Fig 1.
Schedule of enrolment, interventions, and assessments.
I/E criteria = Inclusion and exclusion criteria. FAs = fatty acids. PN = Parenteral nutrition. a. Record known bowel length/type from last surgical resection or other measurement. b. Blood pressure, pulse rate, respiratory rate and temperature. c. Includes height or length, head circumference (for subjects < 36 months), and weight. If a physical examination or nutrition assessment was performed during the 3-day screening period, it will be used for the baseline assessment when available. d. Includes chemistry (including fat-soluble Vitamins A, D, E, and K), hematology, coagulation and C-reactive protein (CRP). Clinical laboratory evaluations obtained at time of screening will serve as the baseline assessment. e. 72-hour fecal collection will occur in the 72 hours preceding treatment day 1 and the final clinic visit day 90. f. Samples of the lipid profile will include cholesterol, high-density lipoprotein (HDL), very low-density lipoprotein (VLDL), and low-density lipoprotein (LDL). Results of samples obtained at time of screening will be analyzed during enrollment period, prior to participant receiving device. g. Samples of total plasma fatty acids will include total saturated, total monosaturated, total polyunsaturated, total Omega-3, total Omega 6, total fatty acids, a-linolenic Acid, linoleic acid, oleic acid, EPA, arachidonic acid, mead acid, DHA, and triene:tetraene ratio. h. Serum alanine aminotransferase (ALT), serum aspartate aminotransferase (AST), and serum gamma-glutamyl transpeptidase (GGT). i. 24-hour nutritional intake/composition (PO and enteral dietary intake and PN volume) will be recorded using a phone-based application or paper diary. This will be especially important during the 72-hour stool collection periods for calculation of the coefficient of fat absorption. j. Adverse events will be recorded daily in an electronic or paper format by the subject’s parent or guardian, and by study staff during clinic visits. Monitoring of these events will also occur through a daily phone call from a study coordinator. If any AEs or concerns are noted by the family, the study coordinator will alert the study team by email. PI or other delegated investigators will determine next steps.
Fig 2.
Nutritional support adjustments based on weight changes.
Additional considerations include other growth parameters, laboratory evaluations (if available), overall clinical status, intake/output ratio, and enteral tolerance. Figure created with Biorender.com.