Complementary food introduction practices in infants with intestinal failure.

BACKGROUND: Oral intake in infants with intestinal failure (IF) may be limited due to intolerance or feeding difficulties. Guidelines for the introduction of semisolid or solid complementary foods (CFs) to infants with IF do not exist. CF intake and caloric contribution from CF is difficult to assess due to malabsorption and incomplete recording. The aim of this study was to identify institutional approaches to introducing CF to infants with IF. METHODS: The American Society for Parenteral and Enteral Nutriton (ASPEN) Pediatric Intestinal Failure Section Registered Dietitian/Nutritionist (RDN) working group designed a 10-question online cloud-based survey to assess group member practice related to the introduction of CF to infants with IF. RESULTS: Twenty-six surveys were completed. Thirteen (50%) RDNs recommend introduction of CF between 4 and 6 months of age. Nineteen (76%) recommend adding pureed foods to gastrostomy tube feedings. Seventeen (65%) follow standard infant feeding practice guidelines with half citing the American Academy of Pediatrics. Approximately half (44%) recommend introducing vegetables first and the majority (80%) recommend delaying the introduction of fruits. The vast majority (92%) recommend specific foods to minimize stool output including green beans, bananas, infant cereals, and meats/protein. CONCLUSION: Institutional practices related to the introduction of CF to infants with IF vary. Similarities with first food choice and foods to avoid were observed. Evidenced-based practice guidelines for the introduction of CF to infants with IF need to be established to determine best practices for reducing stool output, encouraging weaning from parenteral nutrition, and achieving enteral autonomy.

Efficacy and safety of intravenous iron sucrose in children younger than 2 years with intestinal failure.

Iron-deficiency anemia (IDA) is highly prevalent in children with intestinal failure (IF) and oral iron supplementation is often ineffective in this patient population. Even though various intravenous (IV) iron formulations are available, there is a dearth of data on the use of newer parenteral iron products such as IV iron sucrose, especially in infants and young children (<2 years of age) with IF. To determine safety and efficacy, we performed a retrospective chart review on infants and children younger than 2 years with IF who received IV iron sucrose for IDA between October 2019 and August 2021. The review revealed that 10 events of IV iron sucrose replacement were administered to five children aged 4-22 months with IF and IDA. We observed a mean increase in hemoglobin of 1.9 ± 0.6 g/dl, and peak hemoglobin levels were seen at 4.3 ± 0.8 weeks after the IV iron sucrose dose. The infusions were well tolerated, and no short-term adverse reactions or laboratory abnormalities were observed. Based on these observations, the use of IV iron sucrose appears to be safe and effective in infants and young children with IF and could be considered in the management of IDA in this patient population.

Vitamin D status in children with intestinal failure who have achieved enteral autonomy.

BACKGROUND: Vitamin D deficiency is highly prevalent in children with intestinal failure (IF) who receive parenteral nutrition (PN), but data on vitamin D status after achieving enteral autonomy (EA) are limited. We aimed to evaluate the prevalence of vitamin D deficiency in this population while exploring clinical variables that may be associated with its development. METHODS: A retrospective review was performed on 29 children with IF who had achieved EA. Deficiency was defined as a mean serum 25-hydroxyvitamin D <30 ng/ml. DATA RESULTS: Sixty-six percent of children had at least one deficient level during the study period, with 38% being deficient based on the mean vitamin D levels. Eighty-four percent had radiologic evidence of osteopenia. Compared with the sufficient group (n=18), the deficient group (n=11) received higher daily mean vitamin D doses (2246 vs 920 IU; P=.02), had shorter remnant small-bowel length (53.8 vs 82.1 cm; P=.03), and were PN dependent for a longer duration (1.3 vs 0.58 years; P=.01). Univariate analyses revealed longer remnant gut length (odds ratio [OR] = 1.03; P=.04) and shorter duration of PN (OR = 0.26; P=.04) to be significantly associated with sufficient vitamin D status. CONCLUSION: Vitamin D deficiency and osteopenia are highly prevalent in pediatric patients with a history of IF who have achieved EA, despite enteral supplementation with higher than standard doses. Shorter remnant small-bowel length and longer duration of PN were associated with vitamin D deficiency. These findings emphasize the importance of prolonged surveillance and highlight the need for alternate dosing regimens.

Efficacy of Dairy Free Diet and 6-Food Elimination Diet as Initial Therapy for Pediatric Eosinophilic Esophagitis: A Retrospective Single-Center Study.

