Hains David S, Bates Carlton M, Ingraham Susan, Schwaderer Andrew L
Department of Pediatrics, Division of Pediatric Nephrology, The Ohio State University College of Medicine, 700 Children's Drive, Columbus, OH 43205, USA.
Pediatr Nephrol. 2009 Feb;24(2):233-41. doi: 10.1007/s00467-008-0828-8. Epub 2008 May 15.
In children, unilateral multicystic dysplastic kidney (MCDK) is one of the most frequently identified urinary tract abnormalities. A variety of proposed etiologies has been associated with the underlying pathogenesis of MCDK. These include genetic disturbances, teratogens, in utero infections, and urinary outflow tract obstruction. From 5-43% of the time, MCDK has associated genito-urinary anomalies, both structural and functional in nature. A review of the literature reveals that involution rates are reported to be 19-73%, compensatory hypertrophy of the contralateral kidney occurs from 24-81% of the time, and estimated glomerular filtration rates (GFRs) (by the Schwartz formula) range from 86-122 ml/min per 1.73 m(2) body surface area. Most authors suggest serial ultrasonography to monitor contralateral growth, routine blood pressure monitoring, and a serum creatinine monitoring algorithm. The risk of hypertension in those with MCDKs does not appear to be greater than that of the general population, and the rates of malignant transformation of MCDK are small, if at all increased, in comparison with those in the general population. If the patient develops a urinary tract infection or has abnormalities of the contralateral kidney, shown on ultrasound, a voiding cystourethrogram is recommended. Finally, the body of literature does not support the routine surgical removal of MCDKs.
在儿童中，单侧多囊性发育不良肾（MCDK）是最常被发现的泌尿系统异常之一。多种病因被认为与MCDK的潜在发病机制有关。这些病因包括基因紊乱、致畸物、宫内感染和泌尿道梗阻。MCDK有5%至43%的概率伴有生殖泌尿系统异常，包括结构和功能方面的异常。文献综述显示，据报道其退化率为19%至73%，对侧肾代偿性肥大的发生率为24%至81%，估计肾小球滤过率（GFR）（根据施瓦茨公式）范围为每1.73平方米体表面积86至122毫升/分钟。大多数作者建议通过系列超声检查来监测对侧肾的生长情况，进行常规血压监测以及采用血清肌酐监测方案。患有MCDK的患者患高血压的风险似乎并不高于普通人群，而且与普通人群相比，MCDK发生恶性转化的概率即便有所增加也是很小的。如果患者发生尿路感染或超声显示对侧肾有异常，建议进行排尿性膀胱尿道造影。最后，现有文献并不支持常规手术切除MCDK。
