1 | (S)-2-amino-6-(2-hydroxypropanoyl)-7,8-dihydropteridin-4(3H)-one | - | - | - | - | [1] 240 240 |
2 | 5,6,7,8-tetrahydrobiopterin | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
3 | 6R-BH4 | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [3] 86 86, 124, 240 |
4 | A16AX07 | - | - | - | - | [1] 240 240 |
5 | Amino acids | [1] Amino acids Amino acids | - | - | - | [3] 70 70, 240, 298 |
6 | Behavioral: Neuro-psychological Assessment | - | - | - | - | [1] 240 240 |
7 | Behavioral: phenylalanine restricted diet | [1] Phenylalanine Phenylalanine | [1] D00021
D00021
| - | - | [1] 240 240 |
8 | Behavioral: Restricted phenylalanine diet | [1] Phenylalanine Phenylalanine | [1] D00021
D00021
| - | - | [1] 240 240 |
9 | BH4 | - | - | - | - | [5] 51 51, 86, 124, 240, 299 |
10 | Blood samples | - | - | - | - | [9] 13 13, 41, 46, 51, 61, 97, 227, 240, 271 |
11 | BMN 165 | - | - | - | - | [1] 240 240 |
12 | BMN 165 (rAvPAL-PEG) | [1] Pegvaliase Pegvaliase | [1] D11077
D11077
| [1] PAH PAH 💬 | [5] Biosynthesis of amino acids Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬 | [1] 240 240 |
13 | BMN 307 | - | - | - | - | [1] 240 240 |
14 | BMN165 20mg/day | - | - | - | - | [1] 240 240 |
15 | BMN165 40mg/day | - | - | - | - | [1] 240 240 |
16 | CDX | - | - | - | - | [2] 222 222, 240 |
17 | CDX 6114 | - | - | - | - | [1] 240 240 |
18 | CNSA-001 | - | - | - | - | [1] 240 240 |
19 | Cohort 1 0.225g | - | - | - | - | [1] 240 240 |
20 | Cohort 2 0.75g | - | - | - | - | [1] 240 240 |
21 | Cohort 3 2.25 g | - | - | - | - | [1] 240 240 |
22 | Corn | [1] Corn Corn | - | - | - | [2] 46 46, 240 |
23 | Corn/soy oil | [1] Corn Corn | - | - | - | [1] 240 240 |
24 | Diagnostic Test: Whole body Phe oxidation testing | - | - | - | - | [1] 240 240 |
25 | Diet | - | - | - | - | [3] 97 97, 240, 299 |
26 | Dietary Supplement for PKU patients | - | - | - | - | [1] 240 240 |
27 | Docosahexaenoic acid | [1] Doconexent Doconexent | - | - | - | [7] 6 6, 49, 90, 94, 240, 299, 301 |
28 | Free amino acids intake | [1] Amino acids Amino acids | - | - | - | [1] 240 240 |
29 | Glycomacropeptide-based protein substitute | - | - | - | - | [1] 240 240 |
30 | Hepatocyte Transplant | - | - | - | - | [1] 240 240 |
31 | High oleic sunflower oil | [1] Sunflower oil Sunflower oil | [1] D09006
D09006
| - | - | [1] 240 240 |
32 | HMI-103 | - | - | - | - | [1] 240 240 |
33 | Immunosuppression | - | - | - | - | [1] 240 240 |
34 | KUVAN | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
35 | Kuvan 100mg soluble tablet | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
36 | KuvanTM Therapy | - | - | - | - | [1] 240 240 |
37 | Kuvan® | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
38 | Kuvan® (sapropterin dihydrochloride) | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
39 | Large Neutral Amino Acid Therapy | - | - | - | - | [1] 240 240 |
40 | Liver biopsy | - | - | - | - | [4] 93 93, 171, 231, 240 |
41 | Low calorie protein substitute | - | - | - | - | [1] 240 240 |
42 | Medikinet | - | - | - | - | [1] 240 240 |
43 | Microalgal oil | - | - | - | - | [1] 240 240 |
44 | Moxifloxacin | [1] Moxifloxacin Moxifloxacin | [2] D00874
D00874
,
D08237
| - | - | [9] 2 2, 6, 46, 49, 85, 86, 113, 240, 299 |
45 | N.a. | - | - | - | - | [10] 3 3, 6, 8, 13, 46, 96, 97, 240, 256, 271 |
46 | Nap | - | - | - | - | [2] 96 96, 240 |
47 | Other: Diet | - | - | - | - | [1] 240 240 |
48 | Other: Diet treatment | - | - | - | - | [1] 240 240 |
49 | Other: Liver Evaluation | - | - | - | - | [1] 240 240 |
50 | Other: Meal Challenge | - | - | - | - | [1] 240 240 |
51 | Other: Normal (control) diet | - | - | - | - | [1] 240 240 |
52 | Other: Phenylalanine (Phe)-restricted diet | [1] Phenylalanine Phenylalanine | [1] D00021
D00021
| - | - | [1] 240 240 |
53 | Other: Routine | - | - | - | - | [1] 240 240 |
54 | Palynziq | [1] Pegvaliase Pegvaliase | [1] D11077
D11077
| [1] PAH PAH 💬 | [5] Biosynthesis of amino acids Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬 | [1] 240 240 |
