Success with plasmapheresis treatment for recurrent focal segmental glomerulosclerosis in pediatric renal transplant recipients.

FSGS recurs in approximately 30% of transplanted kidneys and may lead to graft loss. We retrospectively examined the efficacy of early and intensive PP without additional IS in pediatric kidney transplant patients with recurrent FSGS at our center. Seven of 24 patients (29%) had nephrotic proteinuria and histologic evidence of FSGS recurrence within 1-5 days post-transplantation. PP was initiated early after transplantation and initially performed daily until sustained decline in proteinuria. PP frequency was then individually tapered according to proteinuria. Recurrent FSGS in all seven patients responded to a four- to 32-wk course of PP. Two of seven patients had a second recurrence of FSGS, and both recurrences remitted after an additional 3-6 wk of PP. Median observation period was 4.5 yr (0.8-16.3 yr). Complete remission of recurrent FSGS has been sustained in all seven patients, and all patients have stable graft function with recent plasma creatinine <1.5 mg/dL in six of seven patients. Most recent urine protein/creatinine is 0.13-0.61 mg/mg in six of seven patients. One patient has heavy proteinuria secondary to chronic allograft nephropathy 16 yr post-transplant. Intensive and prolonged PP, when initiated early in the post-operative period, is effective in treating recurrent FSGS and preventing graft loss without the use of additional immunosuppressants.

T cell CD3 receptor zeta (TCRzeta)-chain expression in children with idiopathic nephrotic syndrome.

Children with idiopathic nephrotic syndrome (INS) have an increased risk of developing life-threatening infections. Several studies have demonstrated functional abnormalities in the T lymphocytes of patients with nephrotic syndrome. Although T cells are activated in INS during relapse, as indicated by an increased expression of interleukin (IL)-2 receptor, these cells have a decreased ability to proliferate. The T-cell receptor (TCR) plays an important role in signal transduction and T cell activation, with the TCR-zeta (TCRzeta) chain being a key element in early signaling. We measured the expression of the TCRzeta chain in patients with INS (steroid resistant and steroid sensitive) during relapse and remission by flow cytometry and by PCR ELISA. The results showed a significant decrease in the expression of the TCRzeta chain at both the protein and mRNA level in INS patients during relapse as compared with normal controls (p < 0.05). In contrast, when patients with INS achieved remission, the expression of TCRzeta normalized and was similar to that expressed in normal controls. Therefore, a decreased expression of the TCRzeta chain may explain the abnormal function of T cells in patients with INS, and it may also contribute to the increased risk for infections seen in these patients.