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Silver-Russell syndrome associated to Mayer-Rokitansky-Kuster-Hauser syndrome, diabetes and hirsutism

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Abstract 

We report an unusual association of two rare conditions, the Silver-Russell syndrome and the Mayer-Rokitansky-Küster-Hauser syndrome, with extra endocrine anomalies. A 15-year old Silver-Russell syndrome girl was referred to our Unit because of primary amenorrhea and hirsutism of adrenal origin. A thorough assessment confirmed previous diagnoses and revealed the presence of diabetes, hyperinsulinism and a complete Mayer-Rokitansky-Küster-Hauser syndrome with an ectopic localization of both ovaries. Although initial medical treatment was not unsatisfactory, future medical, psychological and social implications are expected.

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Authors and Affiliations

  1. Department of Gynecologycal Endocrinology, Hospital Universitario ’’La Fe’’, Calle Regacho, 8–14, Mislata 46920, Valencia, Spain e-mail: jbellverp@sego.es. Tel.: 011-34-963624399, Fax: 011-34-963694735, , , , , , ES

    J. Bellver-Pradas, J. Cervera-Sánchez, A. Boldó-Roda & A. Martín-Cortés

  2. Department of P.O.G. Faculty of Medicine, University of Valencia, Spain, , , , , , ES

    V. Serra-Serra & A. Romeu-Sarrió

  3. Department of Gynecology, Hospital Universitario ’’La Fe’’, Valencia, Spain, , , , , , ES

    L. Ferreres-Gómez

Authors
  1. J. Bellver-Pradas
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  2. J. Cervera-Sánchez
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  3. A. Boldó-Roda
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  4. A. Martín-Cortés
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  5. L. Ferreres-Gómez
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  6. V. Serra-Serra
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  7. A. Romeu-Sarrió
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Additional information

Received: 4 December 2000 / Accepted: 31 January 2001

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Bellver-Pradas, J., Cervera-Sánchez, J., Boldó-Roda, A. et al. Silver-Russell syndrome associated to Mayer-Rokitansky-Kuster-Hauser syndrome, diabetes and hirsutism. Arch Gynecol Obstet 265, 155–157 (2001). https://doi.org/10.1007/s004040100172

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  • DOI: https://doi.org/10.1007/s004040100172

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