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Poster 24
04127 Menstrual cycle-dependent symptoms in patients with acute hepatic porphyria – Data from the German Porphyria Registry (PoReGer)
  1. Mona Mainert1,
  2. Lea Gerischer2,3,
  3. Rajan Somasundaram1,
  4. Sylvia Mechsner4,
  5. Eva Diehl-Wiesenecker1
  1. 1Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Department of Emergency Medicine and Porphyria Clinic, Berlin, Germany
  2. 2Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Department of Neurology, Berlin, Germany
  3. 3Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Neuroscience Clinical Research Center, Berlin, Germany
  4. 4Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Department of Obstetrics and Gynaekology, Berlin, Germany

Abstract

Introduction Acute Porphyrias (APs) pose particular challenges to physicians due to non-specific symptoms requiring specific diagnostics. Irreversible damage can be prevented by early detection. The German Porphyria Registry (PoReGer) was set up to gain differentiated knowledge about all forms of porphyrias.

APs manifest in episodes that are often triggered by factors such as infections, certain medications, but also sex hormones, and can present in a menstrual cycle-dependent manner. In this project, we aim to analyze , whether the pattern of complaints in AP patients can be distinguished from typical menstrual and/or endometriosis complaints.

Materials and Methods In close cooperation with the Charité Berlin Endometriosis Center, a specified, detailed questionnaire was developed to gather information on menstrual cycle-dependent and menstrual cycle-independent symptoms and symptom perception in adult, premenopausal AP patients with a focus on abdominal pain. The information from the questionnaire was then combined with the information from the PoReGer. All adult, premenopausal women included in the registry were additionally asked to complete the questionnaire.

Results Of 13 patients who completed the questionnaire, 4 (31%) suffer menstrual cycle-dependent lower abdominal pain due to their AP, 4 (31%) suffer only typical menstrual pain and 5 (38%) do not experience any menstrual cycle-dependent symptoms. The AP-associated lower abdominal pain occurs mainly before menstruation and is localized umbilically and suprapubically radiating to the back. The quality of the pain ranged from localised to diffuse and the average pain was given as 6.6/10 on the Numeric Rating Scale. [More patients are expected until September 2024]

Summary One third of patients with APs suffer from menstrual cycle-dependent lower abdominal pain, which can also become a more severe relapsing symptom. Therefore, menstrual cycle-dependent pain also marks an initial symptom to think of porphyria before diagnosis. Strategies for the short- and long-term management of this special patient population will be developed in the course of this project.

http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/bmjgast-2024-ICPP.52

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