title: Primary focal segmental glomerular sclerosis in children: clinical course and prognosis.
abstract: To review the clinical course and identify prognostic factors, we retrospectively analyzed 92 children with steroid-resistant primary focal segmental glomerulosclerosis (FSGS). The mean age of onset was 80.4+/-42.4 months. The mean follow-up duration was 98.2+/-63.3 months. Eighty-five patients presented with nephrotic syndrome and seven presented with asymptomatic proteinuria. Thirty-three patients were initial responders to steroid treatment (late non-responders) and 59 were initial nonresponders. At last follow-up, 36 patients (39.1%) were in complete remission, and 29 (31.5%) progressed to chronic renal failure (CRF). Renal survival rates at 5, 10, and 15 years were 84, 64, and 53%, respectively. By morphological classification, there were tip variants (6.1%), collapsing variants (10.6%), cellular variants (1.5%), perihilar variants (9.1%), and NOS (not otherwise specified, 72.7%). Among the variants, there were no significant differences in age of onset, degree of proteinuria, response to treatment, or progression to CRF. Poor prognostic factors for CRF included: asymptomatic proteinuria at presentation, initial renal insufficiency, higher segmental sclerosis (%), severe tubulointerstitial change, initial nonresponse, and absence of remission. In the multivariate analysis, an increase in the initial serum creatinine and resistance to treatment were independent risk factors for CRF. A more prolonged use of corticosteroid therapy and early introduction of cyclosporin A (CsA) may improve the prognosis for primary FSGS in patients with initial steroid nonresponsiveness.
authors.affiliation: Department of Pediatrics, Seoul National University Children's Hospital, South Korea.
authors.forename: Kyung Hoon
authors.initials: KH
authors.lastname: Paik
authors.forename: Bum Hee
authors.initials: BH
authors.lastname: Lee
authors.forename: Hee Yeon
authors.initials: HY
authors.lastname: Cho
authors.forename: Hee Gyung
authors.initials: HG
authors.lastname: Kang
authors.forename: Il Soo
authors.initials: IS
authors.lastname: Ha
authors.forename: Hae Il
authors.initials: HI
authors.lastname: Cheong
authors.forename: Dong-Kyu
authors.initials: DK
authors.lastname: Jin
authors.forename: Kyung Chul
authors.initials: KC
authors.lastname: Moon
authors.forename: Yong
authors.initials: Y
authors.lastname: Choi
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004351:Drug Resistance; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D011507:Proteinuria / Q000209:etiology; D012074:Remission Induction; D012307:Risk Factors
chemical_list: D005938:Glucocorticoids; D011239:Prednisolone