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Akutes Leberversagen im Kindesalter

Acute liver failure in children

Zusammenfassung

Das akute Leberversagen, eine hepatisch bedingte Gerinnungsstörung mit INR (INR: „international normalized ratio“) ≥1,5 (nicht durch Vitamin-K-Gabe korrigierbar) mit oder INR≥2,0 ohne Enzephalopathie, ist lebensbedrohlich. Unter Ausschöpfung aller Therapiemöglichkeiten werden heute durch frühzeitige Verlegung in ein Transplantationszentrum Überlebensraten von rund 70% erreicht. Im Neugeborenen- und Säuglingsalter prädominieren metabolische und infektiöse Ursachen sowie die neonatale Hämochromatose. Ab dem Kleinkindesalter, v. a. aber dem Schulalter, spielen medikamenteninduzierte oder autoimmunologische Ursachen sowie der Morbus Wilson eine zunehmende Rolle. Fast 50% der Leberversagen im Kindes- und Jugendalter bleiben ätiologisch ungeklärt. Die Therapie richtet sich nach der Ätiologie und ist in vielen Fällen symptomatisch. Da das Hirnödem die häufigste Todesursache darstellt, sind eine Flüssigkeits- und Kochsalzrestriktion sowie Kopfhochlagerung wichtig. Blutungen sind trotz schlechter Gerinnungswerte die Ausnahme, deswegen sollte gerinnungsaktives Frischplasma nur zurückhaltend gegeben werden.

Abstract

Acute liver failure, defined as the onset of hepatic coagulopathy with an INR ≥1.5 (which is not corrected by vitamin K administration) with encephalopathy or with an INR ≥2.0 without encephalopathy, is a life-threatening disorder. Timely referral of the patient to a transplant center can, when all therapeutic strategies are employed, result in survival rates of approximately 70%. In neonates and infants infectious and metabolic causes as well as neonatal hemochromatosis are predominant. In small children, particularly school-age children, drug-induced or autoimmune causes as well as Wilson’s disease play an increasing role. Almost 50% of cases remain etiologically uncertain. Therapy is based on etiology and is symptomatic in many cases. Since cerebral edema is the primary cause of death, restriction of fluid and sodium chloride is important; in addition, the head should be maintained in an elevated position. Despite unfavorable coagulation values, bleeding is an exception; fresh plasma should therefore be used with restraint.

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Correspondence to P. Gerner.

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Gerner, P., Hoyer, P. Akutes Leberversagen im Kindesalter. Monatsschr Kinderheilkd 157, 1157 (2009). https://doi.org/10.1007/s00112-009-2073-z

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Schlüsselwörter

  • Akutes Leberversagen
  • Gerinnungsstörung
  • Enzephalopathie
  • Lebertransplantation
  • Hirnödem

Keywords

  • Acute liver failure
  • Coagulopathy
  • Encephalopathy
  • Liver transplantation
  • Cerebral edema