Nutrition management of urea cycle disorders (UCDs) focuses on limiting dietary protein and providing adequate energy to ensure appropriate growth and prevent catabolism. Dietary protein intakes are recommended to patients; however, assessment of actual protein intake is challenging as standard assessment methods are recognized to be inaccurate, time-consuming, and cumbersome. A new dietary collection method utilizes a smartphone camera; participants take photos of meals and snacks for remote analysis by a registered dietitian. This food photography method using the mFood app was compared to traditional 3-day diet records in individuals with UCDs and reported energy intake for both were validated against an objective gold-standard measurement of total energy expenditure (TEE) by doubly labeled water. In weight-stable adults, energy intake is approximately equal to TEE. Eight individuals ≥16 years old participated in a randomized crossover design study. In this cohort, protein intake was 13 % and 15 % of energy for 3-day diet records and mFood, respectively. There was no statistical difference in gram intake of the macronutrients between the two methods. Compared to TEE, participants reported 16 % lower total energy intake by 3-day diet records and 22 % lower energy intake by mFood, demonstrating limited benefit to using mFood. We highlight the importance of utilizing consistent nutrient analysis methods to compare dietary assessments within a population. mFood, although not superior to traditional dietary collection methods, was preferred by the majority of participants and offers a novel method for collecting nutrition information in individuals with UCD who frequently find this task cumbersome.
Keywords: Dietary intake; Doubly labeled water; Food photography; Urea cycle disorder.
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