Korean Journal of Pediatrics 2008;51(12):1355-1358.
Published online December 15, 2008.
Wilms` tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome: Successful treatment of the first case with bilateral Wilms` tumors in Korea
Kyung Sun Min1, Hee Jo Baek1, Dong Kyun Han1, Ju Hee You1, Tai Ju Hwang1, Dong Deuk Kwon2, Hoon Kook1
1Departments of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
2Departments of Urology, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
윌름즈 종양, 무홍체증, 비뇨생식기계 기형, 정신지체 (WAGR) 증후군 : 양측성 윌름즈 종양을 성공적으로 치료한 국내 첫 증례 보고
민경선1, 백희조1, 한동균1, 유주희1, 황태주1, 권동득2, 국 훈1
1전남대학교 의과대학 소아과학교실
2전남대학교 의과대학 비뇨기과학교실
Correspondence: 
Hoon Kook, Email: hoonkook@chonnam.ac.kr
Abstract
Wilms` tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome is caused by deletion of chromosome 11p13, including the Wilms` tumor (WT1) and aniridia gene (PAX6) loci. Here, we report the first case of WAGR syndrome in Korea; the patient was a 2-year-old girl with bilateral aniridia from birth who presented with abdominal distention and mental retardation. Cytogenetically, she had deletion of chromosome 11p11.2-13. Bilateral Wilms` tumors were successfully treated by chemotherapy and surgery. She has been tumor-free for 19 months off chemotherapy with preserved renal function.
Key Words: WAGR syndrome, Wilms` tumor, Aniridia


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