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result sql = Results of liver transplantation for familial amyloid polyneuropathy type I in Brazil.
Results of liver transplantation for familial amyloid polyneuropathy type I in Brazil.
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char = r;code = 114
char = y;code = 121
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = r;code = 114
char = o;code = 111
char = m;code = 109
char = b;code = 98
char = o;code = 111
char = s;code = 115
char = i;code = 105
char = s;code = 115
char = ;code = 32
char = (;code = 40
char = n;code = 110
char = ;code = 32
char = =;code = 61
char = ;code = 32
char = 3;code = 51
char = );code = 41
char = .;code = 46
char = ;code = 32
char = O;code = 79
char = v;code = 118
char = e;code = 101
char = r;code = 114
char = a;code = 97
char = l;code = 108
char = l;code = 108
char = ;code = 32
char = 1;code = 49
char = -;code = 45
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = 5;code = 53
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char = y;code = 121
char = e;code = 101
char = a;code = 97
char = r;code = 114
char = ;code = 32
char = s;code = 115
char = u;code = 117
char = r;code = 114
char = v;code = 118
char = i;code = 105
char = v;code = 118
char = a;code = 97
char = l;code = 108
char = ;code = 32
char = r;code = 114
char = a;code = 97
char = t;code = 116
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = w;code = 119
char = e;code = 101
char = r;code = 114
char = e;code = 101
char = ;code = 32
char = 7;code = 55
char = 0;code = 48
char = %;code = 37
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = 5;code = 53
char = 8;code = 56
char = %;code = 37
char = ,;code = 44
char = ;code = 32
char = r;code = 114
char = e;code = 101
char = s;code = 115
char = p;code = 112
char = e;code = 101
char = c;code = 99
char = t;code = 116
char = i;code = 105
char = v;code = 118
char = e;code = 101
char = l;code = 108
char = y;code = 121
char = .;code = 46
char = ;code = 32
char = M;code = 77
char = o;code = 111
char = s;code = 115
char = t;code = 116
char = ;code = 32
char = p;code = 112
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = s;code = 115
char = ;code = 32
char = h;code = 104
char = a;code = 97
char = d;code = 100
char = ;code = 32
char = s;code = 115
char = t;code = 116
char = a;code = 97
char = b;code = 98
char = i;code = 105
char = l;code = 108
char = i;code = 105
char = z;code = 122
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = ;code = 32
char = o;code = 111
char = r;code = 114
char = ;code = 32
char = i;code = 105
char = m;code = 109
char = p;code = 112
char = r;code = 114
char = o;code = 111
char = v;code = 118
char = e;code = 101
char = m;code = 109
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = s;code = 115
char = y;code = 121
char = m;code = 109
char = p;code = 112
char = t;code = 116
char = o;code = 111
char = m;code = 109
char = s;code = 115
char = ;code = 32
char = a;code = 97
char = f;code = 102
char = t;code = 116
char = e;code = 101
char = r;code = 114
char = ;code = 32
char = a;code = 97
char = ;code = 32
char = m;code = 109
char = e;code = 101
char = d;code = 100
char = i;code = 105
char = a;code = 97
char = n;code = 110
char = ;code = 32
char = f;code = 102
char = o;code = 111
char = l;code = 108
char = l;code = 108
char = o;code = 111
char = w;code = 119
char = -;code = 45
char = u;code = 117
char = p;code = 112
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = 3;code = 51
char = 6;code = 54
char = ;code = 32
char = m;code = 109
char = o;code = 111
char = n;code = 110
char = t;code = 116
char = h;code = 104
char = s;code = 115
char = ;code = 32
char = (;code = 40
char = r;code = 114
char = a;code = 97
char = n;code = 110
char = g;code = 103
char = e;code = 101
char = ,;code = 44
char = ;code = 32
char = 1;code = 49
char = 4;code = 52
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = 8;code = 56
char = 2;code = 50
char = ;code = 32
char = m;code = 109
char = o;code = 111
char = n;code = 110
char = t;code = 116
char = h;code = 104
char = s;code = 115
char = );code = 41
char = .;code = 46
char = ;code = 32
char = S;code = 83
char = u;code = 117
char = r;code = 114
char = v;code = 118
char = i;code = 105
char = v;code = 118
char = o;code = 111
char = r;code = 114
char = s;code = 115
char = ;code = 32
char = h;code = 104
char = a;code = 97
char = d;code = 100
char = ;code = 32
char = a;code = 97
char = ;code = 32
char = s;code = 115
char = h;code = 104
char = o;code = 111
char = r;code = 114
char = t;code = 116
char = e;code = 101
