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KMID : 0811820070110020299
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Journal of Korean Society of Pediatric Nephrology
2007 Volume.11 No. 2 p.299 ~ p.305
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Thin Glomerular Basement Membrane Disease with Herlyn-Werner-Wunderlich Syndrome : Uterus Didelphys, Blind Hemivagina and Ipsilateral Renal Agenesis
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Kim Myoung-Soo
Park Yong-Jun
Park Young-Jun
Park Noh-Hyuck
Song Ji-Sun
Kim Byung-Gil
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Abstract
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Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy- proven thin glomerular basement membrane disease which is not related to the above syndrome.
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KEYWORD
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Herlyn - Werner- Wunderlich syndrome, Mullerian duct anomaly, Renal agenesis, Blind hemivagina, Thin glomeraular basement membrane disease
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