Abstract
Proteinuria at the time of diagnosis of Wilms tumor (WT) is occasionally seen in patients who may also have other manifestations of the nephrotic syndrome. In such instances of what has been termed the Drash Syndrome there are morphologic changes of focal glomerulosclerosis (FGS).
We have recently evaluated 2 children who were not proteinuric at the time of unilateral nephrectomy for Wilms tumor and who first developed signs of biopsy documented FGS 17 and 11.5 yrs later. One patient, a boy undergoing nephrectomy at 3.5 months of age currently has a 24 hour urinary protein excretion of 7.4 gm, hypoproteinemia, and hypercholesterolemia; his serum creatinine is 1.3 mg/dl. The other patient, a girl undergoing nephrectomy at 1.5 yrs, underwent renal transplantation at 21 yrs. Neither had evidence of recurrent tumor or a radiation or immune complex mediated injury. Thus, although FGS may be present at the time of diagnosis of WT in some patients, we have found that it may occur after a decade or more.
FGS can be produced experimentally by removal of 3/4 total renal mass. Glomerular abnormalities (microglomeruli) are frequent in the non-tumorous kidney of some WT patients and, combined with the contralateral nephrectomy, contribute to the reduction in renal mass. Increased perfusion of the remnant nephrons may explain the subsequent development of FGS in these patients.
Patients with WT should be monitored well into adulthood. Modification of dietary protein intake may affect the evolution of this glomerulopathy.
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Welch, T., McAdams, A. 949 LATE DEVELOPMENT OF FOCAL GLOMERULOSCLEROSIS IN TWO PATIENTS FOLLOWING NEPHRECTOMY FOR WILMS TUMOR. Pediatr Res 19, 269 (1985). https://doi.org/10.1203/00006450-198504000-00979
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DOI: https://doi.org/10.1203/00006450-198504000-00979