Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen · 2000
[Allogenic liver transplantation: a form of "gene therapy" in metabolic diseases. Munich results and a review].
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Stangl M J, Beuers U, Schauer R, Lang T, Gerbes A, Briegel J, Da Silva L, Schildberg F W, Rau H G
Аннотация
INTRODUCTION: Liver transplantation is the method of choice for metabolic diseases and end-stage liver failure.
METHODS: At the Klinikum Grosshadern we have performed liver transplantation for inborn errors of metabolism in 24 patients (5.3% of all transplantations, 16 adults, age 39 +/- 13 years; 8 children, age 9 +/- 3 years); 19 patients received a transplant for end-stage liver disease, and in 5 cases because of fulminant hepatic failure.
RESULTS: Twenty-four patients received 27 transplants. In 3 cases, a split-liver transplantation was performed; one patient received a combined lung-liver graft. The 5-year survival rate for children is 100% and for adults 68%.
CONCLUSIONS: Liver transplantation for inborn errors of metabolism not only replaces the diseased organ, but also leads to complete reversal of the metabolic defect.
METHODS: At the Klinikum Grosshadern we have performed liver transplantation for inborn errors of metabolism in 24 patients (5.3% of all transplantations, 16 adults, age 39 +/- 13 years; 8 children, age 9 +/- 3 years); 19 patients received a transplant for end-stage liver disease, and in 5 cases because of fulminant hepatic failure.
RESULTS: Twenty-four patients received 27 transplants. In 3 cases, a split-liver transplantation was performed; one patient received a combined lung-liver graft. The 5-year survival rate for children is 100% and for adults 68%.
CONCLUSIONS: Liver transplantation for inborn errors of metabolism not only replaces the diseased organ, but also leads to complete reversal of the metabolic defect.
Предметные рубрики MeSH
AdultChildCrigler-Najjar SyndromeGenetic TherapyGlycogen Storage DiseaseHemochromatosisHemophilia AHepatolenticular DegenerationHumansHyperlipoproteinemia Type IIHyperoxaluriaInfant, NewbornLiver DiseasesLiver TransplantationMetabolism, Inborn ErrorsMiddle AgedTime FactorsTransplantation, HomologousTyrosinemiasalpha 1-Antitrypsin Deficiency