6533b85cfe1ef96bd12bd545

RESEARCH PRODUCT

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

L. Ferreres-gómezJ. Cervera-sánchezA. Romeu-sarrióVicente Serra-serraJ. Bellver-pradasA. Boldó-rodaA. Martín-cortés

subject

Hirsutismmedicine.medical_specialtyAdolescentmedia_common.quotation_subjectDiagnosis DifferentialDiabetes mellitusDiabetes MellitusmedicineHumansEndocrine systemMayer-Rokitansky-Kuster-Hauser SyndromeGirlAmenorrheahirsutismmedia_commonGynecologyMedical treatmentbusiness.industrySilver–Russell syndromeUterusObstetrics and GynecologySyndromeGeneral Medicinemedicine.diseaseDermatologyKaryotypingVaginaFemalebusinessHyperinsulinism

description

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.

https://doi.org/10.1007/s004040100172