Regular monitoring of clinical and nutritional status to determine adequacy of nutrient intake, response to treatment, and to guide modifications in the nutrition prescription is central to management of individuals with UCDs.

TABLE #2, Monitoring the Nutritional Management of an Individual with UCD when Well delineates the biomarkers recommended for routine monitoring in individuals with UCDs, as well as other biochemical parameters that may be assessed in specific clinical circumstances. These recommendations acknowledge that practice may vary based on clinical judgment and logistical considerations, such as laboratory access and insurance coverage.