phenylalanine and nitisinone

phenylalanine has been researched along with nitisinone in 17 studies

Research

Studies (17)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's1 (5.88)18.2507
2000's1 (5.88)29.6817
2010's10 (58.82)24.3611
2020's5 (29.41)2.80

Authors

AuthorsStudies
Hjalmarson, O; Holme, E; Lindstedt, S; Lock, EA; Strandvik, B1
Al-Dhalimy, M; Finegold, M; Grompe, M; Overturf, K1
Carton, D; Dan, B; De Laet, C; François, B; Goyens, PJ; Jaeken, J; Munoz, VT; Sokal, EM1
Boenzi, S; Della Bona, ML; Dionisi-Vici, C; la Marca, G; Malvagia, S; Martinelli, D; Materazzi, S1
Kassel, R; Rudnick, DA; Sprietsma, L1
Arnoux, JB; Brassier, A; de Lonlay, P; Dubois, S; Grisel, C; Job-Deslandre, C; Le Quan Sang, KH; Ranganath, L; Servais, A; Sireau, N; Wippf, J1
Huijbregts, SCJ; Jahja, R; van Ginkel, WG; van Spronsen, FJ1
Endo, F; Ito, M; Nakamura, K; Shigematsu, Y1
Das, AM; Meyer, U; van Rijn, M; van Spronsen, FJ1
Cox, TF; Davison, AS; Gallagher, JA; Hughes, AT; Khedr, M; Milan, AM; Psarelli, EE; Ranganath, LR; Rhodes, NP; Rovensky, J1
Bou-Gharios, G; Gallagher, JA; Hughes, AT; Hughes, JH; Jarvis, JC; Judd, S; Milan, AM; Ranganath, LR; Sutherland, H; Wilson, PJM1
Burgerhof, JGM; Daly, A; de Blaauw, P; Heiner-Fokkema, MR; Kienstra, NS; MacDonald, A; Rodenburg, IL; Santra, S; van de Krogt, J; van Ginkel, WG; van Reemst, HE; van Spronsen, FJ1
Bierau, J; Cassiman, D; Daly, A; De Laet, C; Eyskens, F; Gissen, P; Goyens, PJ; Huijbregts, SCJ; Jahja, R; Jameson, EA; MacDonald, A; McKiernan, PJ; Morris, AAM; Mumford, N; Rahman, Y; Timmer, C; van Ginkel, WG; van Hasselt, PM; van Spronsen, FJ; van Vliet, K; Vara, R; Wilcox, G1
Cooper, MS; Davison, AS; Deutz, NEP; Fitzgerald, R; Gallagher, JA; Ghane, P; Hughes, AT; Jarvis, JC; Khedr, M; Markinson, L; Milan, AM; Norman, BP; Psarelli, EE; Ranganath, LR; Sutherland, H1
Bärhold, F; Beblo, S; Bogovic, N; Dahl, SV; Das, AM; Dokoupil, K; Fekete, A; Fischer, A; Freisinger, P; Heddrich-Ellerbrok, M; Hochuli, M; Jörg-Streller, M; Korpel, V; Lahl, J; Lier, D; Mayorandan, S; Meyer, U; Möslinger, D; Neugebauer, AK; Och, U; Rohde, C; Rosenbaum-Fabian, S; Thimm, EM; van Teeffelen-Heithoff, A1
Arslan Gulten, Z; Arslan, N; Aydogan, A; Bahceci, H; Eroglu Erkmen, S; Karalar Pekuz, OK; Ozturk, T; Teke Kisa, P; Uysal, S; Yuce Inel, T1
Arnoux, JB; Bou-Gharios, G; Davison, AS; Gallagher, JA; Hughes, AT; Imrich, R; M, K; Milan, AM; Norman, BP; Olsson, B; Ranganath, LR; Rudebeck, M1

Reviews

4 review(s) available for phenylalanine and nitisinone

ArticleYear
Old treatments for new insights and strategies: proposed management in adults and children with alkaptonuria.
    Journal of inherited metabolic disease, 2015, Volume: 38, Issue:5

    Topics: Adult; Age Factors; Alkaptonuria; Antioxidants; Child; Cyclohexanones; Humans; Nitrobenzoates; Ochronosis; Phenylalanine; Tyrosine

2015
Neurological and Neuropsychological Problems in Tyrosinemia Type I Patients.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Humans; Nervous System Diseases; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2017
Dietary Considerations in Tyrosinemia Type I.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Diet; Humans; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2017
Efficacy of Phenylalanine- and Tyrosine-Restricted Diet in Alkaptonuria Patients on Nitisinone Treatment: Case Series and Review of Literature.
    Annals of nutrition & metabolism, 2022, Volume: 78, Issue:1

    Topics: Adult; Alkaptonuria; Cyclohexanones; Diet; Dietary Proteins; Female; Humans; Male; Middle Aged; Nitrobenzoates; Phenylalanine; Tyrosine

2022

Trials

2 trial(s) available for phenylalanine and nitisinone

ArticleYear
Neuropsychological outcome of NTBC-treated patients with tyrosinaemia type 1.
    Developmental medicine and child neurology, 2011, Volume: 53, Issue:10

