Management
Guidelines
Portal
PKU Nutrition Management Guidelines
First Edition
March 2015, v.1.12
Updated: August 2016
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Nutrition Assessment
Patient History

Optimal PKU treatment management requires onging assessment of clinical and nutritional status. Outcome measures from dietary history, anthropometrics, and clinical and laboratory assessment are used to determine appropriateness of therapy recommendations. A thorough medical and social history is also essential for understanding an individual's clinical needs and ability to effectiveluy adhere to therapy recommendations.

Anthropometrics

Evaluation includes: weight, height, head circumference, and weight for length (or weight for height or BMI) measurements.

See: http://www.cdc.gov/growthcharts/cdc_charts.htm and http://www.cdc.gov/growthcharts/who_charts.htm

Nutrition-focused Physical Findings

Evaluation includes observation of abnormal skin or hair characteristics, weight status, and gastrointestinal symptoms (constipation, diarrhea, vomiting).

Laboratory Findings, Medical Tests and Procedures

See: TABLE #1, Laboratory and Clinical Findings in PKU Laboratory and Clinical Findings for PKU.

Food and Nutrition-related History

A dietary history includes: history of food intake and adherence to recommendations, feeding difficulties (mechanical and behavioral or psychological), pharmacological treatment and adherence, and socio-economic factors affecting adherence (family composition and economic or language barriers). Ongoing evaluation for adequacy and effectiveness of the most recent dietary therapy prescription includes: PHE and TYR intake, medical food consumption (type, amount, and nutrient content), and total nutrient intake. Total nutrient intake includes protein (intact and medical food protein), energy, vitamins and minerals, fluid, and essential fatty acids and DHA.

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