RENAL CYSTS IN PEDIATRIC-PATIENTS - A CLASSIFICATION AND OVERVIEW

被引:32
作者
KISSANE, JM
机构
[1] Department of Pathology, Washington University, Barnes and Affiliated Hospitals, St. Louis, 63110, MO
[2] The St. Louis Children's Hospital, St. Louis, 63110, MO
[3] the Jewish Hospital of St. Louis, St. Louis, 63110, MO
关键词
Acquired renal cystic disease; Autosomal dominant (adult) polycystic renal disease; Autosomal recessive (infantile) polycystic renal disease; Congenital hepatic fibrosis; Polycystic renal disease; Renal cysts; Renal dysplasia; Renal medullary cystic disease;
D O I
10.1007/BF00858446
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Renal cysts are relatively common. They may be single, multiple, or innumerable, cortical or medullary. Most renal cysts are spherical, thin-walled, epithelial-lined structures that contain fluid with features of a plasma ultrafiltrate. A clinically useful classification must include characteristics such as age of onset, family history and association with extrarenal lesions, as well as morphologic parameters. Renal dysplasia is disturbed renal histogenesis, either syndromic or non-syndromic, which usually gives rise to morphologically characteristic renal cysts. Two important and distinctive familial forms of renal polycystic disease occur. Autosomal dominant polycystic disease characteristically becomes manifest in adulthood but is becoming increasingly diagnosed in young individuals. Autosomal recessive infantile polycystic disease has complex relationships with "congenital hepatic fibrosis". Other cystic renal lesions are less common in young patients, but their occurrence in association with other abnormalities provokes speculation regarding pathogenesis. © 1990 IPNA.
引用
收藏
页码:69 / 77
页数:9
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