Protein Requirements in Phenylketonuria (PKU) Patients Compared Using PKU Sphere™ | Glycomacropeptide (GMP) and an L-amino Acid-based Product
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 6
- 试验地点
- 2
- 主要终点
- 13 Co2 production
研究概览
简要总结
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.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Supportive Care
- 盲法
- None
入排标准
- 年龄范围
- 19 Years 至 50 Years（Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Adults more than 19 years of age who are diagnosed with PKU and clinically stable with no acute illness
排除标准
- •Adults with PKU under age 19 year
- •Adults diagnosed with PKU but are currently ill with a fever or cold
结局指标
主要结局
13 Co2 production
时间窗: 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.
Breath samples will be collected during the study to measure the rate of oxidation of tracer in the expired breath.
Lysine flux
时间窗: 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.
Urine samples will be collected during the study to measure the flux enrichment in urine.
次要结局
- 16 other amino acids(One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.)
- Phenylalanine concentrations(One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.)
- Tyrosine concentrations(One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.)
研究者
Rajavel Elango, PhD
Associate Professor
University of British Columbia