Studies investigating growth impairment (GI) in children with intestinal failure (IF) have seldom reported its associated factors. We hypothesized that a deficient nutritional intake would affect GI in children with IF. Nineteen patients (2-16 years old) who underwent home-based parenteral nutrition (PN) management at our institution, were divided into patients with or without GI (GI+/GI-). GI was defined as a height-for-age z-score (HAZ) ≤ -2. We performed between-group comparisons of HAZ in the intestinal rehabilitation program (IRP)/nutrition support team (NST) introduction time, PN dependency, residual small intestine length (RSIL), nutritional intake (calorie, protein, lipids, and carbohydrates), caloric intake ratio, and nutrient intake as a percentage of the recommended intake. Six patients were in the GI+ group. PN dependency was significantly higher in the GI+ group (GI+, 109.8 %; GI < median, 61.7 %, P = 0.036). RSIL did not differ significantly between the groups (50 cm vs. 45 cm, P = 0.879). In the GI+ group, the caloric percentage of lipids was significantly lower (11.9 % vs. 17.5 %, P = 0.002), but that for carbohydrates was significantly higher (76.8 % vs. 66.8 %, P = 0.002), while lipid sufficiency was significantly lower (37.5 % vs. 89.6 %, respectively; P < 0.001). Protein intake in the GI+ group was significantly lower; however, the corresponding sufficiency was > 100 % in both groups (136.5 % vs. 209.2 %, P = 0.005). For children with IF, a low intake ratio of proteins and lipids relative to energy requirement and required nutrient levels may inhibit adequate growth.

Infective endocarditis (IE) is a rare but serious complication in pediatric patients with chronic intestinal failure (IF) dependent on long-term parenteral nutrition (PN) via central venous access devices (CVADs). This study aimed to investigate the incidence, clinical features and outcomes of IE in children with chronic IF at a single institution. A retrospective descriptive study was conducted in pediatric patients (0-18 years) with chronic IF requiring PN for > 60 days during 2013-2024. IE was diagnosed using modified Duke criteria. Five IE episodes occurred in 4 patients out of 29 with chronic IF (median follow-up 9 years). The overall incidence was 0.144 per 1000 catheter days (5155.2 per 100,000 person-years). Compared with patients without IE, those with IE had longer catheter durations (median 2941.5 vs. 443.0 days, p = 0.006); more central line-associated bloodstream infections (median 8 vs. 1, p = 0.0015); higher prevalence of catheter-related venous thrombosis (100 % vs. 12 %, p = 0.001); and were more likely to have dysmotility (75 % vs. 16 %, p = 0.02). IE resolved with antimicrobial therapy in all cases. During a median follow-up of 2.2 years, one patient died from superior vena cava obstruction, sepsis and multiorgan failure while awaiting transplantation; one achieved enteral autonomy and was weaned off PN; and two underwent successful intestinal transplantation. Though uncommon, IE poses significant risks in pediatric IF; early cardiac surveillance in high-risk patients may facilitate timely diagnosis and intervention.

The objective of this position statement was to provide evidence-based and expert-informed recommendations for the assessment and treatment of neurodevelopment in children with intestinal failure and those undergoing intestinal transplant. A literature review was conducted to identify and review research articles from 2000 to 2023 that reported on neurodevelopmental, neurocognitive and motor outcomes in children (0-18 years) with intestinal failure or intestinal transplant. Key recommendations to address these outcomes were made based on scientific evidence and expert-informed opinion. The impact of intestinal failure and transplant on developmental outcomes was established, along with key risk factors related to the disease that may result in poorer outcomes. The involvement of rehabilitation specialists was recommended as part of the multidisciplinary intestinal care team to provide assessment and treatment across all developmental domains from infancy onwards. Care teams should provide close follow-up which may include screening during out-patient clinic follow up, along with timely community referrals to address developmental delays. Children undergoing intestinal or multi-visceral transplant require assessment and treatment both pre and post-transplant to maximize developmental outcomes. This position paper reviews available literature related to developmental outcomes in intestinal failure and transplant, delineating risk factors which may contribute to poorer outcomes. It lays the foundation for tangible steps to assist intestinal care teams in advocating for the services of rehabilitation team members to provide ongoing assessment and treatment to optimize clinical outcomes.

