Background: Home enteral nutrition (HEN) use continues to increase in children unable to meet nutritional needs through oral intake. Some patients do not tolerate standard polymeric formula (SPF), which may lead to malnutrition. Use of peptidebased diet (PBD) has demonstrated benefits in adults, however there remains a paucity of data in pediatric population. Methods: Retrospective review of medical records of children receiving HEN betweenOctober 2015 and October 2019 was conducted. Nutrition, tolerance, and healthcare utilization was tracked through May 2020. Children receiving PBD as initial formula or transitioned to PBD from SPF were included. Our objective was to assess gastrointestinal tolerance and impact on healthcare utilization in children receiving PBD.Results: During study period, 30 children (mean age, 9 ± 5.44 years; 20 of 30 [66.7%] male) utilized PBDs. Twenty-one patients started PBD directly with malnutrition as primary indication. Nine patients transitioned from SPF to PBD, most often due to intolerance of SPF (66%). After transition to PBD, no symptoms were reported in 6 of 9 (66.7%) patients, and symptoms of SPF intolerance resolved in 4 of 9 (44.5%) patients.Healthcare utilization declined significantly after transition to PBD, including mean numbers of emergency room visits (0.78 ± 1.09 to 0.11 ± 0.33; P = .025), provider visits

Objectives: Nutrition support is essential in improving outcome and survival in children on extra corporal membranous support (ECMO). We aim to evaluate the association between the timing of enteral nutrition (EN) initiation and its impact on outcome. Methods: We retrospectively reviewed the electronic health records of children (≤18 years) from November 2014 to November 2019 who were on veno-arterial ECMO for ≥48 hours. Abstracted data included demographics, ECMO indication and duration, timing of EN initiation, change in weight-for-age z score (WAZ), and survival rate. The vasoactive-inotropic score (VIS) was calculated to assess illness acuity. Results: We identified 76 children with median age (interquartile range [IQR]) of 0.3 years (0-2.6), 46 of which were infants (59%) who required ECMO for a median (IQR) of 10 days (6-22). Thirty-six (47%) survived to hospital discharge. EN was initiated in 55 (72%) of patients while on ECMO. EN initiation by day 3 of ECMO was positively associated with survival (P = 0.0438). VIS at the time of EN initiation was lower in surviving infants (P = 0.022). Children who achieved enteral autonomy were more likely to survive (P = 0.0024). Survivors had greater WAZs at ECMO completion (P = 0.0004). Conclusions: Initiation of EN by day 3 of ECMO and at a lower VIS is associated with greater likelihood of survival.

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Pancreas and gastrointestinal (GI) tract development is guided by several transcription factors, including Pancreatic and duodenal homeobox gene-1 (PDX1). While it is well known that PDX1 is essential for pancreas development, its role in GI development and function in humans has not been investigated. We have identified two patients with a homozygous point mutation in PDX1 (c.188delC(p.P63fs)), causing total loss of protein function. These patients have additional GI complications however endoscopy and histological analysis of gastric and ?intestinal biopsies did not? reveal any pathologies. We used patient derived GI organoids from induced pluripotent stem cells (iPSCs) as a novel diagnostic approach and identified a myriad of undiagnosed GI pathologies. Analyses of human antral gastric organoids (HAGO) using immunofluorescence staining demonstrated a loss of gastric marker? claudin18 (CLDN18) and increased intestinal villin expression in PDX1-null? organoids, signifying that regions of the antrum are converting to an intestinal?phenotype. Similarly, human intestinal organoids (HIO) demonstrated a loss of intestinal marker ?Cadherin17 and an increase in gastric marker? CLDN18, indicating that regions of the intestine are undergoing gastric metaplasia. We also observed an increase in parietal cells in HAGOs, a lack of gastrin hormone in HAGO, and an increase in mucin production in HIOs. Based on this data we re-analyzed antral and duodenal biopsies of both patients and confirmed gastric and intestinal metaplasia increased parietal cells, decreased gastrin and increased mucin production. Because of our findings, these patients will have additional screening to monitor for development of gastric and intestinal cancer. This clearly demonstrates the utility of organoids in improving patient diagnoses and developing new clinical treatment plans. Disclosure M. Krishnamurthy: None. T.R. Broda: None. X. Zhang: None. J.J. Palermo: None. I.H. Thomas: None. A. Heider: None. D.B. Steien: None. A. Dauber: Consultant; Self; Novo Nordisk Inc. Research Support; Self; Novo Nordisk Inc. J. Wells: None. Funding American Diabetes Association (1-19-PDF-036 to M.K.)