title: [Allogenic liver transplantation: a form of "gene therapy" in metabolic diseases. Munich results and a review].
abstract: BACKGROUND
  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.
authors.affiliation: Chirurgische Klinik und Poliklinik, Ludwig-Maximilians-Universität, Klinikum Grosshadern, München. Manfred.Stangl@gch.med.uni-muenchen.de
authors.forename: M J
authors.initials: MJ
authors.lastname: Stangl
authors.forename: U
authors.initials: U
authors.lastname: Beuers
authors.forename: R
authors.initials: R
authors.lastname: Schauer
authors.forename: T
authors.initials: T
authors.lastname: Lang
authors.forename: A
authors.initials: A
authors.lastname: Gerbes
authors.forename: J
authors.initials: J
authors.lastname: Briegel
authors.forename: L
authors.initials: L
authors.lastname: Da Silva
authors.forename: F W
authors.initials: FW
authors.lastname: Schildberg
authors.forename: H G
authors.initials: HG
authors.lastname: Rau
mesh_terms: D000328:Adult; D002648:Child; D003414:Crigler-Najjar Syndrome / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D015316:Genetic Therapy; D006008:Glycogen Storage Disease / Q000175:diagnosis / Q000601:surgery; D006432:Hemochromatosis / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D006467:Hemophilia A / Q000175:diagnosis / Q000601:surgery; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000175:diagnosis / Q000601:surgery; D006959:Hyperoxaluria / Q000175:diagnosis / Q000601:surgery; D007231:Infant, Newborn; D008107:Liver Diseases / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods / Q000401:mortality; D008661:Metabolism, Inborn Errors / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D008875:Middle Aged; D013997:Time Factors; D014184:Transplantation, Homologous; D020176:Tyrosinemias / Q000175:diagnosis / Q000601:surgery; D019896:alpha 1-Antitrypsin Deficiency / Q000175:diagnosis / Q000235:genetics / Q000601:surgery