char = r;code = 114
char = ;code = 32
char = d;code = 100
char = i;code = 105
char = s;code = 115
char = e;code = 101
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = d;code = 100
char = u;code = 117
char = r;code = 114
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = ;code = 32
char = b;code = 98
char = e;code = 101
char = f;code = 102
char = o;code = 111
char = r;code = 114
char = e;code = 101
char = ;code = 32
char = L;code = 76
char = T;code = 84
char = ;code = 32
char = (;code = 40
char = m;code = 109
char = e;code = 101
char = d;code = 100
char = i;code = 105
char = a;code = 97
char = n;code = 110
char = ,;code = 44
char = ;code = 32
char = 6;code = 54
char = ;code = 32
char = y;code = 121
char = e;code = 101
char = a;code = 97
char = r;code = 114
char = s;code = 115
char = ;;code = 59
char = ;code = 32
char = r;code = 114
char = a;code = 97
char = n;code = 110
char = g;code = 103
char = e;code = 101
char = ,;code = 44
char = ;code = 32
char = 2;code = 50
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = 1;code = 49
char = 7;code = 55
char = ;code = 32
char = y;code = 121
char = e;code = 101
char = a;code = 97
char = r;code = 114
char = s;code = 115
char = ;code = 32
char = v;code = 118
char = ;code = 32
char = 9;code = 57
char = ;code = 32
char = y;code = 121
char = e;code = 101
char = a;code = 97
char = r;code = 114
char = s;code = 115
char = ;;code = 59
char = ;code = 32
char = r;code = 114
char = a;code = 97
char = n;code = 110
char = g;code = 103
char = e;code = 101
char = ,;code = 44
char = ;code = 32
char = 7;code = 55
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = 1;code = 49
char = 2;code = 50
char = ;code = 32
char = y;code = 121
char = e;code = 101
char = a;code = 97
char = r;code = 114
char = s;code = 115
char = ;;code = 59
char = ;code = 32
char = P;code = 80
char = ;code = 32
char = =;code = 61
char = .;code = 46
char = 0;code = 48
char = 2;code = 50
char = );code = 41
char = ,;code = 44
char = ;code = 32
char = g;code = 103
char = r;code = 114
char = e;code = 101
char = a;code = 97
char = t;code = 116
char = e;code = 101
char = r;code = 114
char = ;code = 32
char = a;code = 97
char = l;code = 108
char = b;code = 98
char = u;code = 117
char = m;code = 109
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = l;code = 108
char = e;code = 101
char = v;code = 118
char = e;code = 101
char = l;code = 108
char = s;code = 115
char = ;code = 32
char = (;code = 40
char = m;code = 109
char = e;code = 101
char = d;code = 100
char = i;code = 105
char = a;code = 97
char = n;code = 110
char = ,;code = 44
char = ;code = 32
char = 4;code = 52
char = ;code = 32
char = g;code = 103
char = /;code = 47
char = d;code = 100
char = L;code = 76
char = ;;code = 59
char = ;code = 32
char = r;code = 114
char = a;code = 97
char = n;code = 110
char = g;code = 103
char = e;code = 101
char = ,;code = 44
char = ;code = 32
char = 3;code = 51
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = 4;code = 52
char = .;code = 46
char = 7;code = 55
char = ;code = 32
char = g;code = 103
char = /;code = 47
char = d;code = 100
char = L;code = 76
char = ;code = 32
char = v;code = 118
char = ;code = 32
char = 3;code = 51
char = .;code = 46
char = 6;code = 54
char = ;code = 32
char = g;code = 103
char = /;code = 47
char = d;code = 100
char = L;code = 76
char = ;;code = 59
char = ;code = 32
char = r;code = 114
char = a;code = 97
char = n;code = 110
char = g;code = 103
char = e;code = 101
char = ,;code = 44
char = ;code = 32
char = 2;code = 50
char = .;code = 46
char = 6;code = 54
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = 4;code = 52
char = .;code = 46
char = 1;code = 49
char = ;code = 32
char = g;code = 103
char = /;code = 47
char = d;code = 100
char = L;code = 76
char = ;;code = 59
char = ;code = 32
char = P;code = 80
char = ;code = 32
char = =;code = 61
char = .;code = 46
char = 0;code = 48
char = 3;code = 51
char = );code = 41
char = ,;code = 44
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = g;code = 103
char = r;code = 114
char = e;code = 101
char = a;code = 97
char = t;code = 116
char = e;code = 101
char = r;code = 114
char = ;code = 32
char = m;code = 109
char = o;code = 111
char = d;code = 100
char = i;code = 105
char = f;code = 102
char = i;code = 105
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = b;code = 98
char = o;code = 111
char = d;code = 100
char = y;code = 121
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char = m;code = 109
char = a;code = 97
char = s;code = 115
char = s;code = 115