    Topics: Adolescent; Belgium; Child; Child, Preschool; Cognition Disorders; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Intelligence; Intelligence Tests; Male; Neuropsychological Tests; Nitrobenzoates; Phenylalanine; Treatment Outcome; Tyrosine; Tyrosinemias

2011
Quantification of the flux of tyrosine pathway metabolites during nitisinone treatment of Alkaptonuria.
    Scientific reports, 2019, 07-11, Volume: 9, Issue:1

    Topics: Adult; Alkaptonuria; Cyclohexanones; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Phenylalanine; Pigments, Biological; Tandem Mass Spectrometry; Tyrosine

2019

Other Studies

11 other study(ies) available for phenylalanine and nitisinone

ArticleYear
Treatment of hereditary tyrosinaemia type I by inhibition of 4-hydroxyphenylpyruvate dioxygenase.
    Lancet (London, England), 1992, Oct-03, Volume: 340, Issue:8823

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Acetoacetates; alpha-Fetoproteins; Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Child; Child, Preschool; Cyclohexanones; Erythrocytes; Heptanoates; Humans; Hydroxybenzoates; Infant; Kidney Tubules; Liver; Nitrobenzoates; Phenylalanine; Phosphates; Porphobilinogen Synthase; Proteinuria; Tyrosine

1992
Long-term therapy with NTBC and tyrosine-restricted diet in a murine model of hereditary tyrosinemia type I.
    Molecular genetics and metabolism, 2002, Volume: 75, Issue:1

    Topics: Animals; Cyclohexanones; Diet; Disease Models, Animal; Enzyme Inhibitors; Hydrolases; Mice; Mice, Knockout; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2002
LC-MS/MS method for simultaneous determination on a dried blood spot of multiple analytes relevant for treatment monitoring in patients with tyrosinemia type I.
    Analytical chemistry, 2012, Jan-17, Volume: 84, Issue:2

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Biomarkers; Child; Child, Preschool; Chromatography, Liquid; Cyclohexanones; Diet, Protein-Restricted; Drug Monitoring; Enzyme Inhibitors; Heptanoates; Humans; Infant; Infant, Newborn; Kidney; Liver; Methionine; Nitrobenzoates; Phenylalanine; Tandem Mass Spectrometry; Tyrosine; Tyrosinemias

2012
Pregnancy in an NTBC-treated patient with hereditary tyrosinemia type I.
    Journal of pediatric gastroenterology and nutrition, 2015, Volume: 60, Issue:1

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Combined Modality Therapy; Contraindications; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Phenylalanine; Pregnancy; Pregnancy Complications; Term Birth; Tyrosine; Tyrosinemias

2015
Tyrosinemia Type I in Japan: A Report of Five Cases.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Child, Preschool; Cyclohexanones; Female; Humans; Infant; Japan; Liver; Liver Transplantation; Male; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2017
Dietary restriction of tyrosine and phenylalanine lowers tyrosinemia associated with nitisinone therapy of alkaptonuria.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:2

    Topics: Alkaptonuria; Animals; Cyclohexanones; Diet, Protein-Restricted; Female; Humans; Male; Mice; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2020
The Effect of Various Doses of Phenylalanine Supplementation on Blood Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients.
    Nutrients, 2019, Nov-18, Volume: 11, Issue:11

    Topics: Adolescent; Adult; Child; Cyclohexanones; Dietary Supplements; Enzyme Inhibitors; Female; Heptanoates; Humans; Linear Models; Male; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias; Young Adult

2019
Emotional and behavioral problems, quality of life and metabolic control in NTBC-treated Tyrosinemia type 1 patients.
    Orphanet journal of rare diseases, 2019, 12-04, Volume: 14, Issue:1

    Topics: Adolescent; Adult; Child; Cyclohexanones; Humans; Male; Nitrobenzoates; Phenylalanine; Quality of Life; Tyrosine; Tyrosinemias; Young Adult

2019
Nitisinone causes acquired tyrosinosis in alkaptonuria.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:5

    Topics: Adult; Aged; Alkaptonuria; Amino Acid Metabolism, Inborn Errors; Animals; Cyclohexanones; Female; Humans; Male; Mice; Mice, Inbred BALB C; Middle Aged; Nitrobenzoates; Phenylalanine; Tyrosine; Young Adult

2020
Hepatorenal Tyrosinaemia: Impact of a Simplified Diet on Metabolic Control and Clinical Outcome.
    Nutrients, 2020, Dec-31, Volume: 13, Issue:1

    Topics: Adolescent; Austria; Child; Child, Preschool; Combined Modality Therapy; Cyclohexanones; Diet, Protein-Restricted; Dietary Proteins; Enzyme Inhibitors; Female; Germany; Humans; Male; Nitrobenzoates; Patient Compliance; Phenylalanine; Practice Guidelines as Topic; Prospective Studies; Retrospective Studies; Surveys and Questionnaires; Switzerland; Treatment Outcome; Tyrosine; Tyrosinemias; Young Adult

2020
Determinants of tyrosinaemia during nitisinone therapy in alkaptonuria.
    Scientific reports, 2022, 09-27, Volume: 12, Issue:1

    Topics: Alkaptonuria; Brain Diseases, Metabolic, Inborn; Cyclohexanones; Homogentisic Acid; Humans; Nitrobenzoates; Phenylalanine; Phenylpropionates; Tyrosine; Tyrosinemias

2022