melatonin has been researched along with BH4 Deficiency in 13 studies
| Excerpt | Relevance | Reference |
|---|---|---|
| "Melatonin levels were not increased with the higher dose of Trp supplementation, but dopamine levels were increased with the higher dose of Tyr supplementation." | 2.79 | Melatonin and dopamine as biomarkers to optimize treatment in phenylketonuria: effects of tryptophan and tyrosine supplementation. ( Azen, C; Moseley, K; Yano, S, 2014) |
| "We aimed to study the prevalence of sleep disorders in early-treated PKU children and adolescents and assessed correlations with dopamine and serotonin status." | 1.51 | Prevalence of sleep disorders in early-treated phenylketonuric children and adolescents. Correlation with dopamine and serotonin status. ( Artuch, R; Campistol, J; Cuadras, D; Gassió, R; González, MJ; Ormazabal, A; Sans, O; Sierra, C, 2019) |
| "Conventional treatment of BH4 deficiency, i." | 1.30 | Hyperprolactinemia, a tool in treatment control of tetrahydrobiopterin deficiency: endocrine studies in an affected girl. ( Bieglmayer, C; Birnbacher, R; Blau, N; Frisch, H; Scheibenreiter, S; Waldhauser, F, 1998) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 4 (30.77) | 18.7374 |
| 1990's | 1 (7.69) | 18.2507 |
| 2000's | 2 (15.38) | 29.6817 |
| 2010's | 6 (46.15) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Gassió, R | 1 |
| González, MJ | 1 |
| Sans, O | 1 |
| Artuch, R | 1 |
| Sierra, C | 1 |
| Ormazabal, A | 1 |
| Cuadras, D | 1 |
| Campistol, J | 1 |
| Ney, DM | 1 |
| Murali, SG | 1 |
| Stroup, BM | 1 |
| Nair, N | 1 |
| Sawin, EA | 1 |
| Rohr, F | 1 |
| Levy, HL | 2 |
| Yano, S | 3 |
| Moseley, K | 3 |
| Azen, C | 3 |
| Fu, X | 1 |
| Cederbaum, S | 1 |
| Martinez-Cruz, F | 2 |
| Pozo, D | 1 |
| Osuna, C | 2 |
| Espinar, A | 1 |
| Marchante, C | 1 |
| Guerrero, JM | 2 |
| WOOLLEY, DW | 2 |
| VANDERHOEVEN, T | 1 |
| Birnbacher, R | 1 |
| Scheibenreiter, S | 1 |
| Blau, N | 1 |
| Bieglmayer, C | 1 |
| Frisch, H | 1 |
| Waldhauser, F | 1 |
| Butcher, RE | 1 |
| Vorhees, CV | 1 |
| Kindt, CW | 1 |
| Kazmaier-Novak, KJ | 1 |
| Berry, HK | 1 |
| Polidora, VJ | 1 |
| Van der Hoeven, T | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Phase 2 Study of Glycomacropeptide vs. Amino Acid Diet for the Management of PKU[NCT01428258] | 32 participants (Actual) | Interventional | 2011-09-30 | Completed | |||
| Pilot Study to Evaluate Melatonin Secretion as a Marker of Decreased Serotonin in Individuals With PKU: Evaluation of the CNS Effects of Tetrahydrobiopterin[NCT01617070] | Phase 4 | 10 participants (Actual) | Interventional | 2012-05-31 | Completed | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
Plasma concentration of BSAP was determined as a measure of bone turnover. (NCT01428258)
Timeframe: day 22 of each dietary treatment
| Intervention | micro gram per liter (Mean) |
|---|---|
| GMP Diet/GMP Medical Foods | 17.0 |
| AA Diet/AA Medical Foods | 17.0 |
Plasma will be collected at each base week and after 3 weeks on each of the dietary treatments, glycomacropeptide and amino acid, following an overnight fast. Plasma phenylalanine concentration (along with the complete profile of free amino acids) will be determined with an amino acid analyzer in the Wisconsin State Lab of Hygiene. Statistical analysis to determine the significance of the change in plasma phe concentration when comparing the 2 diets will consist of ANCOVA with covariates for baseline Phe and dietary Phe intake. The change in plasma Phe concentration from day 22 (final) to day 1 (baseline) was determined after adjusting for baseline Phe level and dietary Phe intake. (NCT01428258)
Timeframe: baseline to day 22 on each diet
| Intervention | micro moles per liter plasma (Mean) |
|---|---|
| GMP Diet/GMP Medical Foods | 62 |
| AA Diet/AA Medical Foods | -85 |
