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.