Conduct a cross-sectional analysis of the nutritional status of individuals with PKU treated with pegvaliase for one or more years or who have followed an unrestricted diet for 6 or more months while on pegvaliase treatment. We will evaluate laboratory markers of nutritional status, including markers of protein and micronutrient nutriture. In addition, a three-day food record and food frequency questionnaire will be obtained to assess protein intake and overall diet quality.
Study Type
OBSERVATIONAL
Enrollment
18
Treated with pegvaliase for one or more years or followed an unrestricted diet for 6 or more months while being treated with pegvaliase.
Boston Children's Hospital
Boston, Massachusetts, United States
Mean concentrations of serum marker(s) of protein status
Time frame: through study completion, an average of 1 year
Mean concentrations of serum marker(s) of micronutrient status
Time frame: through study completion, an average of 1 year
Mean concentrations of serum marker(s) of essential fatty acid status
Time frame: through study completion, an average of 1 year
Mean concentrations of serum marker(s) of cardiovascular status
Time frame: through study completion, an average of 1 year
Mean protein intake (g/day) as reported on a three-day food record
Time frame: through study completion, an average of 1 year
Mean micronutrient intake (%DRI) as reported on a three-day food record
Time frame: through study completion, an average of 1 year
Mean Healthy Eating Index-2015 scores calculated from the Diet History Questionnaire III
"The Healthy Eating Index-2015 (HEI-2015) is… used to assess how well a set of foods aligns with the 2015-2020 Dietary Guidelines for Americans. The HEI-2015 includes 13 components that can be summed to a maximum total score of 100 points. The components capture… adequacy components and… moderation components. For the adequacy components, higher scores reflect higher intakes that meet or exceed the standards. For the moderation components, higher scores reflect lower intakes because lower intakes are more desirable. A higher total score indicates a diet that aligns better with the Dietary Guidelines." - https://www.cnpp.usda.gov/sites/default/files/healthy\_eating\_index/HEI2015-ScoresForAmericans.pdf Adequacy components include total fruits, whole fruits, total vegetables, greens and beans, whole grains, dairy, total protein foods, seafood and plant proteins, fatty acids. Moderation components include refined grains, sodium, added sugars, and saturated fats.
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Time frame: through study completion, an average of 1 year
Correlation between serum micronutrient concentrations and average micronutrient intake
Time frame: through study completion, an average of 1 year