Concentrations of Phe in plasma and in dried blood spots collected simultaneously by subjects will be compared using 2 methodologies, regardless of intervention. At each of the 4 study visits (baseline and final for each dietary treatment): 1) venipuncture was used to collect blood and plasma was isolated and analyzed for Phe with ion exchange chromatography and 2) subjects were asked right after the venipuncture to spot their blood on filter paper for analysis of Phe with tandem mass spectroscopy (MS/MS). The discrepancy in Phe concentrations with these 2 methods was compared for each sample pair using Bland-Altman statistical analysis. Each subject should have had 4 sample pairs, 29 x 4 = 116, but we ended up with only 110 sample pairs, as explained below. (NCT01428258)
Timeframe: 4 times total, 2 per treatment
| Intervention | micro moles per liter (Mean) |
|---|---|
| Phe Concentration in Plasma, Ion Exchange Chromatography | 731 |
| Phe Concentration in Dried Blood Spots, Tandem Mass Spec | 514 |
Compliance with the glycomacropeptide and amino acid dietary treatments will be assessed by comparison of the intake of medical food in grams of protein from medical food per day based on subject completion of 3-day food records prior to the final study visit on day 22. Statistical analysis for a dietary treatment effect will consist of ANOVA. (NCT01428258)
Timeframe: 3 week dietary treatment
| Intervention | g protein from MF/kg/day (Mean) |
|---|---|
| GMP Diet | 0.74 |
| AA Diet/AA Medical Foods | 0.76 |
Completion of a standardized test, the Behavior Rating Inventory of Executive Function (BRIEF), by each subject for the GMP diet and the AA diet. Values are T-scores which have a mean of 50 points and a SD of 10 points. A T score of <50 is considered within the normative range. Data are analyzed with a paired t-test. (NCT01428258)
Timeframe: day 22 of each dietary treatment
| Intervention | T score (Mean) |
|---|---|
| GMP Diet/GMP Medical Foods | 49.0 |
| AA Diet/AA Medical Foods | 48.8 |
Plasma concentration of NTX was determined as a measure of bone resorption; higher levels indicate greater bone breakdown (NCT01428258)
Timeframe: day 22 of each dietary treatment
| Intervention | nmol per liter bone collagen equivalents (Mean) |
|---|---|
| GMP Diet/GMP Medical Foods | 17.5 |
| AA Diet/AA Medical Foods | 17.1 |
Vitamin D was measured as a measure of the capacity for calcium absorption. Higher levels of plasma vitamin D are consistent with higher calcium absorption. (NCT01428258)
Timeframe: day 22 of each dietary treatment
| Intervention | ng per ml (Mean) |
|---|---|
| GMP Diet/GMP Medical Foods | 33.8 |
| AA Diet/AA Medical Foods | 33.6 |
Evaluated for each subject under 4 conditions (washout, LNAA only, Kuvan only and LNAA+Kuvan) (NCT01617070)
Timeframe: measured every 4 weeks up to 16 weeks
| Intervention | pg/ml (Mean) |
|---|---|
| LNAA | 266.9 |
| Washout | 205.7 |
| BH4 (Kuvan) | 220.4 |
| BH4 and LNAA | 301.2 |
Evaluated for each subject under 4 conditions (washout, LNAA only, Kuvan only and LNAA+Kuvan) (NCT01617070)
Timeframe: measured every 4 weeks up to 16 weeks
| Intervention | ng/mg Creatinine (Mean) |
|---|---|
| LNAA | 14.5 |
| Washout | 8.2 |
| BH4 (Kuvan) | 8.6 |
| BH4 and LNAA | 13.2 |
Evaluated for each subject under 4 conditions (washout, LNAA only, Kuvan only and LNAA+Kuvan) (NCT01617070)
Timeframe: measured every 4 weeks up to 16 weeks
| Intervention | ug/gCreatinine (Mean) |
|---|---|
| LNAA | 63.7 |
| Washout | 42.6 |
| BH4 (Kuvan) | 46.0 |
| BH4 and LNAA | 67.6 |
3 trials available for melatonin and BH4 Deficiency
| Article | Year |
|---|---|
Metabolomic changes demonstrate reduced bioavailability of tyrosine and altered metabolism of tryptophan via the kynurenine pathway with ingestion of medical foods in phenylketonuria.
