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2015 ; 3
(ä): 104
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Long-Term Outcomes in Children with Steroid-Resistant Nephrotic Syndrome Treated
with Calcineurin Inhibitors
#MMPMID26640779
Beins NT
; Dell KM
Front Pediatr
2015[]; 3
(ä): 104
PMID26640779
show ga
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is an important cause of
chronic kidney disease (CKD) in children that often progresses to end-stage renal
disease (ESRD). Calcineurin inhibitors (CNIs) have been shown to be effective in
inducing short-term remission in some patients with SRNS. However, there are
little data examining their long-term impact on ESRD progression rates. METHODS:
We performed a retrospective chart review of all patients treated for SRNS with
CNIs at our institution from 1995 to 2013. Data collected including demographics,
initial response to medical therapy, number of relapses, progression to ESRD, and
treatment complications. RESULTS: A total of 16 patients met inclusion criteria
with a mean follow-up of 6.6?years (range 0.6-17.6?years). Histopathological
diagnoses were focal segmental glomerulosclerosis (8), mesangial proliferative
glomerulonephritis (4), IgM nephropathy (3), and minimal change disease (1).
Three patients (18.8%) were unresponsive to CNIs while the remaining 13 (81.2%)
achieved remission with CNI therapy. Six patients (37.5%) progressed to ESRD
during the study period, three of whom did so after initially responding to CNI
therapy. Renal survival rates were 87, 71, and 57% at 2, 5, and 10?years,
respectively. Non-Caucasian ethnicity was associated with progression to ESRD.
Finally, a higher number of acute kidney injury (AKI) episodes were associated
with a lower final estimated glomerular filtration rate. DISCUSSION: Despite the
majority of SRNS patients initially responding to CNI therapy, a significant
percentage still progressed to ESRD despite achieving short-term remission.
Recurrent episodes of AKI may be associated with progression of CKD in patients
with SRNS.