Heymans C, Breysem L, Proesmans W
Department of Radiology, University Hospital Gasthuisberg, University of Leuven, Belgium.
Eur J Pediatr. 1998 Aug;157(8):673-5. doi: 10.1007/s004310050909.
In a 6-year period, 41 young infants with multicystic kidney dysplasia were seen in our department. In 30 cases, the diagnosis had already been suspected by prenatal ultrasonography. A prospective protocol was proposed to the parents which comprised ultrasound evaluation every 3 months until the age of 24 months and renal function assessment at the age of 18 months. In 33 patients, the study was completed as scheduled. At the start of the study, the maximal diameter of the multicystic kidney was above the mean length of normal kidneys in all cases where precise measurement was possible. Within 24 months, 7 of the dysplastic kidneys disappeared, 20 regressed in size, 1 remained unchanged and only 5 increased in size. Between the age of 0 to 3 months, renal length of the contralateral kidney was within the normal range in 19 infants and above +2SD in 14 cases. At the age of 18 to 24 months, renal length was, with few exceptions, between 0 and +4SD. Inulin clearance was normal in all 33 individuals with a median value of 112 ml/min per 1.73 m2.
As a rule, multicystic kidneys shrink in the first 2 years of life. In most cases the contralateral kidney maintains a normal renal function as a consequence of progressive compensatory hypertrophy.
在6年期间，我科共诊治41例患有多囊性肾发育不良的幼儿。其中30例在产前超声检查时已被怀疑患有该病。我们向家长提出了一项前瞻性方案，包括每3个月进行一次超声评估直至24个月龄，并在18个月龄时评估肾功能。33例患者按计划完成了研究。在研究开始时，所有能够精确测量的多囊性肾的最大直径均大于正常肾脏的平均长度。在24个月内，7个发育不良的肾脏消失，20个缩小，1个大小不变，仅5个增大。在0至3个月龄时，19例婴儿对侧肾脏的长度在正常范围内，14例高于+2标准差。在18至24个月龄时，除少数例外，肾脏长度在0至+4标准差之间。33例患者的菊粉清除率均正常，中位数为每1.73平方米112毫升/分钟。
通常情况下，多囊性肾在生命的头2年会缩小。在大多数情况下，对侧肾脏由于进行性代偿性肥大而维持正常肾功能。
