Mir S, Rapola J, Koskimies O
Nephron. 1983;33(3):189-95. doi: 10.1159/000182941.
In a series of 6,521 consecutive autopsies of infants and children, renal cysts were found in 136 cases (2%). Of these, 71 were females and 65 males; 103 patients had died in their first month of life. The different types of cystic disease represented were as follows: renal cystic dysplasia 65, cortical cysts in syndromes of multiple malformations 42, polycystic disease 16, simple cortical cysts 7, renal cysts in hereditary syndromes 3, and renal medullary cystic disorders 3. Extrarenal malformations were encountered in 102 of the 136 cases with renal cysts. Gastrointestinal malformations and in particular esophageal atresia very often appeared to be associated with renal disease. Esophageal atresia was found in 29 (21%) cases, 20 in cases with renal cystic dysplasia and 9 in cases with cortical cysts in syndromes of multiple malformations. The possibility of renal cystic disease should therefore be kept in mind in infants with congenital malformation(s), especially those with esophageal atresia.
在对6521例婴幼儿进行的连续尸检中，发现136例（2%）有肾囊肿。其中，女性71例，男性65例；103例患者在出生后第一个月内死亡。所发现的不同类型的囊性疾病如下：肾囊性发育不良65例，多发畸形综合征中的皮质囊肿42例，多囊病16例，单纯皮质囊肿7例，遗传性综合征中的肾囊肿3例，以及肾髓质囊性疾病3例。136例有肾囊肿的病例中，102例存在肾外畸形。胃肠道畸形，尤其是食管闭锁，似乎常常与肾病相关。29例（21%）发现有食管闭锁，其中20例在肾囊性发育不良病例中，9例在多发畸形综合征中的皮质囊肿病例中。因此，对于患有先天性畸形的婴儿，尤其是患有食管闭锁的婴儿，应考虑到肾囊性疾病的可能性。