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = d;code = 100
char = e;code = 101
char = x;code = 120
char = ;code = 32
char = s;code = 115
char = c;code = 99
char = o;code = 111
char = r;code = 114
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = (;code = 40
char = m;code = 109
char = e;code = 101
char = d;code = 100
char = i;code = 105
char = a;code = 97
char = n;code = 110
char = ,;code = 44
char = ;code = 32
char = 7;code = 55
char = 3;code = 51
char = 5;code = 53
char = ;;code = 59
char = ;code = 32
char = r;code = 114
char = a;code = 97
char = n;code = 110
char = g;code = 103
char = e;code = 101
char = ,;code = 44
char = ;code = 32
char = 5;code = 53
char = 0;code = 48
char = 2;code = 50
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = 1;code = 49
char = ,;code = 44
char = 4;code = 52
char = 3;code = 51
char = 2;code = 50
char = ;code = 32
char = v;code = 118
char = ;code = 32
char = 6;code = 54
char = 5;code = 53
char = 9;code = 57
char = ;;code = 59
char = ;code = 32
char = r;code = 114
char = a;code = 97
char = n;code = 110
char = g;code = 103
char = e;code = 101
char = ,;code = 44
char = ;code = 32
char = 4;code = 52
char = 1;code = 49
char = 1;code = 49
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = 8;code = 56
char = 0;code = 48
char = 3;code = 51
char = ;;code = 59
char = ;code = 32
char = P;code = 80
char = ;code = 32
char = =;code = 61
char = .;code = 46
char = 0;code = 48
char = 4;code = 52
char = );code = 41
char = ;code = 32
char = c;code = 99
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char = m;code = 109
char = p;code = 112
char = a;code = 97
char = r;code = 114
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = w;code = 119
char = i;code = 105
char = t;code = 116
char = h;code = 104
char = ;code = 32
char = n;code = 110
char = o;code = 111
char = n;code = 110
char = s;code = 115
char = u;code = 117
char = r;code = 114
char = v;code = 118
char = i;code = 105
char = v;code = 118
char = o;code = 111
char = r;code = 114
char = s;code = 115
char = .;code = 46
char = ;code = 32
char = H;code = 72
char = o;code = 111
char = w;code = 119
char = e;code = 101
char = v;code = 118
char = e;code = 101
char = r;code = 114
char = ,;code = 44
char = ;code = 32
char = o;code = 111
char = n;code = 110
char = l;code = 108
char = y;code = 121
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char = d;code = 100
char = i;code = 105
char = s;code = 115
char = e;code = 101
char = a;code = 97
char = s;code = 115
char = e;code = 101
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char = d;code = 100
char = u;code = 117
char = r;code = 114
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
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char = ;code = 32
char = a;code = 97
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char = d;code = 100
char = ;code = 32
char = a;code = 97
char = l;code = 108
char = b;code = 98
char = u;code = 117
char = m;code = 109
char = i;code = 105
char = n;code = 110
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char = l;code = 108
char = e;code = 101
char = v;code = 118
char = e;code = 101
char = l;code = 108
char = s;code = 115
char = ;code = 32
char = w;code = 119
char = e;code = 101
char = r;code = 114
char = e;code = 101
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char = i;code = 105
char = n;code = 110
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char = e;code = 101
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char = e;code = 101
char = n;code = 110
char = d;code = 100
char = e;code = 101
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char = l;code = 108
char = y;code = 121
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char = a;code = 97
char = s;code = 115
char = s;code = 115
char = o;code = 111
char = c;code = 99
char = i;code = 105
char = a;code = 97
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char = e;code = 101
char = d;code = 100
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char = w;code = 119
char = i;code = 105
char = t;code = 116
char = h;code = 104
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char = s;code = 115
char = u;code = 117
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char = a;code = 97
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char = y;code = 121
char = s;code = 115
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char = s;code = 115
char = .;code = 46
char = ;code = 32
char = I;code = 73
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char = c;code = 99
