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Pubertas tarda bei hypogonadotropem Hypogonadismus

Abklärung, Betreuung, späterer Kinderwunsch

Delayed puberty due to hypogonadotropic hypogonadism

Evaluation, care and support, later fertility

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Gynäkologische Endokrinologie Aims and scope

Zusammenfassung

Die Pubertät leitet von der Kindheit ins Erwachsenenalter über. Die Hypothalamus-Hypophysen-Gonaden-Achse ist während der fetalen und neonatalen Entwicklung aktiviert und fällt danach bis zur Pubertät in eine Ruhephase. Die Pubertät beginnt mit einer Reaktivierung der gonadalen Achse. Es gibt keine allgemeinen Richtlinien dazu, in welchem Alter beim Ausbleiben von Pubertätsmerkmalen eine Abklärung erfolgen muss. Da eine Pubertas tarda dramatische Folgen für die geistige und soziale Entwicklung während der Adoleszenz haben kann, soll bei Mädchen eine erste Evaluation spätestens im Alter von 13 Jahren erfolgen. Bei starkem psychosozialem Druck, der wegen des Entwicklungsrückstands gegenüber Gleichaltrigen auf einem betroffenen Kind lasten kann, muss sie schon früher eingeleitet werden. Ein wichtiger Aspekt, der die Patientin und ihre Eltern oft beunruhigt, sind die möglichen Spätfolgen einer Pubertas tarda für die Fertilität. In der Regel sind bei hypogonadotropem Hypogonadismus die Aussichten auf eine erfolgreiche Schwangerschaft gut, wenn bei Kinderwunsch eine der Diagnose entsprechende adäquate Follikelreifungsinduktion durchgeführt wird. Dieser Beitrag soll dem Nichspezialisten eine Übersicht zu diesem Thema geben.

Abstract

Puberty leads from childhood to adulthood. During fetal and neonatal life the hypothalamo-pituitary-gonadal axis is fully activated and then regresses in a stage of quiescence lasting up to puberty. Puberty then begins with reactivation of the gonadal axis. There are no general rules defining when clinical evaluation should be initiated in the total absence of signs of puberty . Because delayed puberty may have dramatic consequences for the mental and psychosocial development of adolescents, in girls a first evaluation should be started not later than at the age of 13 years; however, evaluation should be initiated earlier if psychosocial pressure is exerted on the child by the peer group because of delayed pubertal development. Another aspect that worries most patients and their parents is the potential long-term problem concerning fertility in adulthood. In general, the chances for a normal pregnancy are good if delayed puberty is due to hypogonadotropic hypogonadism and if in the case of the desire for a child a correct diagnosis is established and an adequate induction of follicular maturation is performed. This article gives a short overview for non-specialized gynecologists of the clinically important aspects of delayed puberty and the measures to be taken.

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Birkhäuser, M. Pubertas tarda bei hypogonadotropem Hypogonadismus. Gynäkologische Endokrinologie 16, 103–114 (2018). https://doi.org/10.1007/s10304-018-0186-y

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