Topics: Adolescent; Adult; Amino Acids; Biological Availability; Caseins; Catecholamines; Cross-Over Studies | 2017 |
Melatonin and dopamine as biomarkers to optimize treatment in phenylketonuria: effects of tryptophan and tyrosine supplementation.
Topics: Adult; Amino Acids, Neutral; Biomarkers; Chromatography, High Pressure Liquid; Cross-Over Studies; D | 2014 |
Large neutral amino acid supplementation increases melatonin synthesis in phenylketonuria: a new biomarker.
Topics: Adult; Aged; Amino Acids, Neutral; Biomarkers; Cross-Over Studies; Dietary Supplements; Double-Blind | 2013 |
10 other studies available for melatonin and BH4 Deficiency
| Article | Year |
|---|---|
Prevalence of sleep disorders in early-treated phenylketonuric children and adolescents. Correlation with dopamine and serotonin status.
Topics: Adolescent; Biomarkers; Child; Dopamine; Female; Humans; Male; Melatonin; Phenylketonurias; Prevalen | 2019 |
Evaluation of Tetrahydrobiopterin Therapy with Large Neutral Amino Acid Supplementation in Phenylketonuria: Effects on Potential Peripheral Biomarkers, Melatonin and Dopamine, for Brain Monoamine Neurotransmitters.
Topics: Adult; Amino Acids, Neutral; Biomarkers; Biopterins; Dietary Supplements; Dopamine; Drug Synergism; | 2016 |
Is melatonin synthesis a new biomarker for the pathogenesis and treatment of phenylketonuria?
Topics: Amino Acids, Neutral; Biomarkers; Dietary Supplements; Female; Humans; Male; Melatonin; Phenylketonu | 2013 |
Oxidative stress induced by phenylketonuria in the rat: Prevention by melatonin, vitamin E, and vitamin C.
Topics: Animals; Animals, Newborn; Antioxidants; Ascorbic Acid; Brain; Cerebellum; Disease Models, Animal; F | 2002 |
PREVENTION OF A MENTAL DEFECT OF PHENYLKETONURIA WITH SEROTONIN CONGENERS SUCH AS MELATONIN OR HYDROXYTRYPTOPHAN.
Topics: 5-Hydroxytryptophan; Animals; Biomedical Research; Indoles; Learning; Melatonin; Mice; Pharmacology; | 1964 |
Mitochondrial damage induced by fetal hyperphenylalaninemia in the rat brain and liver: its prevention by melatonin, Vitamin E, and Vitamin C.
Topics: Aldehydes; Animals; Animals, Newborn; Ascorbic Acid; Disease Models, Animal; Drug Interactions; Fema | 2006 |
Hyperprolactinemia, a tool in treatment control of tetrahydrobiopterin deficiency: endocrine studies in an affected girl.
Topics: Adolescent; Biopterins; Carbidopa; Delayed-Action Preparations; Drug Combinations; Female; Follicle | 1998 |
Induced PKU in rats: effects of age and melatonin treatment.
Topics: Aging; Animals; Animals, Newborn; Behavior, Animal; Body Weight; Female; Humans; Learning; Melatonin | 1977 |
Behavioral effects of "phenylketonuria" in rats.
Topics: Animals; Animals, Newborn; Behavior, Animal; Dogs; Female; Humans; Male; Melatonin; Phenylalanine; P | 1967 |
Serotonin deficiency in infancy as a cause of a mental defect in experimental phenylketonuria.
Topics: 5-Hydroxytryptophan; Animals; Animals, Newborn; Chlorpromazine; Electroshock; Female; Humans; In Vit | 1965 |