The Novel Lipid Emulsion Vegaven Is Well Tolerated and Elicits Distinct Biological Actions Compared With a Mixed-Oil Lipid Emulsion Containing Fish Oil: A Parenteral Nutrition Trial in Piglets.

Background: Vegaven is a novel lipid emulsion for parenteral nutrition (PN) based on 18-carbon n-3 (ω-3) fatty acids, which elicits liver protection via interleukin-10 (IL-10) in the murine model of PN.

Objectives: In a preclinical model of PN in neonatal piglets, Vegaven was tested for efficacy and safety and compared with a mixed-oil lipid emulsion containing fish oil (SMOFlipid).

Methods: Male piglets 4-5 d old were randomly allocated to isocaloric isonitrogenous PN for 14 d, which varied only by the type of lipid emulsion (Vegaven, n = 8; SMOFlipid, n = 8).

Long-term teduglutide associated with improved response in pediatric short bowel syndrome-associated intestinal failure.

Objectives: Patients with short bowel syndrome-associated intestinal failure (SBS-IF) require long-term parenteral nutrition and/or intravenous fluids (PN/IV) to maintain fluid or nutritional balance. We report the long-term safety, efficacy, and predictors of response in pediatric patients with SBS-IF receiving teduglutide over 96 weeks.

Methods: This was a pooled, post hoc analysis of two open-label, long-term extension (LTE) studies (NCT02949362 and NCT02954458) in children with SBS-IF.

Improved long-term outcome of children with congenital diarrhea followed by an intestinal rehabilitation program.

Background: Long-term outcomes of congenital diarrheas and enteropathies (CODE) are poorly described. We evaluated the morbidity and mortality of children with CODE followed by an intestinal rehabilitation program (IRP) compared to children with short bowel syndrome (SBS).

Methods: Matched case-control study of children with intestinal failure (IF) due to CODE (diagnosed between 2006 and 2020; N = 15) and SBS (N = 42), matched 1:3, based on age at diagnosis and duration of parenteral nutrition (PN).

Association between 4%-tetrasodium EDTA and sepsis in neonatal piglets: A retrospective cohort study.

Background: Central line-associated bloodstream infections are a major concern for children with intestinal failure and in animal research using parenteral nutrition (PN). In neonatal piglets receiving PN, we compared sepsis, line occlusions, line replacements, mortality, and costs with and without the use of a 4%-tetrasodium ethylenediaminetetraacetic acid (T-EDTA) locking solution.

Methods: We performed a retrospective review of piglets with a central venous jugular catheter enrolled in 14-day exclusive PN (TPN) trials or in 7-day short bowel syndrome (SBS) trials, before and after initiation of T-EDTA.

Small and large bowel anatomy is associated with enteral autonomy in infants with short bowel syndrome: A retrospective cohort study.

Background: Achievement of enteral autonomy (EA) is the ultimate treatment goal in pediatric intestinal failure (IF). We aimed to assess predictors of EA in pediatric short bowel syndrome (SBS) and explore the impact of residual small bowel (SB) and large bowel (LB) length on EA.

Methods: A retrospective cohort study was performed on infants aged <12 months (n = 367, six centers) with SBS referred between 2010 and 2015.

Cost-utility analysis of teduglutide compared to standard care in weaning parenteral nutrition support in children with short bowel syndrome.

Background & Aims: A growing proportion of children with short bowel syndrome (SBS) remain dependent on long-term parenteral nutrition (PN). Teduglutide offers the potential for more children to decrease PN support and achieve enteral autonomy (EA), but at a significant expense. This study aims to assess the incremental costs of teduglutide plus standard of care compared to standard of care alone in weaning PN support per quality-adjusted life year (QALY) gained in children with SBS.

Properties of ultrasound-rapid MRI clinical diagnostic pathway in suspected pediatric appendicitis.

Objective: to determine diagnostic accuracy of an US-MRI clinical diagnostic pathway to detect appendicitis in the emergency department (ED).

Study Design: prospective cohort study of 624 previously healthy children 4-17 years old undergoing US for suspected appendicitis and clinical re-assessment. Children with non-diagnostic USs and persistent appendicitis concern/conclusive US-reassessment discrepancies underwent ultra-rapid MRI (US-MRI pathway), interpreted as positive, negative or non-diagnostic.

