Zusammenfassung
Das akute Leberversagen ist ein seltenes und lebensbedrohliches Krankheitsbild, welches infolge einer schweren Leberschädigung bei bisher gesunden oder asymptomatischen Patienten auftreten kann. Die hohe Mortalität der Erkrankung ist vor allem auf zwei Komplikationen zurückzuführen, deren Entstehung von der zeitlichen Entwicklung der Leberschädigung abhängt. Das hyperakute Leberversagen kann aufgrund erhöhter arterieller Ammoniakkonzentrationen innerhalb weniger Tage zur Entwicklung eines Hirnödems und zu einer oft letalen Hirnstammeinklemmung führen, welche durch frühzeitige Lebertransplantation verhindert werden kann. Bei Patienten, welche das initiale Hirnödem überleben, wird häufig eine spontane Regeneration mit völliger Gesundung beobachtet. Entwickelt sich eine Leberschädigung hingegegen langsamer (akutes oder subakutes Leberversagen), so kann die zerebrale Osmoregulation eine Einkemmung meist verhindern. Diese Patienten haben jedoch eine schlechte Regenerationsfähigkeit und benötigen die Lebertransplantation vor dem Auftreten lebensbedrohlicher Komplikationen wie Sepsis, Nierenversagen oder Multiorganversagen. Einige Zentren versuchen, die Progression der Erkrankung durch den Einsatz extrakorporaler Entgiftungsverfahren zu verhindern, zumal erhöhte Ammoniakkonzentrationen durch Hämodialyse abgesenkt werden können. Pharmakologische Interventionen, welche die massive Apoptose in der Leber reduzieren und die Regeneration unterstützen könnten, befinden sich in Entwicklung.
Summary
Acute liver failure is a rare and life-threatening clinical syndrome following severe hepatic injury. Depending on the rapidity of its development, two distinct complications contribute to a high mortality: in hyperacute liver failure, rapid development of massive hepatic necrosis and apoptosis gives rise to severe hyperammonemia, hepatic encephalopathy and life-threatening cerebral edema. The high risk of cerebral herniation requires early listing for emergency liver transplantation. Patients with hyperacute liver failure surviving the initial episode of cerebral edema have a substantial potential for hepatic recovery. If progressive hepatic failure develops more slowly, astrocytic osmoregulation prevents cerebral herniation in most intances. Unfortunately, these patients have a small potential of hepatic regeneration and transplantation should be performed before renal failure, sepsis or multiorgan failure emerge. Experimental treatment methods including detoxification by artificial or bioartificial liver support or by stimulating hepatic regeneration are currently evaluated. Recognition of ammonia toxicity has stimulated the search for early ammonia-lowering strategies and strongly renewed the interest in dialytic therapies. Anti-apoptotic interventions are among the most promising pharmacological options for the near future.
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Kramer, L. Acute liver failure. Wien Klin Wochenschr 116, 67–81 (2004). https://doi.org/10.1007/BF03040699
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DOI: https://doi.org/10.1007/BF03040699
Schlüsselwörter
- Akutes Leberversagen
- Ammoniak
- Enzephalopathie
- fulminant
- Glutamin
- Hirnödem
- Leberunterstützung
- Transplantation