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. The evidence to support the use of specific diet regimens and nutritional approaches in most CODE disorders is limited due to the rarity of these diseases and the scant published clinical experience. The goal of this review was to create a comprehensive guide for nutritional management in CODE, based on the currently available literature, disease mechanism, and the PediCODE group experience. Enteral diet management in CODE can be divided into 3 distinct conceptual frameworks: nutrient elimination, nutrient supplementation, and generalized nutrient restriction. Response to nutrient elimination or supplementation can lead to resolution or significant improvement in the chronic diarrhea of CODE and resumption of normal growth. This pattern can be seen in CODE due to carbohydrate malabsorption, defects in fat absorption, and occasionally in electrolyte transport defects. In contrast, general diet restriction is mainly supportive. However, occasionally it allows parenteral nutrition weaning or reduction over time, mainly in enteroendocrine defects and rarely in epithelial trafficking and polarity defects. Further research is required to better elucidate the role of diet in the treatment of CODE and the appropriate diet management for each disease.

Online Acceptance and Commitment Therapy and Nutrition Workshop for Parents of Children with Inflammatory Bowel Disease: Feasibility, Acceptability, and Initial Effectiveness.

Parents of children with inflammatory bowel disease (IBD) are important members of their healthcare team and influence their child's adaptation to disease. The primary aim of this research was to test the feasibility and acceptability of a three-session online parent workshop based on acceptance and commitment therapy (ACT) and address concerns about eating well and nutrition in IBD. The secondary aim was to explore the initial effectiveness of this workshop in parent reported psychological flexibility, mindfulness, experiential avoidance, cognitive fusion, valued living, and symptoms of depression, anxiety, and stress. We used a single arm pragmatic prospective study design with parents of children attending the IBD program at a tertiary pediatric healthcare centre in Canada. Mixed methods patient reported outcomes were measured at baseline, immediate post participation, and 3 months post participation in the workshop. Thirty-seven parents enrolled in the study and feasibility and acceptability goals were largely met. Parents qualitatively described changes to their parenting, what aspects of the workshop were most helpful, and targeted feedback on how to improve workshop. Findings suggest that providing parents of children with IBD a brief online ACT workshop including nutrition guidance is feasible and leads to changes in parenting behaviours.

Body Composition Using Air Displacement Plethysmography in Children With Inflammatory Bowel Disease.

OBJECTIVE: The aim of the study was to assess the body composition of children with inflammatory bowel disease (IBD) and to study the accuracy of clinically available tools in predicting excess body fatness. We aimed at also exploring the influence of adiposity on pharmacokinetics during early Infliximab exposure. METHODS: Prospective cohort study in 5- to 17-year-old children with IBD initiating Infliximab therapy. Patient demographic, phenotypic, and laboratory data at the time of Infliximab initiation were recorded. Body composition was assessed using air displacement plethysmography (ADP). fat mass index (FMI = fat mass [kg]/(height [m])) was calculated to determine excess adiposity (defined as FMI ≥75th centile). Anthropometrics (weight, height, mid upper arm circumference [MUAC] and triceps skin fold thickness [TSF]) were obtained and MUAC and TSF measurements were used to calculate arm fat area (AFA) and arm muscle area z-scores. Statistical analysis was applied as appropriate. RESULTS: Fifty-three (68% male; 55% Crohn disease [CD], 45% ulcerative colitis [UC], median [IQR] age 15 [13-16] years) children with IBD were included. Twenty-four percentage of children with IBD (21% CD, 29% UC) had excess adiposity. Four children (31%) with FMI ≥75th centile were not identified by body mass index (BMI) alone (kappa of 0.60), and 2 children (15%) were not identified by AFA z-score alone. The intra- and interobserver reliability of MUAC and TSFT measurements was excellent. There was no difference in Infliximab trough levels at the end of induction between those with FMI less than or ≥75th centile. CONCLUSIONS: Excess adiposity affects approximately 1 in 4 young patients with IBD and can be missed by routine obesity screening. Our exploratory study did not raise concerns of underexposure to infliximab in those children with excess adiposity during early drug exposure.

Online education for gluten-free diet teaching: Development and usability testing of an e-learning module for children with concurrent celiac disease and type 1 diabetes.

