Article
Focal segmental glomerulosclerosis in patients with complete deletion of one WT1 allele.
Pediatrics - 1 Jun 2012
Iijima Kazumoto, Someya Tomonosuke, Ito Shuichi, Nozu Kandai, Nakanishi Koichi, Matsuoka Kentaro, Ohashi Hirofumi, Nagata Michio, Kamei Koichi, Sasaki Satoshi
Abstract excerpt
The renal prognosis of patients with Wilms' tumor, aniridia, genitourinary anomalies, and mental retardation syndrome (WAGR) is poor. However, the renal histology and its mechanisms are not well understood. We performed renal biopsies in 3 patients with WAGR syndrome who had heavy proteinuria. The complete deletion of one WT1 allele was detected in each patient by constitutional chromosomal deletion at 11p13...
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