Journal of Human Reproductive Science
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CASE REPORT Table of Contents   
Year : 2013  |  Volume : 6  |  Issue : 4  |  Page : 277-279
A rare case of Turner's syndrome presenting with Mullerian agenesis

Department of Medicine, GSL Medical College and Hospital, Rajahmundry, Andhra Pradesh, India

Correspondence Address:
Suresh Vaddadi
Department of Medicine, GSL Medical College and Hospital, Rajahmundry - 500 082, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-1208.126313

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Turner's syndrome also called as Ullrich Turner's syndrome, is a disease of unclear pathogenesis characterized by complete or partial absence of one sex chromosome, with or without cell line mosaicism in a phenotypic female with short stature. Various anomalies result in a constellation of features, of which the most disturbing is primary amenorrhea due to gonadal dysgenesis. Hormone therapy in these patients can often result in successful menstruation, and scope for subsequent pregnancy because of anatomically normal uterus and vagina. Coexisting Mullerian agenesis in these patients can jeopardize the chances of future pregnancy as they have associated structural abnormalities of the uterus and vagina. We report a rare case of middle-aged female with Turner's syndrome and Mullerian agenesis having absent secondary sexual characters and missing uterus with incompletely formed vagina.

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