John U, Rudnik-Schöneborn S, Zerres K, Misselwitz J
Division of Paediatric Nephrology, University Children's Hospital, Jena, Germany.
Pediatr Nephrol. 1998 Sep;12(7):567-71. doi: 10.1007/s004670050506.
The natural history of multicystic dysplastic kidney (MCDK) is not well established. We analyzed kidney growth and renal function in 33 children with prenatally diagnosed unilateral MCDK in a long-term study. The mean observation period was 4.9 years with a range of 1-11.6 years. Abnormalities of the contralateral kidney were found in 10 of 33 patients (30%): ureteropelvic junction obstruction (5), ureterovesical junction obstruction (2), and vesicoureteral reflux (3). In 6 children the dysplastic kidney had been removed. Complete involution was observed in 48% and a decrease of size in 33% of 27 dysplastic kidneys. At the time of last examination, 27 of 29 children showed a volume of the contralateral kidney above the normal range (>145%). Hypertrophy of the contralateral kidney, defined as kidney length above 2 standard deviation scores (SDS), was seen in 24% of 33 children at birth, thus showing that hypertrophy of the contralateral kidney starts in utero and continues throughout childhood. The extent of contralateral hypertrophy was independent of associated abnormalities in this study. Mean creatinine was increased in the whole group (mean +1.13 SDS). Calculated creatinine clearance in 21 patients over 2 years was within normal limits, with a median of 102 ml/min per 1.73 m2 (range 84-143). Based on the results of this and previous studies, nephrectomy cannot be recommended in typical cases, but a regular follow-up of these patients seems necessary.
多囊性发育不良肾（MCDK）的自然病史尚未完全明确。在一项长期研究中，我们分析了33例产前诊断为单侧MCDK患儿的肾脏生长情况及肾功能。平均观察期为4.9年，范围为1至11.6年。33例患者中有10例（30%）对侧肾脏存在异常：肾盂输尿管连接处梗阻（5例）、输尿管膀胱连接处梗阻（2例）以及膀胱输尿管反流（3例）。6例患儿的发育不良肾脏已被切除。在27个发育不良肾脏中，48%观察到完全萎缩，33%体积减小。在最后一次检查时，29例患儿中有27例对侧肾脏体积高于正常范围（>145%）。33例患儿中有24%在出生时对侧肾脏肥大，定义为肾脏长度高于2个标准差评分（SDS），这表明对侧肾脏肥大始于子宫内并持续至整个儿童期。在本研究中，对侧肥大的程度与相关异常无关。全组平均肌酐水平升高（平均+1.13 SDS）。21例患者2年的计算肌酐清除率在正常范围内，中位数为每1.73 m² 102 ml/min（范围84 - 143）。基于本研究及既往研究结果，对于典型病例不建议行肾切除术，但对这些患者进行定期随访似乎是必要的。
