Many pediatric centers utilize a variety of protocols including preemptive plasmapheresis to prevent the recurrence of FSGS post-transplant. But the effectiveness of this expensive, time-consuming process of plasmapheresis in the prevention of FSGS recurrence is still unclear. We retrospectively reviewed all pediatric cases of FSGS in our center that received a kidney transplant and compared the transplant and patient outcomes of those transplanted after 2006 who received pretransplant plasmapheresis to those prior to 2006 who did not. Of the 57 children with FSGS, 31 and 26 were transplanted before and after 2006, respectively. The cohorts differed significantly in keeping with the center immunosuppression protocol changes, and prior to 2006, the recipients were significantly younger. All children with FSGS transplanted after 2006 underwent three and one sessions of 1.0 plasma volume/exchange plasmapheresis with fresh frozen plasma replacement prior to the transplant in living and deceased donors, respectively, in addition to five sessions of every other day post-transplant pheresis. The incidence (27% vs 26%, P = 1.0) and time to recurrence of FSGS in the kidney allograft (P = .22) were not significantly different in patients that did and did not undergo prophylactic plasmapheresis. We need to re-evaluate the role of preemptive plasmapheresis in the prevention of FSGS recurrence in a prospective multicenter study.

The effect of peri-transplant plasmapheresis in the prevention of recurrent FSGS.

Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage renal disease and a common pathologic diagnosis of idiopathic nephrotic syndrome (NS), especially in steroid-resistant cases. FSGS is known to recur after kidney transplantation, frequently followed by graft loss. However, not all patients with FSGS suffer from recurrence after kidney transplantation, and genetic and secondary FSGS have a negligible risk of recurrence. Furthermore, many cases of recurrence achieve remission with the current management of recurrence (intensive plasmapheresis/immunosuppression, including rituximab), and other promising agents are being evaluated. Therefore, a pathologic diagnosis of FSGS itself should not cause postponement of allograft kidney transplantation. For patients with a high risk of recurrence who presented with classical symptoms of NS, that is, severe edema, proteinuria, and hypoalbuminemia, close monitoring of proteinuria is necessary, followed by immediate, intensive treatment for recurrence.

/pdf/recurrence-and-treatment-after-renal-transplantation-in-4k80o2ju7o.pdf

Recurrence and Treatment after Renal Transplantation in Children with FSGS

https://www.ackdjournal.org/article/S1548-5595(14)00064-0/pdf

The Treatment of Idiopathic Focal Segmental Glomerulosclerosis in Adults

Focal segmental glomerulosclerosis (FSGS) represents one of the most severe glomerular diseases, with frequent progression to end-stage renal disease and a high rate of recurrence in renal allografts (30%-50%). Recurrent FSGS portends a negative outcome, with the hazard ratio of graft failure being two-fold higher then that of other glomerulonephritis. Two patterns of clinical presentations are observed: Early recurrence, which is characterized by massive proteinuria within hours to days after implantation of the renal graft, and late recurrence, which occurs several months or years after the transplantation. Many clinical conditions have been recognized as risk factors for recurrence, including younger age, rapid progression of the disease to end-stage renal disease on native kidneys, and loss of previous renal allografts due to recurrence. However, much less is known about the incidence and risk factors of the so-called "de novo" type of FSGS, for which sufferers are transplanted patients without disease on native kidneys; but, rapid development of allograft failure is frequently observed. Management of both forms is challenging, and none of the approaches proposed to date have been demonstrated as consistently beneficial or effective. In the present review we report an update on the available therapeutic strategies for FSGS in renal transplantation within the context of a critical overview of the current literature.