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char = i;code = 105
char = o;code = 111
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char = ,;code = 44
char = ;code = 32
char = L;code = 76
char = T;code = 84
char = ;code = 32
char = i;code = 105
char = s;code = 115
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char = a;code = 97
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char = f;code = 102
char = f;code = 102
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char = e;code = 101
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char = h;code = 104
char = e;code = 101
char = r;code = 114
char = a;code = 97
char = p;code = 112
char = y;code = 121
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char = f;code = 102
char = o;code = 111
char = r;code = 114
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char = F;code = 70
char = A;code = 65
char = P;code = 80
char = -;code = 45
char = I;code = 73
char = .;code = 46
char = ;code = 32
char = M;code = 77
char = o;code = 111
char = r;code = 114
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char = a;code = 97
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char = i;code = 105
char = t;code = 116
char = y;code = 121
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char = a;code = 97
char = f;code = 102
char = t;code = 116
char = e;code = 101
char = r;code = 114
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char = s;code = 115
char = u;code = 117
char = r;code = 114
char = g;code = 103
char = e;code = 101
char = r;code = 114
char = y;code = 121
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char = i;code = 105
char = s;code = 115
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char = a;code = 97
char = s;code = 115
char = s;code = 115
char = o;code = 111
char = c;code = 99
char = i;code = 105
char = a;code = 97
char = t;code = 116
char = e;code = 101
char = d;code = 100
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char = w;code = 119
char = i;code = 105
char = t;code = 116
char = h;code = 104
char = ;code = 32
char = p;code = 112
char = o;code = 111
char = o;code = 111
char = r;code = 114
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char = n;code = 110
char = u;code = 117
char = t;code = 116
char = r;code = 114
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char = t;code = 116
char = i;code = 105
char = o;code = 111
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char = a;code = 97
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char = s;code = 115
char = t;code = 116
char = a;code = 97
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char = u;code = 117
char = s;code = 115
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char = a;code = 97
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char = d;code = 100
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char = l;code = 108
char = o;code = 111
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char = g;code = 103
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char = s;code = 115
char = t;code = 116
char = a;code = 97
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char = d;code = 100
char = i;code = 105
char = n;code = 110
char = g;code = 103
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char = d;code = 100
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char = s;code = 115
char = e;code = 101
char = a;code = 97
char = s;code = 115
char = e;code = 101
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char = b;code = 98
char = e;code = 101
char = f;code = 102
char = o;code = 111
char = r;code = 114
char = e;code = 101
char = ;code = 32
char = L;code = 76
char = T;code = 84
char = .;code = 46
char = ;code = 32
char = T;code = 84
char = h;code = 104
char = u;code = 117
char = s;code = 115
char = ,;code = 44
char = ;code = 32
char = L;code = 76
char = T;code = 84
char = ;code = 32
char = s;code = 115
char = h;code = 104
char = o;code = 111
char = u;code = 117
char = l;code = 108
char = d;code = 100
char = ;code = 32
char = b;code = 98
char = e;code = 101
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char = p;code = 112
char = e;code = 101
char = r;code = 114
char = f;code = 102
char = o;code = 111
char = r;code = 114
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char = e;code = 101
char = d;code = 100
char = ;code = 32
char = a;code = 97
char = s;code = 115
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char = e;code = 101
char = a;code = 97
char = r;code = 114
char = l;code = 108
char = y;code = 121
