Primary focal segmental glomerular sclerosis in children: clinical course and prognosis

• DOI: 10.1007/s00467-006-0301-5
• Publication Date: 2006-10-20T11:59:06Z
• Authors: Kyung Hoon Paik; Bum Hee Lee; Hee Yeon Cho; Hee Gyung Kang; Il Soo Ha; Hae Il Cheong; Dong-Kyu Jin; Kyung Chul Moon; Yong Choi
• Keywords: Male; Nephrotic Syndrome; Adolescent; Glomerulosclerosis, Focal Segmental; Prednisolone; Remission Induction; Drug Resistance; Infant, Newborn; Infant; Prognosis; 3. Good health; Proteinuria; 03 medical and health sciences; 0302 clinical medicine; Risk Factors; Child, Preschool; Disease Progression; Humans; Kidney Failure, Chronic; Female; Child; Glucocorticoids

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.