Monday, November 29, 2010

Type IV Glycogen Storage Disease

Liver transplantation eliminates cirrhosis and may even improve the degree of amylopectin storage in the heart. Careful follow-up is, however, necessary in this respec.
Hemophilia  
Four patients who had severe post-transfusion hepatitis cirrhosis underwent liver transplantation. In yhe three surviving patients, the plasma coagulating activity of factor VIII was normal, confirming that the liver is the main source of factor VIII synthesis and secretion.  


Protoporphyria 
Five adult and adolescent patients with cirrhosis underwent liver transplantation.

This resulted in normal liver function and disappearance of or significant improvement in skin photosensitivity. Careful follow-up remains necessary because of the impotant role of erythropoietic tissue in protoporphyrin production in such patients, which may result in relapsing accumulation of protoporphyrin in the transplanted liver.


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