Primary focal segmental glomerular sclerosis in children: clinical course and prognosis.
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.
Paik
Kyung Hoon
KH
Department of Pediatrics, Seoul National University Children's Hospital, South Korea.
Lee
Bum Hee
BH
Cho
Hee Yeon
HY
Kang
Hee Gyung
HG
Ha
Il Soo
IS
Cheong
Hae Il
HI
Jin
Dong-Kyu
DK
Moon
Kyung Chul
KC
Choi
Yong
Y
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
D005938:Glucocorticoids; D011239:Prednisolone