char = C;code = 67
char = o;code = 111
char = g;code = 103
char = n;code = 110
char = i;code = 105
char = t;code = 116
char = i;code = 105
char = v;code = 118
char = e;code = 101
char = ;code = 32
char = f;code = 102
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char = c;code = 99
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = s;code = 115
char = ;code = 32
char = i;code = 105
char = n;code = 110
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 = w;code = 119
char = i;code = 105
char = t;code = 116
char = h;code = 104
char = ;code = 32
char = p;code = 112
char = h;code = 104
char = e;code = 101
char = n;code = 110
char = y;code = 121
char = l;code = 108
char = k;code = 107
char = e;code = 101
char = t;code = 116
char = o;code = 111
char = n;code = 110
char = u;code = 117
char = r;code = 114
char = i;code = 105
char = a;code = 97
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = l;code = 108
char = o;code = 111
char = n;code = 110
char = g;code = 103
char = -;code = 45
char = t;code = 116
char = e;code = 101
char = r;code = 114
char = m;code = 109
char = ;code = 32
char = t;code = 116
char = r;code = 114
char = e;code = 101
char = a;code = 97
char = t;code = 116
char = m;code = 109
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = ;code = 32
char = w;code = 119
char = i;code = 105
char = t;code = 116
char = h;code = 104
char = ;code = 32
char = t;code = 116
char = e;code = 101
char = t;code = 116
char = r;code = 114
char = a;code = 97
char = h;code = 104
char = y;code = 121
char = d;code = 100
char = r;code = 114
char = o;code = 111
char = b;code = 98
char = i;code = 105
char = o;code = 111
char = p;code = 112
char = t;code = 116
char = e;code = 101
char = r;code = 114
char = i;code = 105
char = n;code = 110
char = .;code = 46
result sql = Cognitive functions in patients with phenylketonuria in long-term treatment with tetrahydrobiopterin.
Cognitive functions in patients with phenylketonuria in long-term treatment with tetrahydrobiopterin.
char = C;code = 67
char = o;code = 111
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char = i;code = 105
char = v;code = 118
char = e;code = 101
char = ;code = 32
char = f;code = 102
char = u;code = 117
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char = c;code = 99
char = t;code = 116
char = i;code = 105
char = o;code = 111
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char = ;code = 32
char = w;code = 119
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char = e;code = 101
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char = s;code = 115
char = s;code = 115
char = e;code = 101
char = s;code = 115
char = s;code = 115
char = e;code = 101
char = d;code = 100
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char = n;code = 110
char = ;code = 32
char = 9;code = 57
char = ;code = 32
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char = s;code = 115
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char = w;code = 119
char = i;code = 105
char = t;code = 116
char = h;code = 104
char = ;code = 32
char = m;code = 109
char = i;code = 105
char = l;code = 108
char = d;code = 100
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char = t;code = 116
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char = m;code = 109
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char = e;code = 101
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char = y;code = 121
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char = k;code = 107
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char = u;code = 117
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char = a;code = 97
char = ;code = 32
char = (;code = 40
char = P;code = 80
char = K;code = 75
char = U;code = 85
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char = ;code = 32
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char = f;code = 102
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char = o;code = 111
char = m;code = 109
char = ;code = 32
char = 6;code = 54
char = ;code = 32
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char = o;code = 111
char = ;code = 32
char = 1;code = 49
char = 8;code = 56
char = ;code = 32
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char = a;code = 97
char = r;code = 114
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char = ;code = 32
char = o;code = 111
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char = ;code = 32
char = a;code = 97
char = g;code = 103
char = e;code = 101
char = ,;code = 44
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char = o;code = 111
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char = e;code = 101
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char = i;code = 105
char = n;code = 110
char = ;code = 32
char = l;code = 108
char = o;code = 111
char = n;code = 110
char = g;code = 103
char = -;code = 45
char = t;code = 116
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char = m;code = 109
char = ;code = 32
char = t;code = 116
char = r;code = 114
char = e;code = 101
char = a;code = 97
char = t;code = 116
char = m;code = 109
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = ;code = 32
char = (;code = 40
char = >;code = 62
char = 5;code = 53
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char = /;code = 47
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char = a;code = 97
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char = y;code = 121
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char = b;code = 98
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char = o;code = 111
char = p;code = 112
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char = e;code = 101
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char = n;code = 110
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char = (;code = 40