Body Composition and Physical Activity in Pediatric Intestinal Failure On and Off Parenteral Nutrition.

Objectives: Data on the relationship between body composition (BC) and physical activity (PA) in children with intestinal failure (IF) are lacking. The objectives were to collect data on PA and BC in children with IF, both parenterally and enterally fed, and to assess the relationship between PA and BC.

Methods: Cross-sectional study in children 5-18 years with IF including those receiving parenteral nutrition (PN) and those fully enterally fed.

In parenteral nutrition-fed piglets, fatty acids vary by lipid emulsion and tissue sampled.

Background: Children with intestinal failure without liver disease may be given soy-based lipid emulsion (SLE) or mixed lipid emulsion (MLE; containing soy, medium-chain triglyceride, olive, and/or fish oils). Both differ in essential fatty acid content: MLE has added arachidonic acid (AA) and docosahexaenoic acid (DHA). The aim of this study, in neonatal piglets, was to compare serum and tissue fatty acid composition when the emulsions were given at unrestricted doses.

Composite lipid emulsion use and essential fatty acid deficiency in pediatric patients with intestinal failure with high parenteral nutrition dependence: A retrospective cohort study.

Background: Reports of essential fatty acid deficiency (EFAD) in patients receiving parenteral nutrition (PN) and a composite lipid (mixed oil intravenous lipid emulsion [MO ILE]) are predominantly when managed by lipid restriction. The objective of this study was to determine the prevalence of EFAD in patients with intestinal failure (IF) who are PN dependent without lipid restriction.

Methods: We retrospectively evaluated patients, ages 0-17 years, followed by our intestinal rehabilitation program between November 2020 and June 2021 with PN dependency index (PNDI) of >80% on a MO ILE.

Assessment of body composition in pediatric intestinal failure: A comparison study.

Background: The objective of the study was to compare bioelectrical impedance analysis (BIA) and skinfolds with dual energy x-ray absorptiometry (DXA) in the assessment of body composition of children with intestinal failure. DXA is the reference method for body composition assessment in clinical settings.

Methods: Children aged 1-18 years with intestinal failure whohave DXA as part of routine clinical monitoring were eligible.

Intestinal failure among adults and children: Similarities and differences.

Intestinal failure (IF) is a complex medical condition that is caused by a constellation of disorders, resulting in the gut's inability to adequately absorb fluids and nutrients to sustain hydration, growth, and survival, thereby requiring the use of parenteral fluid and/or nutrition. Significant advancements in intestinal rehabilitation have resulted in improved survival rates for individuals with IF. There are distinct differences, however, related to etiology, adaptive potential and complications, and medical and surgical management when comparing children with adults.

Use of a combined transient elastography and biochemical strategy to determine liver fibrosis in pediatric intestinal failure.

Background & Aims: Non-invasive monitoring of intestinal failure (IF) associated liver disease is an ongoing challenge in children with IF. Our objective was to develop a combined algorithm of clinical, transient elastography (TE) and biochemical parameters to identify liver fibrosis in this population.

Methods: A retrospective cohort study of IF patients followed by our intestinal rehabilitation program between November 2015 to October 2019.

Predictors of Social-Emotional Development and Adaptive Functioning in School-Age Children with Intestinal Failure.

Objectives: Despite a focus on neurocognition in pediatric intestinal failure (IF) to date, we examined social-emotional and adaptive functioning.

Methods: Children (N = 63) in our IF rehabilitation program underwent neuropsychological assessments including caregiver- and teacher-reported questionnaires. Results were compared to norms using z-tests.

Stress, anxiety, depression, and health-related quality of life in caregivers of children with intestinal failure receiving parenteral nutrition: A cross-sectional survey study.

Background: Improved survival rates for children with intestinal failure (IF) have resulted in an increased population of children receiving long-term parenteral nutrition (PN). Our objective was to determine burden on caregivers of children with IF receiving long-term PN.

Methods: We performed a cross-sectional study of caregivers of children with IF receiving long-term PN in our intestinal rehabilitation program.

