Are enteral devices risk factors for central line-associated bloodstream infections in children with intestinal failure?

Background & Aims: Central line-associated bloodstream infections (CLABSI) represent one of the most common and serious complications in children with intestinal failure (IF). This study aimed to assess if there is an association between the use of enteral devices (feeding tubes and stomas) with rate of CLABSI after adjusting for clinically relevant factors. Second, association between enteral devices with time to first CLABSI event was evaluated.

Characteristics and outcomes of home parenteral nutrition among children with severe neurological impairment.

Objectives: To describe the characteristics, secular trends, and outcomes of home parenteral nutrition (HPN) use among children with severe neurological impairment (SNI) and non-primary digestive disorders from 2010 to 2023 and compare outcomes to children with primary digestive disorders on HPN.

Methods: A retrospective review of all children with SNI and non-primary digestive disorders, where HPN was initiated between January 2010 and September 2023 at a tertiary care pediatric hospital. The Mann-Kendall trend test was used to assess trends in HPN initiation.

Long-term neurodevelopmental outcomes following liver transplantation for metabolic disease-a single centre experience.

This study describes the neurodevelopmental outcome of children with urea cycle disorders (UCD) and organic acidemias (OA) preliver transplant (LT), 1-year, and 3-years post-LT. We performed a retrospective chart review of children with OA or UCD transplanted between January 2014 and December 2021. Standardized motor and cognitive assessment scores were collected from children who had ≥1 motor/cognitive assessment at any timepoint.

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).

Diet management in congenital diarrheas and enteropathies - general concepts and disease-specific approach, a narrative review.

Congenital diarrheas and enteropathies (CODE) are a group of rare, heterogenous, monogenic disorders that lead to chronic diarrhea in infancy. Definitive treatment is rarely available, and supportive treatment is the mainstay. Nutritional management in the form of either specialized formulas, restrictive diet, or parenteral nutrition support in CODE with poor enteral tolerance is the cornerstone of CODE treatment and long-term growth.

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.

The Development of the International Intestinal Failure Registry and an Overview of its Results.

Pediatric intestinal failure (IF) is a rare disease that represents an evolving field in pediatric gastroenterology and surgery. With only a limited number of multicenter collaborations, much of the research in pediatric IF is often confined to single-center reports with small sample sizes. This has resulted in challenges in data interpretation and left many knowledge gaps unanswered.

Organ Donation in Canadian PICUs: A Cross-Sectional Survey, 2021-2022.

Objectives: To understand contemporary pediatric organ donation programs in Canadian PICUs, including: policies and practices, data collection and reporting, and system and process barriers.

Design: A cross-sectional survey carried out 2021-2022.

Setting: Canadian PICUs affiliated with a donor physician network.

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.

Outcome After Intestinal Transplantation From Living Versus Deceased Donors: A Propensity-matched Cohort Analysis of the International Intestinal Transplant Registry.

Objective: To describe the worldwide experience with living donation (LD) in intestinal transplantation (ITx) and compare short-term and long-term outcomes to a propensity-matched cohort of deceased donors.

Background: ITx is a rare life-saving procedure for patients with complicated intestinal failure (IF). Living donation (LD)-ITx has been performed with success, but no direct comparison with deceased donation (DD) has been performed.

Physical function in children and adolescents pre- and 1-year post-liver transplant.

Background: Several studies describe poorer motor developmental motor outcomes post-liver transplant (LT) in younger children. Limited studies examine physical function in older children and adolescents pre- and post-LT.

Methods: Retrospective review of standard of care physical function outcome measures pre- and 1-year post-LT in children ≥6 years at LT.

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.

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.

Unmasking a New Type of Gastrointestinal Foreign Body in the COVID-19 Era.

The incidence of foreign body ingestion has increased during the coronavirus disease 2019 pandemic. As face masks became increasingly available, we report a case of accidental ingestion of the metal strip insert of a surgical mask. After initially progressing, its progress halted after 24 hours.

Immunogenicity of a quadrivalent human papillomavirus vaccine in pediatric kidney and liver transplant recipients.

Background: Solid-organ transplant recipients are at increased risk of developing human papillomavirus-related diseases.

Methods: To evaluate the immunogenicity of a quadrivalent vaccine, a prospective observational study included females aged 12-19 years who had received kidney or liver transplants, or were otherwise healthy volunteers. With the three-dose vaccination, serum antibodies were measured.

HERV1-env Induces Unfolded Protein Response Activation in Autoimmune Liver Disease: A Potential Mechanism for Regulatory T Cell Dysfunction.

Regulatory T cells (Tregs) are not terminally differentiated but can acquire effector properties. Here we report an increased expression of human endogenous retrovirus 1 (HERV1-env) proteins in Tregs of patients with de novo autoimmune hepatitis and autoimmune hepatitis, which induces endoplasmic reticulum (ER) stress. HERV1-env-triggered ER stress activates all three branches (IRE1, ATF6, and PERK) of the unfolded protein response (UPR).

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.

Development of a core outcome set for pediatric chronic intestinal failure.

Background: In research on pediatric chronic intestinal failure, heterogeneity in reported definitions and outcomes exists. This leads to a risk of reporting bias and impossibility of evidence synthesis. Also, reported outcomes should be relevant to both healthcare providers and patients and their parents.

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.