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Abstract
Antecedentes: El síndrome de Mayer-Rokitansky-Kuster-Hauser es una entidad que se presenta en 1 por cada 4.500-5.000 nacidos vivos y se caracteriza por cursar con amenorrea primaria en una paciente con desarrollo sexual secundario normal y un cariotipo 46 XX normal.
Resumen del caso: Se presentan dos casos de dos niñas, la primera de 14 años que consulta por amenorrea y la segunda de 10 años de edad, que consultó por cistitis y sinequias de labios mayores. Posteriormente, en los estudios imaginológicos se evidencian anomalías en los genitales internos, como ausencia de útero y anomalías en las trompas uterinas y 2/3 superiores de la vagina.
Conclusión: El SMRKH es una entidad que debe sospecharse en las pacientes que cursen con amenorrea primaria y un desarrollo sexual secundario normal, con cariotipo 46 XX, perfil hormonal normal y en donde las imágenes diagnósticas, la ecografía, en menor grado, y principalmente la resonancia magnética serán de gran ayuda para confirmar el diagnóstico.
Abstract
The Mayer-Rokitansky-Kuster-Hauser Syndrom is an entity with an incidence of 4500-5000 live born and the current presentation is a female with onset of normal secondary sexual characters, amenorrea with 46XX karyotype.
We describe the clinical cases of two girls, the first, a 14 years old girl with amenorrhea and the second a 10 years old girl, with chronic cystitis and synechias of labium major.
It was performed radiologic studies with abnomalites of internal genitalia, like uterus absent, and abnomalities of fallopian tubes and 2/3 upper vagina.
Conclusion: The SMRKH is an entity to suspect in case of female patient with primary amenorrhea, normal onset of secondary sexual characteristics, 46XX karyotype, normal hormone profile and with radiological images like ultrasonography and MRI that confirms the anatomical defects.
References
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