Extracorporeal Perfusion
Fig. 9.1. Extracorporeal liver perfusion circuit (see text).

9.5) While all etiologies share common features of liver failure once an advanced stage is reached, unique aspects of each etiology influence management during and following transplantation.

Liver transplantation is also indicated for patients with certain metabolic diseases that can present with liver failure in the absence of cirrhosis.(Table 9.6) This is more common in the pediatric population, but can occasionally extend into young adulthood. Other congenital abnormalities (urea cycle enzyme deficiencies, familial hypercholesterolemia, familial amyloidosis) can present with extra-hepatic manifestations that are so severe that liver transplantation is recommended in the absence of hepatic disease. Finally, a miscellaneous group of chronic disorders may require transplantation in the absence of both cirrhosis and hepatic failure.

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