55 | PEG | - | - | - | - | [12] 6 6, 13, 41, 46, 65, 78, 96, 97, 140, 144, 240, 299 |
56 | PEGVALIASE | [1] Pegvaliase Pegvaliase | [1] D11077
D11077
| [1] PAH PAH 💬 | [5] Biosynthesis of amino acids Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬 | [1] 240 240 |
57 | Phe | - | - | - | - | [2] 75 75, 240 |
58 | Phenoptin | - | - | - | - | [2] 124 124, 240 |
59 | Phenylalanine | [1] Phenylalanine Phenylalanine | [1] D00021
D00021
| - | - | [1] 240 240 |
60 | Phenylalanine restricted diet | [1] Phenylalanine Phenylalanine | [1] D00021
D00021
| - | - | [1] 240 240 |
61 | Phenylalanine-free protein substitute in tablet form (XPhe minis) | [1] Phenylalanine Phenylalanine | [1] D00021
D00021
| - | - | [1] 240 240 |
62 | Phenylalanine-free protein substitute tablets | [1] Phenylalanine Phenylalanine | [1] D00021
D00021
| - | - | [1] 240 240 |
63 | Phenylketonuria-type diet | - | - | - | - | [1] 240 240 |
64 | PKU Explore | - | - | - | - | [1] 240 240 |
65 | PKU express plus | - | - | - | - | [1] 240 240 |
66 | PKU Sphere | - | - | - | - | [1] 240 240 |
67 | PKU sphere liquid | - | - | - | - | [1] 240 240 |
68 | PKU Start | - | - | - | - | [1] 240 240 |
69 | PKU/TYR GMP Protein Substitute | - | - | - | - | [1] 240 240 |
70 | Protein intake | - | - | - | - | [1] 240 240 |
71 | PTC923 | - | - | - | - | [1] 240 240 |
72 | Radiation: Preparative Radiation Therapy | - | - | - | - | [1] 240 240 |
73 | RAvPAL-PEG | [1] Pegvaliase Pegvaliase | [1] D11077
D11077
| [1] PAH PAH 💬 | [5] Biosynthesis of amino acids Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬 | [1] 240 240 |
74 | RAvPAL-PEG 0.001 mg/kg | [1] Pegvaliase Pegvaliase | [1] D11077
D11077
| [1] PAH PAH 💬 | [5] Biosynthesis of amino acids Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬 | [1] 240 240 |
75 | RAvPAL-PEG 0.003 mg/kg | [1] Pegvaliase Pegvaliase | [1] D11077
D11077
| [1] PAH PAH 💬 | [5] Biosynthesis of amino acids Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬 | [1] 240 240 |
76 | RAvPAL-PEG 0.01 mg/kg | [1] Pegvaliase Pegvaliase | [1] D11077
D11077
| [1] PAH PAH 💬 | [5] Biosynthesis of amino acids Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬 | [1] 240 240 |
77 | RAvPAL-PEG 0.03 mg/kg | [1] Pegvaliase Pegvaliase | [1] D11077
D11077
| [1] PAH PAH 💬 | [5] Biosynthesis of amino acids Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬 | [1] 240 240 |
78 | RAvPAL-PEG 0.1 mg/kg | [1] Pegvaliase Pegvaliase | [1] D11077
D11077
| [1] PAH PAH 💬 | [5] Biosynthesis of amino acids Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬 | [1] 240 240 |
79 | RTX | - | - | - | - | [5] 11 11, 46, 65, 222, 240 |
80 | RTX-134 | - | - | - | - | [1] 240 240 |
81 | Sapropterin | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [3] 86 86, 124, 240 |
82 | Sapropterin (Kuvan) | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
83 | SAPROPTERIN DIHYDROCHLORIDE | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
84 | Sapropterin dihydrochloride, 6R-BH4, tetrahydrobiopterin | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
85 | Sapropterin Dihydrocholoride | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
86 | SAPROPTERIN HYDROCHLORIDE | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
87 | Sapropterin, sapropterin dihydrochloride | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
88 | Sapropterin, sapropterin hydrochloride | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
89 | Sapropterindihydrochloride | - | - | - | - | [1] 240 240 |
90 | Saproterin dihydrochloride | - | - | - | - | [1] 240 240 |
91 | Sunflower oil | [1] Sunflower oil Sunflower oil | [1] D09006
D09006
| - | - | [3] 46 46, 240, 299 |
92 | SYNB1618 | - | - | - | - | [1] 240 240 |
93 | SYNB1934 | - | - | - | - | [1] 240 240 |
94 | Synergy | - | - | - | - | [1] 240 240 |
95 | T1401 | - | - | - | - | [1] 240 240 |
96 | Tetrahydrobiopterin | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |
97 | Tetrahydrobiopterin (BH4) | [1] Sapropterin Sapropterin | [1] D08505
D08505
| - | - | [1] 240 240 |