char = B;code = 66
char = H;code = 72
char = 4;code = 52
char = );code = 41
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char = c;code = 99
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char = s;code = 115
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char = e;code = 101
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char = u;code = 117
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char = e;code = 101
char = ,;code = 44
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char = e;code = 101
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char = S;code = 83
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char = i;code = 105
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char = k;code = 107
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char = I;code = 73
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char = .;code = 46
char = ;code = 32
char = A;code = 65
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char = i;code = 105
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char = e;code = 101
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char = s;code = 115
char = t;code = 116
char = u;code = 117
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char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = c;code = 99
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char = g;code = 103
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char = t;code = 116
char = i;code = 105
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char = e;code = 101
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char = l;code = 108
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char = c;code = 99
char = e;code = 101
char = ;code = 32
char = q;code = 113
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char = i;code = 105
char = e;code = 101
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char = t;code = 116
char = ;code = 32
char = (;code = 40
char = I;code = 73
char = Q;code = 81
char = );code = 41
char = ,;code = 44
char = ;code = 32
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char = o;code = 111
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char = a;code = 97
char = l;code = 108
char = ,;code = 44
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char = a;code = 97
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char = m;code = 109
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char = m;code = 109
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char = o;code = 111
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char = ,;code = 44
char = ;code = 32
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char = e;code = 101
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char = ;code = 32
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char = t;code = 116
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char = a;code = 97
char = l;code = 108
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char = f;code = 102
char = u;code = 117
char = n;code = 110
char = c;code = 99
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = s;code = 115
char = );code = 41
char = ;code = 32
char = w;code = 119
char = a;code = 97
char = s;code = 115
char = ;code = 32
char = c;code = 99
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char = n;code = 110
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char = ;code = 32
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char = ;code = 32
char = A;code = 65
char = D;code = 68
char = H;code = 72
char = D;code = 68
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char = E;code = 69
char = F;code = 70
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char = d;code = 100
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char = f;code = 102
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char = P;code = 80
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char = U;code = 85
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char = H;code = 72
char = 4;code = 52
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char = u;code = 117
char = d;code = 100
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char = ;code = 32
char = T;code = 84
char = h;code = 104
char = e;code = 101
char = s;code = 115
char = e;code = 101
char = ;code = 32
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char = s;code = 115
char = u;code = 117
char = l;code = 108
char = t;code = 116
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char = ;code = 32
char = h;code = 104
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char = w;code = 119
char = e;code = 101
char = v;code = 118
char = e;code = 101
char = r;code = 114
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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
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = t;code = 116
char = e;code = 101
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char = p;code = 112
char = r;code = 114
char = e;code = 101
char = t;code = 116
char = e;code = 101
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char = ;code = 32
char = w;code = 119
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char = h;code = 104
char = ;code = 32
char = c;code = 99
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char = u;code = 117
char = t;code = 116
char = i;code = 105
char = o;code = 111
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char = .;code = 46
char = ;code = 32
char = I;code = 73
char = t;code = 116
char = ;code = 32
char = i;code = 105
char = s;code = 115
char = ;code = 32
char = n;code = 110
char = e;code = 101
char = c;code = 99
char = e;code = 101
char = s;code = 115
char = s;code = 115
char = a;code = 97
char = r;code = 114
char = y;code = 121
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