An international multicenter validation study of the Toronto listing criteria for pediatric intestinal transplantation.

Deciding which patients would benefit from intestinal transplantation (IT) remains an ethical/clinical dilemma. New criteria* were proposed in 2015: ≥2 intensive care unit (ICU) admissions, loss of ≥3 central venous catheter (CVC) sites, and persistently elevated conjugated bilirubin (CB ≥ 75 μmol/L) despite 6 weeks of lipid modification strategies. We performed a retrospective, international, multicenter validation study of 443 children (61% male, median gestational age 34 weeks [IQR 29-37]), diagnosed with IF between 2010 and 2015.

Muscle Strength, Agility, and Body Composition in Children With Intestinal Failure on Parenteral Nutrition.

Objectives: With increasing number of children with intestinal failure (IF) on long-term parenteral nutrition (PN), this study assesses the impact of IF on muscle strength, speed, and agility and body composition (BC), identifying clinical factors that may predict impairment.

Methods: Cross-sectional study in children 5-18 years with IF on PN. Assessments included Bruininks-Oseretsky Test of Motor Proficiency-2 strength and agility subtest (BOT-2), and grip strength.

Management of pediatric intestinal failure related to short bowel syndrome.

Intestinal failure (IF) secondary to short bowel syndrome is a challenging and complex medical condition with significant risk for surgical and medical complications. Significant advancements in the care of this patient population have led to improved survival rates. Due to their intensive medical needs children with IF are at risk for long-term complications that require comprehensive management and close monitoring.

Carrying the Burden: Informal Care Requirements by Caregivers of Children with Intestinal Failure Receiving Home Parenteral Nutrition.

Objective: To measure the time that caregivers spend on tasks related to providing care to their child with intestinal failure receiving home parenteral nutrition (PN).

Study Design: We conducted an exploratory cross-sectional study of caregivers of children with intestinal failure receiving long-term PN followed by our intestinal rehabilitation program. Caregivers completed a daily diary of care-related tasks.

Probiotic treatment vs empiric oral antibiotics for managing dysbiosis in short bowel syndrome: Impact on the mucosal and stool microbiota, short-chain fatty acids, and adaptation.

Background: Infants and children with short bowel syndrome (SBS) are presumed to be at risk of gut microbial dysbiosis with potential sequelae of bacterial overgrowth that include sepsis, d-lactic acidosis, mucosal inflammation, and malabsorption. In neonatal piglets with SBS, we compared intestinal microbial composition, short-chain fatty acids (SCFAs), and adaptation given probiotic (PRO) treatment (Lactobacillus and Bifidobacterium spp) vs oral metronidazole (MET).

Methods: Following 75% distal small intestinal resection, piglets were allocated to PRO (500 mg twice a day, n = 7), MET (15 mg/kg twice a day, n = 8), and placebo (PLA) (500 mg twice a day, n = 8).

Treatment and Outcomes of Congenital Ovarian Cysts A Study by the Canadian Consortium for Research in Pediatric Surgery (CanCORPS).

Objective: We conducted a multicenter study to assess treatments and outcomes in a national cohort of infants with congenital ovarian cysts.

Summary Background Data: Wide variability exists in the treatment of congenital ovarian cysts. The effects of various treatment strategies on outcomes, specifically ovarian preservation, are not known.

The Diverse Phenotype of Intestinal Dysmotility Secondary to ACTG2-related Disorders.

Background And Aims: The initial description of a heterozygous dominant ACTG2 variant in familial visceral myopathy was followed by the identification of additional variants in other forms of intestinal dysmotility disorders. we aimed to describe the diverse phenotype of this newly reported and rare disease.

Methods: Report of 4 new patients, and a systematic review of ACTG2-related disorders.

Differentiating congenital ovarian cysts from other abdominal cystic lesions in female infants: A study by the Canadian Consortium for Research in Pediatric Surgery (CanCORPS).

Purpose: The origin of congenital abdominal cysts in the female fetus often dictates management. While most arise from the ovary and are often managed non-operatively, some are non-ovarian and are frequently removed. We analyzed a national sample of female infants with congenital abdominal cysts to elucidate prenatal and postnatal factors associated with the diagnosis of a non-ovarian cyst.