Mathieu H, Loirat C, Macher M A, Weisgerber G, Guedeney J, Pillion G, Guesnu M
Int J Pediatr Nephrol. 1985 Jan-Mar;6(1):3-12.
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.
对125例严重畸形性泌尿系统疾病患者进行了为期12年的研究，其中包括42例尿道瓣膜（V）、52例III度膀胱输尿管反流（VUR）、18例输尿管膀胱连接部狭窄（UVJ）、13例肾盂输尿管连接部狭窄（PUJ）。基于预后取决于残余肾单位数量的假设，我们将肾小球滤过率（GFR）作为基本参考指标。我们的病例中有62%的初始GFR低于50 ml/min/1.73 m²，30%的GFR低于25。早期诊断和干预对于提高GFR很重要。在病情好转的32%的患者中，大多数在出生后第一年被诊断出来，只有极少数在2年后被诊断出来。初始肾损伤的程度也是一个限制因素。当初始GFR低于30 ml/min/1.73 m²时，很少能看到病情好转。初始和最终GFR水平之间存在相关性。肾脏退化（28%的病例）受随访时间的影响最大。终末期肾衰竭（ESRF）发病的平均年龄为11岁4个月，但V患者比VUR患者发病更早。当初始损伤更严重时，发病甚至更早。由于正常的GFR并不排除后期肾功能退化，应该采用另一种这种类型演变风险的指标。
