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| Name | Class |
|---|---|
| Vitaflo International, Ltd | INDUSTRY |
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Phenylketonuria (PKU) is an inherited inborn error of phenylalanine (PHE) metabolism caused by decreased activity of phenylalanine hydroxylase (PAH) enzyme. Therefore, PHE accumulates in plasma leading to mental problems. Treatment is a phenylalanine-restricted diet with sufficient protein. However, the optimum protein requirements are still unknown and compliance with diet is not satisfactory in PKU adults. A Previously established technique called indicator amino acid oxidation (IAAO) will be used to determine protein requirements from amino acid based formula vs. glycomacropeptide (GMP) in adults with PKU (≥ 19y). This study will help treat adults with enough protein ensuring maintenance of health.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Protein intake | Experimental | Free amino acids vs. Glycomacropeptide (GMP) |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Free amino acids intake | Dietary Supplement | Oral consumption of eight hourly experimental meals- -4 tracer free experimental meals containing a mixture of free amino acids and calories from protein free flavoured liquid, protein free cookies and corn oil -4 isotopically labeled experimental meals. The same protocol will be repeated with glycomacropeptide (GMP). |
| Measure | Description | Time Frame |
|---|---|---|
| 13 Co2 production | Breath samples will be collected during the study to measure the rate of oxidation of tracer in the expired breath. | 8 hours (1 study day). 3 samples will collected as a baseline prior to isotope protocol and 6 samples after 2 hours and 30 minutes of starting the tracer protocol. Data will be reported an average of 2 years. |
| Lysine flux | Urine samples will be collected during the study to measure the flux enrichment in urine. | 8 hours (1 study day). 1 sample will collected as a baseline prior to isotope protocol and 2 samples after 2 hours and 30 minutes of starting the tracer protocol. Data will be reported an average of 2 years. |
| Measure | Description | Time Frame |
|---|---|---|
| Phenylalanine concentrations | Phenylalanine concentrations will be measured in blood. | One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years. |
| Tyrosine concentrations |
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Inclusion Criteria:
-Adults more than 19 years of age who are diagnosed with PKU and clinically stable with no acute illness
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Rajavel Elango, PhD | BC Children's Hospital Research Institute, University of British Columbia | Principal Investigator |
| Sandra Sirrs, MD | University of British Columbia | Study Chair |
| Sylvia Stockler, MD | University of British Columbia | Study Chair |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| BC Children's Hospital Research Institute, University of British Columbia | Vancouver | British Columbia | V5Z4H4 | Canada |
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| ID | Term |
|---|---|
| D010661 | Phenylketonurias |
| ID | Term |
|---|---|
| D020739 | Brain Diseases, Metabolic, Inborn |
| D001928 | Brain Diseases, Metabolic |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
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| ID | Term |
|---|---|
| C065989 | caseinomacropeptide |
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Tyrosine concentrations will be measured in blood.
| One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years. |
| 16 other amino acids | 16 other amino acids will be measured in blood. | One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years. |
| D009422 | Nervous System Diseases |
| D000592 | Amino Acid Metabolism, Inborn Errors |
| D008661 | Metabolism, Inborn Errors |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |