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Was Morbus Osler und Präeklampsie (nicht) gemeinsam haben

What Osler–Weber–Rendu disease and pre-eclampsia do (not) have in common

  • Gynäkologie aktuell
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Zusammenfassung

Das Rendu-Osler-Weber-Syndrom Typ 1 (hereditäre hämorrhagische Teleangiektasie 1, HHT1) und die Präeklampsie basieren beide auf einer pathologischen Funktion des Endoglins. Patienten mit HHT1 haben einen heterozygoten Endoglingendefekt, der zu einer systemischen vaskulären Dysplasie führt. Bei der Präeklampsie produziert der Trophoblast lösliches Endoglin (sEng). sEng wirkt wie ein „Antikörper“, der das mütterliche Endoglinrezeptorsubstrat hemmt und antagonisiert. Dies führt zu einer endothelialen Dysfunktion. Im Beitrag werden 2 verschiedene endoglinbedingte Erkrankungen miteinander verglichen, um eine fundiertere Einsicht in die spezifischen klinischen Merkmale zu gewinnen. Diskutiert werden ferner Managementoptionen für schwangere Patientinnen mit HHT.

Abstract

Rendu–Osler–Weber syndrome type 1 (hereditary hemorrhagic telangiectasia 1, HHT 1) and pre-eclampsia both base on a pathological function of endoglin. Patients with HHT 1 have a heterozygous endoglin gene defect, resulting in systemic vascular dysplasia. In pre-eclampsia, trophoblast produces soluble endoglin (sEng). sEng acts like an “antibody” which inhibits and antagonizes the maternal endoglin-receptor substrate, resulting in endothelial dysfunction. In this article, two different endoglin-related disorders are compared with each other with the aim to provide more insight into the specific clinical features. Additionally, management options for pregnant patients with HHT are discussed.

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Authors and Affiliations

  1. Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Essen, Hufelandstraße 55, 45147, Essen, Deutschland

    Angela Köninger, Buu-Phuc Nguyen, Alexandra Gellhaus & Rainer Kimmig

  2. Klinik für Hals-Nasen-Ohren-Heilkunde, Universitätsklinikum Essen, Hufelandstraße 55, 45147, Essen, Deutschland

    Freya Dröge

  3. Klinik für Hals-Nasen-Ohren-Heilkunde, Universitätsklinikum Marburg, Philipps Universität Marburg, Baldingerstraße, 35043, Marburg, Deutschland

    Urban Geisthoff

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  1. Angela Köninger
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  2. Buu-Phuc Nguyen
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Correspondence to Angela Köninger.

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Interessenkonflikt

A. Köninger, B.-P. Nguyen, A. Gellhaus, F. Dröge, R. Kimmig und U. Geisthoff geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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T. Fehm, Düsseldorf

L. Kiesel, Münster

R. Kimmig, Essen

N. Maass, Kiel

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Köninger, A., Nguyen, BP., Gellhaus, A. et al. Was Morbus Osler und Präeklampsie (nicht) gemeinsam haben. Gynäkologe 52, 52–58 (2019). https://doi.org/10.1007/s00129-018-4336-z

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  • DOI: https://doi.org/10.1007/s00129-018-4336-z

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