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KMID : 1103720100620040383
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Journal of the Korean Society of Radiology
2010 Volume.62 No. 4 p.383 ~ p.388
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Herlyn-Werner-Wunderlich Syndrome with Unilateral Hemivaginal Obstruction, Ipsilateral Renal Agenesis, and Contralateral Renal Thin GBM Disease: A Case Report with Radiological Follow Up
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Park Noh-Hyuck
Park Chan-Sup
Park Seong-Il
Park Hee-Jin
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Abstract
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Herlyn-Werner-Wunderlich syndrome is a rare Mullerian ductal anomaly that is characterized by the presence of a hemivaginal septum, a didelphic uterus and ipsilateral renal agenesis. It is generally difficult to diagnose the uterine malformation before menarche owing to its small size. Therefore, a follow-up study is very important for confirming the uterine malformation in girls with renal agenesis. We report a patient with renal agenesis and microscopic hematuria, who showed symptoms before menarche. A follow-up study eventually revealed uterine didelphys with a hemivaginal obstruction. A biopsy proved that the microscopic hematuria was caused by thin glomerular basement membrane disease of the contralateral kidney.
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KEYWORD
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Herlyn-Werner-Wunderlich syndrome, Thin glomerular basement membrane disease, Ultrasonography, Tomography, X-Ray Computed, Magnetic Resonance Imaging, Abnormalities, multiple, Uterus
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