Frequently Relapsing Nephrotic Syndrome Research Articles

Rights, Killing, and Suffering: Moral Vegetarianism and Applied Ethics

<h3>Summary</h3> <h3>Background and objectives</h3> Although the safety and efficacy of cyclosporine in children with frequently relapsing nephrotic syndrome (FRNS) have been confirmed, no prospective follow-up data on relapse after cyclosporine have appeared. This study is a prospective follow-up trial after 2-year treatment with cyclosporine to investigate cyclosporine dependency after its discontinuation. <h3>Design, setting, participants, &amp; measurements</h3> Participants who had undergone 2-year protocol treatment with microemulsified cyclosporine for FRNS between January 2000 and December 2005 were followed for an additional 2 years. The primary end point was relapse-free survival after the complete discontinuation of cyclosporine, and the secondary end point was regression-free survival (time to regression to FRNS). <h3>Results</h3> After exclusion of 7 patients who showed regression to FRNS during the 2-year treatment period, 49 children (median age, 6.5 years) were followed, and classified as children without (<i>n</i>=32; group A) and with (<i>n</i>=17; group B) relapse during the initial cyclosporine treatment. Overall, relapse-free survival probability at 24 months after cyclosporine discontinuation was 15.3% and regression to FRNS-free survival probability was 40.8%. By group, the probability of relapse-free survival was significantly higher in group A (17.9%) than in group B (8.3%) (<i>P</i>&lt;0.001). <h3>Conclusions</h3> Children with FRNS who receive cyclosporine are at high risk of relapse after discontinuation, particularly those who experience relapse during cyclosporine treatment.

1067 PATTERN OF RESPONSE TO PREDNISONE AND IMMUNOPATHOLOGIC FINDINGS IN PATIENTS WITH IDIOPATHIC, MINIMAL LESION, NEPHROTIC SYNDROME

Sixty-seven patients with idiopathic, nephrotic syndrome underwent renal biopsy. All had minimal lesion by light microscopy. Immunopathologic studies for complement (C3) and immunoglobulins (IgG, IgM and IgA) were carried out in all the specimens. The patients were divided according to their response to prednisone into steroid resistant (11), frequent relapser (39) and infrequent relapser (17). Immunofluorescent findings on renal biopsy were the following: There was no correlation between the deposition of immunoglobulins and the pattern of response to prednisone. However, complement (C3) was found only in patients with a steroid resistant or frequent relapsing nephrotic syndrome (p < 0.05).

1068 AN ANALYSIS OF RELAPSES AFTER CYCLOPHOSPHAMIDE THERAPY IN NEPHROTIC SYNDROME

Although the dose and duration of therapy with cyclophosphamide (cy) are known to influence the incidence of subsequent relapses in children with nephrotic syndrome, other factors which predispose to relapses after cy have not been extensively evaluated. The clinical course of 30 children with steroid-sensitive, frequently-relapsing nephrotic syndrome was analyzed. Each of the pts had a renal biopsy at the time of initiation of cy, and all of the children had a complete remission while on cy (2 mg/Kg/d for 12 wks). After cy, one or more relapses occurred in 14 pts, while 16 pts had a sustained remission. There were no significant differences between the two groups of pts concerning: 1) age at onset (4.5 vs 4.9 yrs), 2) interval between onset and cy (6.35 vs 6.52 yrs), and 3) duration of follow-up after cy (4.0 vs 4.4 yrs).The most important factor associated with relapses after cy was the histopathologic lesion. Of the 14 pts with relapses, 9 (64%) had focal and segmental glomerulosclerosis (FGS), whereas only 5 (36%) had minimal change lesions (MCL) (P<0.05). In contrast, only 3 (19%) of the 16 pts in remission had FGS, while 13 (81%) had a MCL (P<0.05).These data indicate: 1) the histopathologic lesion at the time of cy is an important determinant of subsequent relapses and 2) late onset FGS predisposes to relapses after cy.

CYCLOPHOSPHAMIDE IN THE TREATMENT OF STEROID-RESISTANT MINIMAL-CHANGE NEPHROTIC SYNDROME

Although cyclophosphamide is effective in the treatment of steroid-sensitive, frequently-relapsing nephrotic syndrome, little data are available on the use of this agent in the treatment of children with steroid-resistant nephrotic syndrome. Six children who had a minimal change lesion on both light and electron microscopy and who had failed to respond to an initial 8-10 week course of prednisone (2 mg/kg/d) were studied. All 6 children had a complete remission during treatment with cyclophosphamide (2 mg/kg/d for 12 weeks) and prednisone (0.5-1.0 mg/kg/d for the first 6 weeks). Three of these children have subsequently relapsed 6-18 months after treatment with cyclophosphamide, and, because of the potential gonadal toxicity of this drug, treatment was reinstituted with prednisone alone. All 3 patients sustained a complete remission within 2 weeks after restarting only steroid therapy.These observations suggest: a) cyclophosphamide may be effective in the treatment of steroid-resistant minimal-change nephrotic syndrome; b) patients who are initially steroid resistant prior to treatment with cyclophosphamide may become steroid sensitive following such therapy; and, therefore, c) initial treatment of relapses which occur after a course of cyclophosphamide should be glucosteroids alone.