Optimal PROP management requires ongoing assessment of clinical and nutritional status. Outcome measures from dietary history, anthropometrics, and clinical and laboratory assessment are used to determine appropriateness of current therapy recommendations.
A thorough medical and social history is essential for understanding an individual's clinical needs and ability to effectively adhere to therapy recommendations.
Anthropometric assessment includes: weight, height, head circumference, and weight for length (or weight for height) measurements to assess growth velocity for infants and children and body mass index for older patients and comparing to national and international standards
Assessment includes observation of skin and hair, odor, weight status, level of physical activity, muscle tone, and gastrointestinal symptoms (constipation, diarrhea, vomiting).
Laboratory assessment of nutritional status includes protein status, plasma amino acid concentrations and balance, and adequacy of those limited in the nutrition management of PROP (VAL, ILE, MET, THR); and indicators of PROP management include plasma acylcarnitine profile and plasma carnitine.
See detailed information in the Nutrition Recommendations section, Question 4: Monitoring and Table TABLE #7, Monitoring the Nutritional Management of Well/Stable Individuals with PROP: Monitoring the Nutritional Management of Well/Stable Individuals with PROP.
A dietary history includes: reported consumption from enteral and parenteral sources ((type, amount, and nutrient content), use of supplements, feeding schedule, oral eating ability, and adherence to recommendations and factors affecting adherence.
Assessment of nutrient intake includes protein (intact and medical food protein), energy, vitamins and minerals, fluid, and essential fatty acids and DHA.