ISSN:
1432-2277
Keywords:
Key words Familial amyloidosis
;
Inborn errors of metabolism
;
Liver transplantation
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract The purpose of the present study was to evaluate the impact of liver transplantation on familial amyloidotic polyneuropathy (FAP met-30) patients' survival. Forty-five FAP patients were involved in the study; 15 non-transplanted FAP patients and 30 liver-transplanted patients. All patients' records were scrutinised for information on disease duration. Preoperative nutritional status was evaluated in all patients. No difference in survival was observed for transplanted patients overall compared to historical controls. However, for cases in good nutritional status, an increased survival can be expected as a significantly increased mortality rate for malnourished patients was observed (P 〈 0.05). Increased survival has so far not been found for transplanted FAP patients. However, none of the transplanted cases has yet reached the expected survival time for non-transplanted FAP control patients, which is 14 years. A high fatality rate of malnourished patients transplanted late in the course of the disease contributed significantly to the mortality among transplanted patients.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s001470050451
Permalink
