Impact of recurrent nephrotic syndrome after renal transplantation in young patients.
Recurrent disease is a frequent complication of patients transplanted for steroid-resistant nephrotic syndrome associated with focal segmental glomerulosclerosis. Its long-term prognosis has rarely been studied. We examined 39 patients aged 4-25 (mean 13.5) years at the time of first transplantation (TX). Twelve of these (30%) developed nephrotic syndrome after the first TX and 2 of 8 after the second TX. The mean observation period from first TX to last observation with a functioning graft or graft loss was 5.4 (0.1-19.3) years. We confirmed that recurrent disease is associated with older age at onset of the primary disease, shorter time from onset to end-stage renal disease, and diffuse mesangial proliferation in the initial kidney biopsy. Remissions occurred in all 3 children undergoing early repeated plasma exchange and in 1 adolescent following introduction of cyclosporin A 7 years after TX. At last observation 42% of relapsing and 48% of non-relapsing patients with a similar follow-up period had a functioning first graft. Median first graft survival was almost identical in the relapsing and the non-relapsing patients (4.3 vs. 4.2 years). Histological lesions of focal glomerulosclerosis were detected in the posttransplant biopsies of only 3 patients. In conclusion, young patients with nephrotic syndrome associated with focal segmental sclerosis have a similar graft survival with and without recurrence of the nephrotic syndrome.
Division of Pediatric Nephrology, University of Heidelberg, Germany.
E
E
Wühl
J
J
Fydryk
M
M
Wiesel
O
O
Mehls
F
F
Schaefer
K
K
Schärer
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use; D004351:Drug Resistance; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications*; D006085:Graft Survival; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000473:pathology; D016030:Kidney Transplantation / Q000473:pathology*; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology / Q000601:surgery; D010951:Plasma Exchange; D011379:Prognosis; D012008:Recurrence; D013256:Steroids / Q000627:therapeutic use
D007166:Immunosuppressive Agents; D013256:Steroids; D016572:Cyclosporine