[Show abstract][Hide abstract] ABSTRACT: Cyclosporine A (CsA) has relieved children with steroid-dependent nephrotic syndrome (NS) from steroid toxicity. However, most patients frequently relapse again when CsA is withdrawn, resulting in the development of CsA nephropathy for its long-term use. In order to assess the efficacy of mycophenolate mofetil (MMF) therapy, we prospectively analyzed 12 children with idiopathic steroid-dependent NS requiring long-term CsA therapy with MMF for at least 6 months. Mean follow-up after starting MMF was 11 months (range 6-42). The mean MMF dose required was 610+/-95 mg/m(2)/12 h, which maintained mean predose mycophenolic acid (C0-MPA) levels of 2.4+/-1.1 mcg/ml. Treatment with MMF resulted in CsA and/or prednisolone (PSL) sparing, with a reduction in mean CsA dose from 3.5+/-1.3 to 1.5+/-2.4 mg/kg/day (p<0.01), and mean PSL dose from 0.29+/-0.16 to 0.21+/-0.11 mg/kg/day (p<0.05). Nine of 12 patients (75%) were finally able to be weaned off CsA. Mean relapse rates decreased from 2.7+/-1.6 to 0.6+/-0.9 episodes/year (p<0.01). Relapse-free ratio on MMF therapy was lower in patients whose average C0-MPA levels were less than 2 mcg/ml (p<0.05). Our experience demonstrates that MMF therapy results in significant CsA and/or steroid sparing and reduction in relapse rates in children with CsA-dependent NS.
No preview · Article · Feb 2007 · Pediatric Nephrology
[Show abstract][Hide abstract] ABSTRACT: Thin basement membrane nephropathy (TBMN) is characterized clinically by persistent hematuria, minimal proteinuria, normal renal function, another family member with hematuria, and a benign course. Especially in childhood TBMN, proteinuria of any degree is reported to be uncommon. We report on a boy with benign familial hematuria found by urinary screening at 3 years of age who presented with nephrotic syndrome (NS) at 15 years of age. His renal histology showed TBMN associated with minimal change disease (MCD). Treatment with corticosteroid resulted in complete remission of NS in a short period of time, while isolated hematuria persisted during the follow-up period despite this therapy. We speculate, therefore, that the nephrotic range proteinuria is not due to TBMN but rather is the manifestation of associated MCD. Several cases of TBMN with NS have been reported in adults, but it has not yet been reported in children in the literature. To our knowledge, this is the first case of childhood TBMN associated with NS resulting from coincidental MCD.
No preview · Article · Mar 2006 · Pediatric Nephrology