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Hipogonadismo hipogonadotrófico

HIPOGONADISMO HIPOGONADOTRÓFICO

¿Qué es el hipogonadismo masculino?

El hipogonadismo masculino es la imposibilidad del testículo para producir cantidades normales de testosterona junto con la presencia de síntomas y signos de deficiencia androgénica (deficiencia de hormonas sexuales masculinas). El descenso significativo de la acción androgénica se asocia a un síndrome consistente en osteoporosis, debilidad, redistribución de la grasa corporal, anemia, descenso de la libido y de la función sexual, malestar y anomalías cognitivas. Se trata de una alteración caracterizada por una falta parcial o completa de desarrollo puberal o maduración sexual, debida a una alteración en la secreción de las gonadotrofinas pituitarias LH y FSH, como consecuencia de la deficiencia de GnRH (hormona liberadora de gonadotrofina).

¿Cuáles son las causas y síntomas?

El hipogonadismo masculino se evidencia cuando los testículos dejan de producir espermatozoides, testosterona o ambos. Existen tres mecanismos básicos:

  1. Un daño testicular intrínseco (hipogonadismo primario).

  2. Una anomalía del eje hipotálamo- hipófisis (hipogonadismo secundario).

  3. Una respuesta disminuida o ausente de los órganos blanco (piel, vello y próstata) a los andrógenos (resistencia androgénica).

Algunos de los signos característicos del hipogonadismo hipogonadotrófico pueden incluir ausencia de características sexuales secundarias, como vello púbico, axilar y facial, falta de desarrollo en la pubertad con desarrollo incompleto o retraso significativo, testículos subdesarrollados y, en algunos casos, estatura baja. El HH puede sospecharse desde el nacimiento por la presencia de un micropene en los varones, normalmente asociado a una criptorquidia, por un retraso o ausencia total de la pubertad en la adolescencia o por infertilidad en la edad adulta. El HH es también un rasgo de diversos síndromes como el síndrome de Kallmann, el síndrome de Prader-Willi, el síndrome de Bradet-Biedl, el síndrome de Laurence-Moon y el síndrome CHARGE.

Se sabe que existen determinadas mutaciones en genes relacionados con el desarrollo de hipogonadismo hipogonadotrópico. Las mutaciones que alteran o inactivan al receptor de GnRH fueron las primeras alteraciones detectadas. Sin embargo, en los últimos años, se han identificado otras mutaciones que están íntimamente ligadas al desarrollo de este trastorno.

¿Qué ofrecemos?

Desde OmicasLab, ofrecemos un panel compuesto por una serie de genes implicados en el desarrollo de hipogonadismo hipogonadotrópico, haciendo posible detectar si el paciente posee predisposición genética a desarrollar esta enfermedad. Para la realización de este estudio es necesario una muestra de sangre del paciente, cuyo ADN será extraído y procesado posteriormente utilizando secuenciacion de nueva generación. De esta manera, se informará al paciente de la presencia o ausencia de variantes genéticas asociadas al desarrollo de hipogonadismo hopogonadotrópico.

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