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Renal angiomyolipomas are found in more than half of the patients with tuberous sclerosis. We report on 3 patients with tuberous sclerosis and pathologically aggressive renal angiomyolipoma with retroperitoneal lymph node involvement and/or renal cell carcinoma. All patients have had a benign course. The literature is reviewed and supports the benign nature of this seemingly aggressive disorder. An approach to the evaluation and treatment of a child with tuberous sclerosis and renal angiomyolipoma is presented.

We compared retrospectively the outcome of renal transplantation in 18 children with a diagnosis of posterior urethral valves to 18 matched children with nonobstructed renal failure to identify the disadvantages of renal transplantation into the valve bladder. There was no statistical difference in patient survival. The 5-year graft survival rate was 62.3% in children with posterior urethral valves and 48% in the control group. The difference was not statistically significant (p = 0.32). A statistically significant increase in serum creatinine from 1.2 to 2.2mg./dl. was noted in the patients with posterior urethral valves, which was not seen in the control group (p = 0.0009). The clinical significance of this finding is unknown.