Long-term outcome of children with malformative uropathies

125 cases of severe malformative uropathies, 42 urethral valves (V), 52 degree III vesicoureteral refluxes (VUR), 18 ureterovesical junction stenoses (UVJ), 13 pyeloureteral junction stenoses (PUJ), were studied for a period of 12 years. Based on the hypothesis that prognosis depends on the number of residual nephrons, we used the glomerular filtration rate (GFR) as our basic reference. 62% of our cases had an initial GFR below 50 ml/min/1.73 m2 and 30% had GFR's below 25. Early diagnosis and intervention are important for improvement of GFR. Of the 32% who improved, most were diagnosed in the first year of life, and the exceptional few after 2 years. The extent of initial renal damage is also a limiting factor. Improvement was rarely seen when the initial GFR was below 30 ml/min/1.73 m2. There was a correlation between the initial and final GFR levels. Renal degradation (28% of cases) is most influenced by follow up time. The average age of end stage renal failure (ESRF) onset was 11 years 4 months, but is earlier for V than for VUR. Onset is even earlier when initial damage is more severe. As normal GFR does not exclude later degradation of renal function, another indicator of the risk of this type of evolution should be adopted.