Lebertransplantation bei metabolischen Lebererkrankungen im Erwachsenenalter

Zusammenfassung

Das klinische Erscheinungsbild der metabolischen Lebererkrankungen ist heterogen. Es reicht vom akuten Leberversagen über Leberzirrhose und Lebertumoren bis hin zur gesunden Leber mit extrahepatischen Komplikationen. Die extrahepatischen Manifestationen haben häufig einen entscheidenden Einfluss auf die Prognose im Spontanverlauf sowie nach Lebertransplantation. Viele metabolische Lebererkrankungen können bei frühzeitiger Diagnose erfolgreich konservativ behandelt werden. Kommt es zum Leberversagen oder drohen irreversible extrahepatische Schäden, ist eine Lebertransplantation indiziert. Die Indikationsstellung orientiert sich an den Standardkriterien zur Lebertransplantation, wobei abhängig von der Grunderkrankung jeweils spezifische Kriterien hinzutreten. Bei extrahepatischen Manifestationen kann sich auch die Notwendigkeit einer Mehrorgantransplantation ergeben. Im Allgemeinen ist die Prognose nach Lebertransplantation bei metabolischen Erkrankungen gut. Zusätzlich werden viele metabolische Defekte durch die Lebertransplantation phänotypisch geheilt. Alternative Ansätze wie die Hepatozytentransplantation oder die Gentherapie sind demgegenüber noch experimentell.

Abstract

The clinical presentation of metabolic liver disease is highly variable, covering acute liver failure, liver cirrhosis, hepatic cancer and various extrahepatic manifestations. Both natural course and prognosis after liver transplantation are substantially influenced by extrahepatic manifestations. In many types of metabolic liver disease, timely diagnosis allows for successful medical treatment. However, progressive liver failure and severe extrahepatic damage can be the indication for liver transplantation. In general, standard transplantation criteria also apply for metabolic liver disease. They have to be modified by disease-specific criteria, and extrahepatic damage may necessitate multiorgan transplantation. The overall prognosis after liver transplantation for metabolic liver disease is favorable. Furthermore, several metabolic defects are phenotypically cured by liver transplantation. Alternative treatments like hepatocyte transplantation or gene therapy are still in the experimental stage.

Metabolische Lebererkrankungen - Lebertransplantation - M. Wilson - hereditäre Hämochromatose - α₁-Antitrypsinmangel - zystische Fibrose - Glykogenspeichererkrankungen - Amyloidose - primäre Hyperoxalurie

Metabolic liver disease - liver transplantation - Wilson’s disease - hereditary hemochromatosis - α-1-antitrypsin deficiency - cystic fibrosis - glycogen storage disease - amyloidosis - primary hyperoxaluria

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