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We offer a case study in pre- and post-transplant focal segmental glomerulosclerosis (FSGS).
In 2004, an 8-year-old boy was referred to the Kidney Clinic at Cincinnati Children’s for new-onset nephrotic syndrome with gross hematuria. Although the presence of the latter suggested the nephrotic syndrome’s cause was not minimal change disease, the child responded to a standard treatment course of steroids by going into remission. Nevertheless, relapses occurred quickly and he rapidly became steroid resistant, leading to a diagnostic kidney biopsy, which showed one globally sclerotic glomerulus and otherwise only minimal changes.
While not proving focal segmental glomerulosclerosis (FSGS), these pathological findings − paired with the child’s clinical course − were not inconsistent with FSGS. Accordingly, second- and third-line therapies, including courses of cyclophosphamide, mycophenolate mofetil and tacrolimus, were initiated without much success. Instead, multiple hospitalizations ensued, beginning at the age of 10, for infectious and thrombotic complications of the boy’s persistent nephrotic state and gradual deterioration of renal function.
Persistent nephrosis and progressive chronic kidney disease, in addition to related cardiac dysfunction, subsequently necessitated bilateral native nephrectomies (which confirmed the diagnosis of FSGS) and access placement for hemodialysis initiation. While undergoing an aggressive dialysis regimen, the boy’s nephrotic state resolved and his cardiac function stabilized, paving the way for a kidney transplant.
FSGS is one of the most common causes of end-stage renal disease in children and is associated with particularly high pre- and post-transplant morbidity. Most important, the disease recurs in approximately one-third of all cases, typically soon after transplantation, and can lead to graft loss.
Strategies to prevent or treat recurrent FSGS remain controversial and substantially resource-intense and include plasmapheresis and anti-CD20 antibody (rituximab) infusions, in addition to standard post-transplant care including immunosuppression. At Cincinnati Children’s, end-stage renal disease (ESRD) secondary to FSGS is managed in a step-wise, multidisciplinary approach led by Jens Goebel, MD, medical director of our Kidney Transplant Program. Our nephrology and transplant surgery teams are involved along with the pheresis service at the University of Cincinnati’s Hoxworth Blood Center.
At 12 years of age, the boy received a kidney transplant from his mother, which was complicated by immediate disease recurrence without compromise of graft function. Pheresis was accordingly continued, initially three times weekly, and gradually tapered based on the degree of proteinuria. After almost a year of therapy for FSGS recurrence, the boy was successfully weaned, has maintained good transplant function and has remained free of proteinuria. His cardiac function has normalized; he continues to receive standard immunosuppression to prevent rejection and is back in school.
Learn more about the ongoing research efforts in the Division of Nephrology and Hypertension.
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