Long-term quantitative evaluation of liver transplantation in XXXX (Portuguese V30M).

@entity1 @entity14755 @entity3144 @entity7031 @entity20117 @entity204
@entity14755 ( @entity14755 ) is associated with massive @entity3144 of amyloid, which isformed from a mutated @entity20117 ( @entity20117 ) protein. Ninety percent of @entity20117 protein is produced in liver. Liver transplantation (LT) is the only treatment that can halt @entity14755 clinical progression. We studied 35 LT @entity1 . The mean age of the first symptoms was 36.6 years (ranging from 27 to 56), 19 were males, and 16 females, they underwent LT after a mean time of 5 years of symptomatic disease. Fifteen @entity1 followed for more than 24 months after LT had periodic evaluations with clinical and neurophysiological scores (CS and @entity7031 ). Ten were first evaluated before LT (mean follow-up time of 44 months after LT), and 5 were evaluated only after LT (or a mean time of 41 months). Five @entity1 were followed periodically before LT (mean time of 44 months) to study the natural course of this condition. The mortality rate was of 14% in the first 6 months and was related to known complications of the surgery. No @entity204 occurred in the period 6 months to 1 year after LT. Five @entity1 (14%) died 1-2 years after LT, 4 of whom were transplanted in advance stages. In the survival group, CS tended to stabilize shortly after LT and to remain invariable later on. The NSprogressed in the first year following LT, and subsequently it did not increase significantly. LT changed the natural course of @entity14755 -I.
