Drozdz D, Drozdz M, Gretz N, Möhring K, Mehls O, Schärer K
Division of Pediatric Nephrology, Children's Hospital, University of Heidelberg, Germany.
Pediatr Nephrol. 1998 Oct;12(8):630-6. doi: 10.1007/s004670050517.
Diagnostic and therapeutic strategies in boys with congenital posterior urethral valves (PUV) have much improved in past decades, but the impact of these changes on the progression to end-stage renal disease (ESRD) has rarely been investigated. We followed renal function in 20 boys with PUV from diagnosis to ESRD. From the first observation period (1969-1978) to the second period (1979-1992) we found a marked drop in age at diagnosis, at valve resection, at first increase of serum creatinine (SCr), and at onset of ESRD. The progression was analyzed by calculating the slope of 1/SCr and the probability of renal survival. In all patients combined, renal survival at the age of 10 years was 35%. In children undergoing valve resection in the 1st year of life, renal survival was worse than in those undergoing later surgery (15% vs. 65% after 10 years, P=0.006). Patients with a SCr>1.2 mg/dl before the age of 12 months progressed more rapidly to ESRD than those attaining this level later. The lower the minimum level of SCr observed after initial surgery, the older the patient at the onset of ESRD. The presence of renal dysplasia or hypoplasia, but not of vesicoureteric reflux, was associated with a more rapid progression. Mean body height at ESRD was -2.3+/-1.3 standard deviation score compared with controls, and was lower if PUV was diagnosed before the age of 6 months.
在过去几十年中，先天性后尿道瓣膜（PUV）患儿的诊断和治疗策略有了很大改善，但这些变化对终末期肾病（ESRD）进展的影响很少得到研究。我们对20例患有PUV的男孩从诊断到ESRD进行了肾功能随访。从第一个观察期（1969 - 1978年）到第二个观察期（1979 - 1992年），我们发现诊断时、瓣膜切除时、血清肌酐（SCr）首次升高时以及ESRD发病时的年龄显著下降。通过计算1/SCr的斜率和肾脏存活概率来分析疾病进展情况。所有患者综合来看，10岁时的肾脏存活率为35%。在1岁时接受瓣膜切除的儿童中，肾脏存活率低于那些接受较晚手术的儿童（10年后分别为15%和65%，P = 0.006）。12个月龄前SCr>1.2 mg/dl的患者比之后达到该水平的患者更快进展至ESRD。初次手术后观察到的最低SCr水平越低，患者ESRD发病时的年龄越大。存在肾发育异常或发育不全，但不存在膀胱输尿管反流，与疾病进展更快相关。与对照组相比，ESRD时的平均身高为 -2.3±1.3标准差评分，若在6个月龄前诊断为PUV则身高更低。