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char = d;code = 100
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char = c;code = 99
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char = u;code = 117
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char = t;code = 116
char = h;code = 104
char = e;code = 101
char = r;code = 114
char = ;code = 32
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char = u;code = 117
char = d;code = 100
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char = e;code = 101
char = s;code = 115
char = ;code = 32
char = w;code = 119
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char = t;code = 116
char = h;code = 104
char = ;code = 32
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char = r;code = 114
char = ;code = 32
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char = m;code = 109
char = b;code = 98
char = e;code = 101
char = r;code = 114
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
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char = a;code = 97
char = t;code = 116
char = i;code = 105
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char = n;code = 110
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char = s;code = 115
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char = ;code = 32
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char = o;code = 111
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char = s;code = 115
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char = s;code = 115
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char = t;code = 116
char = i;code = 105
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char = e;code = 101
char = ;code = 32
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char = e;code = 101
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char = t;code = 116
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char = ;code = 32
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char = o;code = 111
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char = ;code = 32
char = f;code = 102
char = u;code = 117
char = n;code = 110
char = c;code = 99
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = u;code = 117
char = s;code = 115
char = i;code = 105
char = n;code = 110
char = g;code = 103
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = f;code = 102
char = o;code = 111
char = r;code = 114
char = m;code = 109
char = u;code = 117
char = l;code = 108
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 = f;code = 102
char = ;code = 32
char = B;code = 66
char = H;code = 72
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char = ;code = 32
char = t;code = 116
char = h;code = 104
char = a;code = 97
char = t;code = 116
char = ;code = 32
char = i;code = 105
char = s;code = 115
char = ;code = 32
char = c;code = 99
char = u;code = 117
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char = r;code = 114
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = l;code = 108
char = y;code = 121
char = ;code = 32
char = a;code = 97
char = v;code = 118
char = a;code = 97
char = i;code = 105
char = l;code = 108
char = a;code = 97
char = b;code = 98
char = l;code = 108
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result sql = Cognitive functions were assessed in 9 patients with mild to moderate phenylketonuria (PKU) ranging from 6 to 18 years of age, who were in long-term treatment (>5 years) with 5-9 mg/kg/day tetrahydrobiopterin (BH4) on compassionate use, provided by Schircks Inc. An extensive study of cognitive functions (intelligence quotient (IQ), visuospatial, visual memory, fine motor, executive and attentional functions) was conducted, and behavior was assessed using the ADHD Rating Scale and the Behavior Rating Inventory of Executive Function (BRIEF). All patients had normal IQ (M=107, SD=10). The most notable area of impairment was fine motor function, but no significant difference was found between the PKU patients in BH4 treatment who participated in the current study and PKU patients in dietary treatment who participated in a previous study. These results, however, should be interpreted with caution. It is necessary to conduct further studies with a larger number of patients, using more sensitive tests of motor function and using the formulation of BH4 that is currently available.

Publication: Mol. Genet. Metab. 2010 ;99 Suppl 1:S75-8. :doi: 10.1016/j.ymgme.2009.10.187.
Publication Date: 2010
Study Author(s): Gassió, R;Vilaseca, M A;Lambruschini, N;Boix, C;Fusté, M E;Campistol, J;
Institution: Neuropediatrics Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Spain. [email protected]
Shortcut link to this study:
Cognitive functions were assessed in 9 patients with mild to moderate phenylketonuria (PKU) ranging from 6 to 18 years of age, who were in long-term treatment (>5 years) with 5-9 mg/kg/day tetrahydrobiopterin (BH4) on compassionate use, provided by Schircks Inc. An extensive study of cognitive functions (intelligence quotient (IQ), visuospatial, visual memory, fine motor, executive and attentional functions) was conducted, and behavior was assessed using the ADHD Rating Scale and the Behavior Rating Inventory of Executive Function (BRIEF). All patients had normal IQ (M=107, SD=10). The most notable area of impairment was fine motor function, but no significant difference was found between the PKU patients in BH4 treatment who participated in the current study and PKU patients in dietary treatment who participated in a previous study. These results, however, should be interpreted with caution. It is necessary to conduct further studies with a larger number of patients, using more sensitive tests of motor function and using the formulation of BH4 that is currently available.
PMID: 20123475

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