BACKGROUND AND OBJECTIVE: Celiac disease (CD), the most common genetically-based food intolerance, affects 3% to 16% of children with type 1 diabetes (T1D). Treatment involves lifelong adherence to a gluten-free diet (GFD). Individualized dietary education is resource-intensive. We, therefore, sought to develop and test the usability of an e-learning module aimed at educating patients and caregivers regarding implementation of the GFD in children with concurrent CD and T1D. METHODS: An interactive e-learning module was developed based on extensive review of CD, T1D, and educational literature. A mixed-methods usability testing approach was used to refine and evaluate the module, using qualitative semi-structured interviews, observations, and satisfaction and knowledge questionnaires in two iterative cycles. The module was refined based on themes identified from each usability cycle. RESULTS: Eighteen patients (8 in cycle 1, 10 in cycle 2) and 15 caregivers (7 in cycle 1, 8 in cycle 2) participated. Patient participants had CD and T1D for a mean (SD) of 6.1 ± 5.1 and 8.3 ± 5.5 years, respectively. Their mean age was 13.5 ± 4.5 years. Thematic analysis of usability interviews showed the module to be appealing and resulted in minor module revisions after each cycle to improve usability. Mean satisfaction scores post-module completion were high (4.67 ± 0.54), indicating participants were "very satisfied" with the education. Knowledge test scores increased significantly from pre- to post-module completion (P = 0.001). CONCLUSION: A multifaceted user-centered usability approach demonstrated that an innovative, interactive e-learning module is effective in knowledge retention and can provide comprehensive and accessible information in the implementation of the GFD teaching in children with CD and T1D.

Long-term Exposure of Children to a Mixed Lipid Emulsion Is Less Hepatotoxic Than Soybean-based Lipid Emulsion.

Lipid emulsions have been associated with liver injury. Newer mixed emulsions (ML), such as SMOFlipid (Fresenius Kabi, Germany), are thought to be more hepatoprotective than soybean-based emulsions (SL), such as Intralipid (Baxter). Pediatric studies comparing long-term use between the 2 are limited. This study compares the severity of hepatic injury between a prospective cohort of hospitalized children on ML (n = 20) and a historical age- and diagnosis-matched cohort of hospitalized children on SL (n = 20). Median exposure to ML and SL were 10 versus 6 weeks (P = 0.030), respectively, at similar median lipid doses (2.2 vs 2.1 g ·â€Škg ·â€Šday). Using a generalized estimating equations approach, conjugated bilirubin trajectory was found to be lower in patients on ML compared with SL (P < 0.001), suggesting that prolonged exposure (≥4 weeks) to ML is associated with decreased liver injury compared with SL in hospitalized children.

Using an Allometric Equation to Accurately Predict the Energy Expenditure of Children and Adolescents With Nonalcoholic Fatty Liver Disease.

BACKGROUND: Pediatric patients with nonalcoholic fatty liver disease (NAFLD) require targeted nutrition therapy that relies on calculating energy needs. Common energy equations are inaccurate in predicting resting energy expenditure (REE), influencing total energy expenditure (TEE) estimates. Equations based on allometric scaling are simple, accurate, void of subjective activity and/or stress factor bias, and they estimate TEE. OBJECTIVE: To investigate the predictive accuracy of an allometric energy equation (AEE) in predicting TEE of children and adolescents with NAFLD. METHODS: Retrospective study performed in a single institution. The allometric equation was used to calculate AEE, and the results were compared with TEE calculated using indirect calorimetry data (measured REE) multiplied by an activity factor (AF) of 1.5 or 1.7. RESULTS: Fifty-six patients with a mean age of 13 years were included in this study. The agreement between TEE (using an AF of 1.5) and AEE was -96 kcal/d (confidence interval, -29 to 221). The predictive accuracy of the allometric equation was not different between obese and nonobese patients. CONCLUSIONS: Allometric equations allow for accurate estimation of TEE in children with NAFLD.

Resting Energy Expenditure of Children and Adolescents With Nonalcoholic Fatty Liver Disease.

BACKGROUND: The mainstay of treatment for pediatric nonalcoholic fatty liver disease (NAFLD) is lifestyle modification, which includes dietary changes that lead to slow but sustained weight loss or weight stabilization in growing children. Accurate estimation of energy requirements is necessary to achieve this goal. The objective of this study was to assess the accuracy of the most commonly used equations in predicting the resting energy expenditure (REE) of children with NAFLD. METHODS: This was a retrospective study performed in a single institution. The predictive accuracy of various equations was assessed by comparing their estimates against the measured REE obtained with indirect calorimetry. Accuracy was defined as an estimate within 10% of measured REE. RESULTS: Fifty-six children (70% male; 52% white and 36% Asian) with a median age of 13 years were included. The median measured REE was 1829 kcal/d. Of the equations studied, the Schofield had the smallest average bias (-32 kcal/d; confidence interval, -121 to 56). The Schofield and Molnar equations were the most accurate, providing REE estimates within 10% of measured in 59% of cases. The remaining equations had lower and variable predictive accuracy. The use of adjusted body weight in predictive equations did not improve the predictive accuracy. CONCLUSION: In a cohort of children and adolescents with NAFLD, the Schofield and Molnar equations performed best in predicting energy expenditure. However, predictive equations were often inaccurate, suggesting that clinicians should interpret their results with caution and consider using indirect calorimetry when available.