[Allogenic liver transplantation: a form of "gene therapy" in metabolic diseases. Munich results and a review].

Abstract

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.

• Authors: M J Stangl, U Beuers, R Schauer, T Lang, A Gerbes, J Briegel, L Da Silva, F W Schildberg, H G Rau
• Journal: Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen (Chirurg) Vol. 71 Issue 7 Pg. 808-19 (Jul 2000) ISSN: 0009-4722 [Print] Germany
• Vernacular Title: Die allogene Lebertransplantation--eine Form der "Gentherapie" bei metabolischen Erkrankungen. Münchener Ergebnisse und Ubersicht.
• PMID: 10986603 (Publication Type: Comparative Study, English Abstract, Journal Article, Review)

Topics

• Adult
• Child
• Crigler-Najjar Syndrome (diagnosis, genetics, surgery)
• Genetic Therapy
• Glycogen Storage Disease (diagnosis, surgery)
• Hemochromatosis (diagnosis, genetics, surgery)
• Hemophilia A (diagnosis, surgery)
• Hepatolenticular Degeneration (diagnosis, genetics, surgery)
• Humans
• Hyperlipoproteinemia Type II (diagnosis, surgery)
• Hyperoxaluria (diagnosis, surgery)
• Infant, Newborn
• Liver Diseases (diagnosis, genetics, surgery)
• Liver Transplantation (methods, mortality)
• Metabolism, Inborn Errors (diagnosis, genetics, surgery)
• Middle Aged
• Time Factors
• Transplantation, Homologous
• Tyrosinemias (diagnosis, surgery)
• alpha 1-Antitrypsin Deficiency (diagnosis, genetics, surgery)