Akutes Leberversagen, akut-auf-chronisches Leberversagen, hepatorenales Syndrom, hepatopulmonales Syndrom und portopulmonale Hypertonie, Leberunterstützungsverfahren auf der Intensivstation

Zusammenfassung

Hintergrund Die akute Leberfunktionsstörung als akutes Leberversagen (ALV) oder akut-auf-chronisches Leberversagen (ACLV) ist ein intensivmedizinisches, interdisziplinär zu behandelndes Krankheitsbild mit einem hohen Letalitätsrisiko.

Ziel Nomenklatur, Pathophysiologie, Prognose und mögliche Therapieoptionen des ALV und des ACLV sollen erläutert werden einschließlich der Möglichkeiten der extrakorporalen Leberersatztherapie an der Schwelle zur Lebertransplantation (LTx).

Methode Narrative Übersicht mit selektiver Literaturrecherche und eigenen repräsentativen Fallbeispielen.

Ergebnisse/Eckpunkte ALV und ACLV haben vielfältige Ursachen und eine hohe Letalität. Beim ALV kommt es auf die genaue Ermittlung der Ursache an, weil teilweise spezielle Therapiemöglichkeiten zur Verfügung stehen. Sowohl bei ALV und ACLV kann zum Überleben eine Lebertransplantation erforderlich sein, für ALV und ACLV existieren unterschiedliche Kriterien für Indikationsstellung und Transplantatallokation. Zur extrakorporalen Leberunterstützung haben sich 2 Verfahren etabliert (MARS [molecuar adsorbent recirculating system] und FPSA [fractionated plasma separation and adsorption] Prometheus^®). Beide Verfahren können in der Lage sein, bei einem ALV oder einem ACLV die Überlebenswahrscheinlichkeit zu erhöhen, und sie werden in einzelnen Fällen zum Bridging zur LTx eingesetzt, außerdem in Einzelfällen zur primären Giftelimination, z. B. nach Amatoxiningestion. Beide Verfahren sind nicht als Dauertherapie geeignet.

Schlussfolgerung Akutes Leberversagen und akut-auf-chronisches Leberversagen sind schwerwiegende Krankheitsbilder mit hohem Letalitätsrisiko. Betroffene Patienten sollten schnellstmöglich einer interdisziplinären Intensivtherapie in einem (tertiären) Zentrum zugeführt werden mit dem Ziel, die Krankheitsursache und mögliche Therapieoptionen hinsichtlich vorgehaltener extrakorporaler Leberunterstützungsverfahren und Lebertransplantation abzuklären.

Abstract

Background Acute hepatic dysfunction in the form of acute liver failure (ALF) or acute-on-chronic liver failure (ACLF) is a disease with a high risk of mortality and requires interdisciplinary intensive care.

Aim This article explains the nomenclature, pathophysiology, prognosis and possible treatment options of ALF and ACLF, including the possibilities of extracorporeal liver support therapy at the point of liver transplantation (LTx).

Method Narrative review with a selective literature review and representative case studies.

Results/Corner Points ALF and ACLF may have several causes and are associated with high mortality. The causes of ALF must be accurately diagnosed because targeted treatment options are available. Both ALF and ACLF may require a liver transplantation for the patientʼs survival. For ALF and ACLF there are different criteria for decision-making on liver transplantation and graft allocation. For extracorporeal liver support therapy, two methods have been established (MARS [molecuar adsorbent recirculating system] and FPSA [fractionated plasma separation and adsorption] Prometheus^®). Both approaches may have the potential to increase the probability of survival of patients with ALF or ACLF. In some cases they can be used for bridging to liver transplantation, in individual cases also for primary poison elimination, e.g. after Amatoxin ingestion. Both methods are not suitable for long-term therapy.

Conclusion Acute liver failure (ALF) and acute on chronic liver failure (ACLF) are serious diseases with a high risk of mortality. Affected patients should receive immediate interdisciplinary intensive care in a (tertiary) centre with the aim to clarify the cause of the disease as well as possible treatment options with respect to available extracorporeal liver support therapy and liver transplantation.

Schlüsselwörter

akutes Leberversagen - akut-auf-chronisches Leberversagen - hepatorenales Syndrom - hepatopulmonales Syndrom - Leberdialyse - MARS

Key words

hepatorenal syndrome - MARS - artificial liver support - hepatopulmonary syndrome - acute-on-chronic liver failure - acute liver failure

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