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char = l;code = 108
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char = ;code = 32
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char = u;code = 117
char = r;code = 114
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char = a;code = 97
char = ;code = 32
char = r;code = 114
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char = c;code = 99
char = e;code = 101
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char = i;code = 105
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char = o;code = 111
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char = r;code = 114
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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 = 46
result sql = Long-term follow-up of patients with phenylketonuria receiving tetrahydrobiopterin treatment.
Long-term follow-up of patients with phenylketonuria receiving tetrahydrobiopterin treatment.
char = T;code = 84
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char = t;code = 116
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char = o;code = 111
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char = l;code = 108
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char = e;code = 101
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char = ;code = 32
char = l;code = 108
char = e;code = 101
char = v;code = 118
char = e;code = 101
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char = s;code = 115
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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
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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
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char = B;code = 66
char = H;code = 72
char = (;code = 40
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char = );code = 41
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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
char = );code = 41
char = .;code = 46
char = ;code = 32
char = A;code = 65
char = ;code = 32
char = n;code = 110
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char = f;code = 102
char = ;code = 32
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char = d;code = 100
char = i;code = 105
char = e;code = 101
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char = a;code = 97
char = s;code = 115
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char = r;code = 114
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char = e;code = 101
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char = t;code = 116
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char = m;code = 109
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char = H;code = 72
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char = t;code = 116
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char = e;code = 101
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char = ;code = 32
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char = i;code = 105
char = e;code = 101
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char = t;code = 116
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 = P;code = 80
char = K;code = 75
char = U;code = 85
char = ,;code = 44
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char = b;code = 98
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char = ;code = 32
char = l;code = 108
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char = a;code = 97
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char = a;code = 97
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char = e;code = 101
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char = l;code = 108
char = a;code = 97
char = c;code = 99
char = k;code = 107
char = i;code = 105
char = n;code = 110
char = g;code = 103
char = .;code = 46
char = ;code = 32
char = H;code = 72
char = e;code = 101
char = r;code = 114
char = e;code = 101
char = ,;code = 44
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char = w;code = 119
char = e;code = 101
char = ;code = 32
char = d;code = 100
char = e;code = 101
char = s;code = 115
char = c;code = 99
char = r;code = 114
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char = b;code = 98
char = e;code = 101
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char = t;code = 116
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = e;code = 101
char = f;code = 102
char = f;code = 102
char = e;code = 101
char = c;code = 99
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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 = m;code = 109
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char = w;code = 119
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char = B;code = 66
char = H;code = 72
char = (;code = 40
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char = );code = 41
char = ;code = 32
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char = 1;code = 49
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char = ;code = 32
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char = h;code = 104
char = o;code = 111
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char = e;code = 101
char = d;code = 100
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char = a;code = 97
char = ;code = 32
char = >;code = 62
char = 2;code = 50
char = 8;code = 56
char = %;code = 37
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char = r;code = 114
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char = d;code = 100
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char = c;code = 99
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char = i;code = 105
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char = l;code = 108
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char = i;code = 105
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char = g;code = 103
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char = ;code = 32
char = B;code = 66
char = H;code = 72
char = (;code = 40
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char = );code = 41
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result sql = Treatment with tetrahydrobiopterin (BH(4)), the natural cofactor of phenylalanine hydroxylase (PAH), can reduce blood phenylalanine (Phe) levels in patients with BH(4)-responsive phenylketonuria (PKU). A number of studies has reported on the short-term BH(4) treatment of patients with PKU, but long-term data are lacking. Here, we describe the effects of long-term treatment with BH(4) on 16 patients, who showed a >28% reduction in blood Phe following testing for BH(4) overload. The mean dose of BH(4) was 16 mg/kg body weight (range 5-36 mg/kg body weight). The mean treatment duration was 56 months (range 24-110 months). Of 16 patients, 14 achieved long-term Phe control with BH(4) treatment, with a mean blood Phe concentration of 321 +/- 236 micromol/l. The mean decrease from baseline in blood Phe levels in these 14 patients was 54.6%. Of the seven patients who required continued dietary restriction, Phe intake increased from 200-300 mg/day to 800-1000 mg/day. Factors that may cause fluctuation of Phe levels in BH(4)-treated patients include patients' PAH genotype, Phe intake, changes in protein catabolism or anabolism, and periods of illness or infection.

Publication: J. Inherit. Metab. Dis. 2010 Mar 9 ;:.
Publication Date: 2010 Mar 9
Study Author(s): Trefz, Friedrich K;Scheible, D;Frauendienst-Egger, G;
Institution: Klinik für Kinder und Jungendmedizin, Reutlingen, Klinik am Steinenberg, Steinenbergstrasse 31, Reutlingen, 72764, Germany, [email protected].
Shortcut link to this study:
Treatment with tetrahydrobiopterin (BH(4)), the natural cofactor of phenylalanine hydroxylase (PAH), can reduce blood phenylalanine (Phe) levels in patients with BH(4)-responsive phenylketonuria (PKU). A number of studies has reported on the short-term BH(4) treatment of patients with PKU, but long-term data are lacking. Here, we describe the effects of long-term treatment with BH(4) on 16 patients, who showed a >28% reduction in blood Phe following testing for BH(4) overload. The mean dose of BH(4) was 16 mg/kg body weight (range 5-36 mg/kg body weight). The mean treatment duration was 56 months (range 24-110 months). Of 16 patients, 14 achieved long-term Phe control with BH(4) treatment, with a mean blood Phe concentration of 321 +/- 236 micromol/l. The mean decrease from baseline in blood Phe levels in these 14 patients was 54.6%. Of the seven patients who required continued dietary restriction, Phe intake increased from 200-300 mg/day to 800-1000 mg/day. Factors that may cause fluctuation of Phe levels in BH(4)-treated patients include patients' PAH genotype, Phe intake, changes in protein catabolism or anabolism, and periods of illness or infection.
PMID: 20217238

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