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char = a;code = 97
char = s;code = 115
char = ;code = 32
char = p;code = 112
char = o;code = 111
char = s;code = 115
char = s;code = 115
char = i;code = 105
char = b;code = 98
char = l;code = 108
char = e;code = 101
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char = a;code = 97
char = f;code = 102
char = t;code = 116
char = e;code = 101
char = r;code = 114
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result sql = Familial amyloid polyneuropathy type I (FAP-I) is an inherited amyloidosis secondary to systemic deposition of amyloid fibrils containing mutant transthyretin (TTR) variants. The disease has a progressive clinical course and is usually fatal 10 years after its onset. TTR is mainly produced in hepatocytes, and liver transplantation (LT) has been proposed as an effective treatment for FAP-I. The aim of this study is to evaluate the results of LT for FAP-I in Brazil and analyze prognostic factors associated with survival after surgery. Twenty-four patients (median age, 36 years; range, 25 to 52 years) who underwent LT with the diagnosis of FAP-I were evaluated. Surgery was uneventful in all but six patients who died of complications of primary liver nonfunction (n = 1), cardiogenic shock (n = 1), sepsis (n = 3), and hepatic artery thrombosis (n = 3). Overall 1- and 5-year survival rates were 70% and 58%, respectively. Most patients had stabilization or improvement of symptoms after a median follow-up of 36 months (range, 14 to 82 months). Survivors had a shorter disease duration before LT (median, 6 years; range, 2 to 17 years v 9 years; range, 7 to 12 years; P =.02), greater albumin levels (median, 4 g/dL; range, 3 to 4.7 g/dL v 3.6 g/dL; range, 2.6 to 4.1 g/dL; P =.03), and greater modified body mass index scores (median, 735; range, 502 to 1,432 v 659; range, 411 to 803; P =.04) compared with nonsurvivors. However, only disease duration and albumin levels were independently associated with survival in multivariate analysis. In conclusion, LT is an effective therapy for FAP-I. Mortality after surgery is associated with poor nutritional status and long-standing disease before LT. Thus, LT should be performed as early as possible after the onset of FAP-I symptoms to avoid major disability and improve survival.

Publication: Liver Transpl. 2002 Jan ;8(1)::34-9.
Publication Date: 2002 Jan
Study Author(s): Bittencourt, Paulo Lisboa;Couto, Claudia Alves;Farias, Alberto Queiroz;Marchiori, Paulo;Bosco Massarollo, Paulo Celso;Mies, Sérgio;
Institution: Liver Unit, University of São Paulo School of Medicine, Brazil. [email protected]
Shortcut link to this study:
Familial amyloid polyneuropathy type I (FAP-I) is an inherited amyloidosis secondary to systemic deposition of amyloid fibrils containing mutant transthyretin (TTR) variants. The disease has a progressive clinical course and is usually fatal 10 years after its onset. TTR is mainly produced in hepatocytes, and liver transplantation (LT) has been proposed as an effective treatment for FAP-I. The aim of this study is to evaluate the results of LT for FAP-I in Brazil and analyze prognostic factors associated with survival after surgery. Twenty-four patients (median age, 36 years; range, 25 to 52 years) who underwent LT with the diagnosis of FAP-I were evaluated. Surgery was uneventful in all but six patients who died of complications of primary liver nonfunction (n = 1), cardiogenic shock (n = 1), sepsis (n = 3), and hepatic artery thrombosis (n = 3). Overall 1- and 5-year survival rates were 70% and 58%, respectively. Most patients had stabilization or improvement of symptoms after a median follow-up of 36 months (range, 14 to 82 months). Survivors had a shorter disease duration before LT (median, 6 years; range, 2 to 17 years v 9 years; range, 7 to 12 years; P =.02), greater albumin levels (median, 4 g/dL; range, 3 to 4.7 g/dL v 3.6 g/dL; range, 2.6 to 4.1 g/dL; P =.03), and greater modified body mass index scores (median, 735; range, 502 to 1,432 v 659; range, 411 to 803; P =.04) compared with nonsurvivors. However, only disease duration and albumin levels were independently associated with survival in multivariate analysis. In conclusion, LT is an effective therapy for FAP-I. Mortality after surgery is associated with poor nutritional status and long-standing disease before LT. Thus, LT should be performed as early as possible after the onset of FAP-I symptoms to avoid major disability and improve survival.
PMID: 11799483

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