Wilms' tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome: Successful treatment of the first case with bilateral Wilms' tumors in Korea.
10.3345/kjp.2008.51.12.1355
- Author:
Kyung Sun MIN
1
;
Hee Jo BAEK
;
Dong Kyun HAN
;
Ju Hee YOU
;
Tai Ju HWANG
;
Dong Deuk KWON
;
Hoon KOOK
Author Information
1. Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea. hoonkook@chonnam.ac.kr
- Publication Type:Case Report
- Keywords:
WAGR syndrome;
Wilms' tumor;
Aniridia
- MeSH:
Aniridia;
Humans;
Intellectual Disability;
Korea;
Parturition;
Preschool Child;
WAGR Syndrome;
Wilms Tumor
- From:Korean Journal of Pediatrics
2008;51(12):1355-1358
- CountryRepublic of Korea
- Language:English
-
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.
