ABSTRACT
Tuberous sclerosis is associated commonly with renal angiomyolipoma. However, the radiographic differentiation of angiomyolipoma and renal carcinoma can be difficult, and the natural history of angiomyolipoma may be associated with serious complications. We present a case of bilateral renal cell carcinoma associated with tuberous sclerosis. Aggressive but conserving renal surgery should be considered in patients with tuberous sclerosis and enlarging renal masses.
Subject(s)
Carcinoma, Renal Cell/complications , Kidney Neoplasms/complications , Tuberous Sclerosis/complications , Adult , Angiography , Carcinoma, Renal Cell/diagnostic imaging , Carcinoma, Renal Cell/surgery , Diagnosis, Differential , Female , Hemangioma/diagnostic imaging , Humans , Kidney Neoplasms/diagnostic imaging , Kidney Neoplasms/surgery , Lipoma/diagnostic imaging , Tomography, X-Ray ComputedABSTRACT
The course of 54 patients (35 boys and 19 girls) with end-stage renal disease resulting from obstructive uropathy was reviewed. The mean age at the initial sign of obstructive uropathy was 3.5 years. Twenty-two patients (41%) manifested evidence of obstructive uropathy during the first year of life. The mean age at the time of onset of ESRD (dialysis) was 12.2 years and was similar in boys and girls. The mean time interval between the first sign of obstructive uropathy and the initiation of dialysis was nine years. Fourteen patients operated upon at less than one year of age developed ESRD one to 20 years (mean ten years) following their initial surgery. Progression to ESRD occurred despite appropriate surgical management, including corrective as well as diversionary urologic procedures. However, because the patients were selectively referred for care of ESRD, no assessment of the incidence of ESRD caused by obstructive uropathy was possible. The data indicate that prolonged follow-up periods are necessary to assess the ultimate outcome of renal function in young patients with obstructive uropathy. Despite early intervention and intact renal function for many years during childhood, progression to ESRD may occur.
Subject(s)
Kidney Diseases/etiology , Urethral Obstruction/complications , Urinary Bladder Neck Obstruction/complications , Urinary Tract Infections/complications , Adolescent , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Kidney/abnormalities , Kidney/surgery , Kidney Diseases/surgery , Kidney Diseases/therapy , Kidney Failure, Chronic/etiology , Male , Ureter/surgery , Urethra/surgery , Urethral Obstruction/surgery , Urinary Bladder Neck Obstruction/surgery , Urinary Bladder Neck Obstruction/therapy , Urinary Tract Infections/surgeryABSTRACT
The outcome of renal transplantation was examined in 52 pediatric patients (mean age 13 years) whose primary renal disease was obstructive uropathy. The bladder was used at transplantation in 45 allograft recipients, 39 of whom had had a previous lower urinary tract operation or bladder defunctionalization. An ileal loop was used in 7 recipients. The 52 patients received 73 renal allografts from 58 cadaver and 15 live-related donors. Presently, 40 patients (77 per cent) have functioning allografts, 4 have returned to dialysis and 8 (15 per cent) have died. The results indicate that the outcome of renal transplantation in patients with obstructive uropathy is similar to that of other transplant recipients. Damaged and defunctionalized bladders may be used successfully in most cases. If necessary an ileal conduit is an effective alternative. Post-transplant urologic complications occur with increased frequency but with appropriate management allograft salvage and patient survival are excellent.