PURPOSE: Management of eosinophilic esophagitis (EoE) varies from center to center. In this study, we evaluated the effectiveness of a dairy-free diet (DFD) and the 6-Food Elimination Diet (SFED) as initial therapies for the treatment of EoE in our practice. METHODS: This was a retrospective study of children who had been treated for EoE at Connecticut Children's Medical Center, Hartford, CT, USA. Pre- and post-treatment endoscopy findings and histology results of patients treated with DFD or SFED were examined. RESULTS: One hundred fifty-two patients (age 9.2±5.2 years, 76.3% male, 69.7% caucasian) met the inclusion criteria for initial treatment with DFD (n=102) or SFED (n=50). Response for DFD was 56.9% and for SFED was 52.0%. Response based on treatment duration (<10, 10-12, and >12 weeks) were 81.8%, 50.0%, and 55.1% for DFD, and 68.8%, 50.0%, and 40.0% for SFED. Response based on age (<6, 6-12, and >12 years) were 59.3%, 42.9%, and 67.5% for DFD, and 36.4%, 58.8%, and 72.7% for SFED. In patients treated with DFD, concomitant proton pump inhibitor (PPI) administration resulted in improved outcomes (p=0.0177). Bivariate regression analysis showed that PPI with diet is the only predictor of response (p=0.0491), however, there were no significant predictors on multiple regression analysis. CONCLUSION: DFD and SFED are effective first line therapies for EoE. DFD should be tried first before extensive elimination diets. Concomitant therapy with PPI's may be helpful.

Transition to a Tube Feeding Formula With Real Food Ingredients in Pediatric Patients With Intestinal Failure.

Due to concerns related primarily to allergic response and malabsorption, enteral nutrition therapy has traditionally relied on the use of elemental formulas in children with intestinal failure (IF). Blended food diets via a gastrostomy tube have been reported to improve feeding tolerance in pediatric populations receiving long-term enteral nutrition therapy. Complex macronutrients have been shown to stimulate intestinal adaptation in animal models. We report on our experience in children with IF who had an overall improvement in stool output when transitioned from an elemental formula to a tube feeding formula with real food ingredients (TFRF). Data were collected in a retrospective chart review of children with IF, >1 year of age, who were receiving enteral nutrition via continuous infusion, bolus feeding, or both. Indications for the TFRF trial were diarrhea or inconsistent stooling patterns. Ten children with a mean small bowel length of 48.3 cm were trialed on TFRF. Nine of 10 (90%) children tolerated the transition to 100% TFRF, of which 7 of 9 (78%) had their entire colon in continuity. The average age at successful transition was 29.2 months, and the average length of time to transition to 100% TFRF was 67.3 days. TFRF is well tolerated in children >1 year of age with IF; it also improves their stooling patterns. A commercially available TFRF is a cost-effective and nutritionally adequate means of providing nutrition to this patient population.

Effects of Ethanol Lock Therapy on Central Line Infections and Mechanical Problems in Children With Intestinal Failure.

OBJECTIVES: Although use of 70% ethanol lock therapy (ELT) has been shown to decrease the rate of catheter-related bloodstream infections (CRBSIs) in patients with intestinal failure and central venous catheters (CVCs), concerns have been raised about its association with higher rates of mechanical problems and CVC replacements (CVC-Rs). We sought to compare the rates of CRBSI, mechanical problems, and CVC-Rs in a cohort of pediatric patients with intestinal failure, with and without ELT (ELT+ and ELT-, respectively). METHODS: Data were collected in a retrospective chart review from February 2007 to May 2014. Mann-Whitney and Wilcoxon signed-rank tests were used to compare nonparametric and paired data, respectively. RESULTS: Twenty-nine children had 9033 catheter days (CDs). The ELT+ group (vs ELT-) had lower rate of infection and significantly fewer CVC-Rs due to infection but significantly more mechanical events and related CVC-Rs with significantly shorter mean CVC survival. In 13 children who had a pre-ELT and post-ELT period, ELT was associated with a decrease in the rate of CVC-Rs due to infection (0.36 vs 4.74/1000 CDs, P = .046) and an increase in the rate of CVC-Rs due to mechanical problems (5.05 vs 0/1000 CDs, P = .018). CONCLUSIONS: While ELT+ is associated with a lower rate of CRBSIs and related CVC-Rs, it is also associated with higher rates of mechanical problems and related CVC-Rs. In addition to investigating the ideal concentration, duration, and timing of ELT to preserve the integrity of the CVC, alternatives to exclusively ethanol-based lock solutions should be developed.

Interdisciplinary approach to the management of intestinal transplant recipients: evaluation, discharge, and lifetime management.

Patients suffering from intestinal failure present unique and difficult challenges to the transplant team. Augmenting the need for interdisciplinary teamwork is the higher incidence of death on the intestinal transplant waiting list. Successful management of this population requires an interdisciplinary approach at each stage of care, beginning with evaluation and continuing through discharge and lifetime management. A close relationship between patients, their caregivers, and all members of the transplant team is an essential component to successful lifetime management. Open communication between team members and unlimited accessibility to each other enables work flow to be managed efficiently, and enables the provision of optimal care. In this article, we describe the functions of the nonphysician clinical personnel needed to manage the intestinal transplant patient--beginning at the evaluation through lifetime follow-up care. The goal of each professional is the same: to restore the patient to the best quality of life possible.