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KMID : 1040420180220010012
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Childhood Kidney Diseases
2018 Volume.22 No. 1 p.12 ~ p.16
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Herlyn-Werner-Wunderlich Syndrome: A Mini-review
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Lee Ji-Won
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Abstract
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Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.
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KEYWORD
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Mullerian duct anomaly, renal agenesis, hemivagina, Uterus didelphys, female genital anomalies
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