dependant nephrotic syndrome (SDNS) and 33 (35%) had steroid-resistant nephrotic syndrome (SRNS). Cyclosphosphamide was used in SDNS, and this produced complete remission (CR) in 25/36 (69.4%), partial response (PR) in 4/36 (11%) and no response in 7/36 (19.4%) cases. Cyclosporine was used in SRNS and some SDNS children, and showed a CR in 30 (52.6%), PR in 20 (35%) and no response in seven (12.2%) cases. Tacrolimus was used in seven (7.44%) children. CR was obtained in two (28.5%) and PR in five (71.4%) cases. Renal insufficiency developed in 12 (12.7%) children. Results from this study show that majority of the children with primary FSGS at our center could achieve high rates of sustained remission with second- and third-line immunosuppressive therapies with fairly good prognosis.
authors.affiliation: Department of Pediatric Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. alilanewala@yahoo.com
authors.forename: Ali
authors.initials: A
authors.lastname: Lanewala
authors.forename: Muhammed
authors.initials: M
authors.lastname: Mubarak
authors.forename: Javed Iqbal
authors.initials: JI
authors.lastname: Kazi
authors.forename: Fazal
authors.initials: F
authors.lastname: Akhter
authors.forename: Atika
authors.initials: A
authors.lastname: Sher
authors.forename: Amir
authors.initials: A
authors.lastname: Fayyaz
authors.forename: Sajid
authors.initials: S
authors.lastname: Bhatti
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D005455:Fluorescent Antibody Technique; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents; D007223:Infant; D007678:Kidney Glomerulus / Q000187:drug effects / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D009404:Nephrotic Syndrome / Q000209:etiology / Q000473:pathology; D010154:Pakistan; D012074:Remission Induction; D051437:Renal Insufficiency / Q000209:etiology / Q000473:pathology; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use; D016559:Tacrolimus / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D013256:Steroids; D016572:Cyclosporine; D003520:Cyclophosphamide; D016559:Tacrolimus