/pdf/update-on-the-treatment-of-focal-segmental-2q2d4w4h91.pdf

Update on the treatment of focal segmental glomerulosclerosis in renal transplantation.

https://www.ajkd.org/article/S0272-6386(13)01310-3/pdf

Diagnostic Tests and Treatment Options in Glomerular Disease: 2014 Update

Focal segmental glomerular sclerosis (FSGS) is a leading cause of steroidresistant nephrotic syndrome. Forty percent of patients with primary FSGS have increased levels of a circulating factor that increases glomerular permeability to protein. FSGS recurs after transplant in approximately 30% of cases, with graft loss in up to 50% of these patients. Recurrence typically occurs early, often within hours posttransplant, which suggests the causative role of the FSGS permeability factor (FSPF) (1). FSPF consists of anionic lowmolecular-weight protein(s) that have a high affinity for galactose (1). The FSPF alters phosphorylation of several glomerular proteins, and incubation with a phosphatase enzyme inhibits permeability activity (1). Galactose is a sugar that can be given intravenously or orally, which has no known side effects. Prior studies have shown that increased levels of the FSPF, assayed based on changes in glomerular capillary permeability (Palb) in vitro, are decreased by treatment with galactose (2). Savin et al. (1) have shown that oral galactose therapy diminished FSPF activity in a series of samples collected from patients with FSGS. Thus, testing for Palb is considered the best correlate of FSPF activity. Palb in pooled human serum is taken as 0. Values between 0.2 and 0.5 are nonspecific and common in patients with renal disease. Any value more than 0.5 has been associated with high incidence of FSGS after kidney transplantation (1). In a recent case report, long-standing remission of native kidney FSGS was achieved with oral galactose therapy given for 7 months (3). We present the first case of a patient with posttransplant recurrence of FSGS that was treated with a multimodality therapy including a high-galactose diet and oral galactose supplementation leading to remission for more than 8 months. A 46-year-old white woman with biopsy-proven primary tip variant FSGS diagnosed in 1984 had recurrence of her disease after a living-related renal transplant in December 2009. On a basiliximab induction and triple drug regimen (prednisone, tacrolimus, and mycophenolic acid), she developed massive proteinuria, 37 g/24 hr, within 2 days after transplantation. This was followed by progressive deterioration in renal function with a peak creatinine concentration of 3.5 mg/dL after a nadir level of 1.5 mg/dL. A kidney biopsy performed 1 week posttransplant revealed diffuse effacement of podocytes on electron microscopy, a change from the 0-hr biopsy, which was normal. All viral serologies including parvovirus B19, cytomegalovirus, BK and adenovirus were negative. Kidney biopsy did not show any component of antibody-mediated rejection (C4d negative, donor-specific antibody negative). Soluble CD80 was determined in the urine using a commercially available ELISA kit (Bender MedSystems, Burlingame), and the result was negative. This finding excluded minimal change disease as the cause of the proteinuria (4). Despite multimodal therapies with 15 treatments of plasmapheresis (PE), two doses of intravenous immunoglobulin, and two doses of rituximab (1 g), the patient’s proteinuria remained in the nephrotic range (9 g/24 hr). Two months after transplantation, she was started on a high-galactose diet (celery, beets, fresh basil, milk, and honey), and a month later, supplemental powder galactose 0.2 g/kg orally two times per day was added. After 2 months of therapy (Fig. 1), she came off PE, and her spot urine protein:creatinine ratio stabilized at 0.3. A

Treatment of recurrent focal segmental glomerular sclerosis posttransplant with a multimodal approach including high-galactose diet and oral galactose supplementation.

Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease usually seen in middle-aged and older adults but which is rare in children and adolescents. We sought to determine if there has been a change in the incidence of this disorder. Methods: Single-center, retrospective review. Results: Over the last 2 years, we have encountered a striking increase in the frequency of this disease in pediatric patients. All eight patients seen during this period had renal involvement and 5 patients rapidly progressed to end stage kidney disease. The prognosis was worse in younger patients, those with microscopic polyangiitis, and those with chronic kidney damage in the diagnostic renal biopsy. Conclusions: We report these observations to highlight this change in the epidemiology of ANCA-associated vasculitis and to promote earlier recognition and treatment of this severe form of glomerulonephritis.

/pdf/anti-neutrophil-cytoplasmic-antibody-vasculitis-in-pediatric-4lqsev381v.pdf

Anti-Neutrophil Cytoplasmic Antibody Vasculitis in Pediatric Patients: Is the Incidence Rising?

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Treatment of recurrent focal segmental glomerular sclerosis posttransplant with a multimodal approach including high-galactose diet and oral galactose supplementation.

Anti-Neutrophil Cytoplasmic Antibody Vasculitis in Pediatric Patients: Is the Incidence Rising?