Intestinal transplantation (IT) remains the only treatment modality for patients with irreversible intestinal failure who cannot be maintained on chronic parenteral nutrition. Multiple reports have demonstrated an association between donor-specific antibodies (DSA) in IT graft rejection, however these studies have largely been single-center analyses. The goal of this study was to examine the available immunological data from the International Intestinal Transplant Registry (IITR) and evaluate the association with rejection. Demographic, outcomes, and serologic HLA data from donor/recipient pairs was obtained from the IITR. Outcomes including rejection, graft survival, and patient survival were analyzed based on age, DSA reporting, and molecular HLA mismatch. Eplet mismatch analysis was performed using HLAMatchmaker. Our analysis revealed significant limitations in the granularity of data contained in the IITR. The IITR database is a promising option to perform a multicenter analysis of IT outcomes, but there are limitations in the data to allow a thorough immunologic examination.

Background:The significance of foreign body (FB) ingestion in children with Intestinal Failure (IF) is unknown. We aimed to characterize differences in procedural management and clinical outcomes related to gastrointestinal FB removal in pediatric patients with IF compared to children without this condition.Material and Methods:This ten-year retrospective review utilized electronic healthcare data from a single pediatric center. We evaluated patients who underwent post-esophageal gastrointestinal FB removal procedures between 2011 and 2020 and compared medical history, presentation, procedure type, and procedure outcomes in children with and without IF.Results:There were 12 patients with IF and 185 controls. All FBs ingested by IF patients carried low intrinsic risk of perforation or obstruction. Esophagogastroduodenoscopy (EGD) was the most common removal procedure in both groups. Patients with IF were more likely to have FBs removed from a post-pyloric location (100 vs. 20.5 %, p < 0.0001), require lower endoscopy (41.7 vs. 9.7 %, p = 0.006), require multiple procedures for definitive removal (41.7 vs. 8.6 %, p = 0.0041), involve > 1 endoscopist or pediatric surgeon (66.7 vs. 7.0 %, p < 0.0001), and require hospital admission (83.3 vs. 28.6 %, p < 0.0001).Conclusion:FB ingestion by children with IF is associated with increased risk of procedural complexity and hospital admission, even when the object’s intrinsic risk of gastrointestinal hazard is low. These differences may be related to altered intestinal anatomy and dysmotility. Physicians should consider involvement of an advanced proceduralist during removal. Education to prevent ingestion should be part of routine IF care.

Fecal microbiota transplantation (FMT) treats patients with recurrent Clostridioides difficile infections (CDI) by restoring the colonic flora with a balanced microbiome. There are currently no reports of safe and successful FMT in pediatric patients with history of intestinal transplant on immunosuppression. Our case report describes a 6-year-old boy with a multi-visceral transplant (including: stomach, small bowel, colon, liver, pancreas, and en-bloc bilateral kidneys) who had recurrent CDI episodes treated with antibiotics (including prolonged tapers). After multiple recurrences, he had a FMT via colonoscopy. The fecal preparation was inserted in the transplanted cecum without complication. The patient did well without any adverse events or need for hospitalization and the FMT was a success, as he had no recurrent symptoms in the 3 months following FMT.

Patients with short bowel syndrome (SBS) have a loss of absorptive bowel surface area and are at risk for dehydration. Oral rehydration solutions (ORS) promote intestinal fluid absorption and may be used to maintain adequate hydration. Currently, there are no standard ORS recommendations for children with SBS. The objectives of this study are to (1) characterize the ORS practice patterns of pediatric intestinal rehabilitation providers and (2) understand the perceived barriers to patient compliance. A survey was created with questions regarding provider details, current practice recommendations for ORS, and the factors affecting ORS compliance. The survey was distributed electronically to intestinal rehabilitation providers in North America. 66 individuals with varying clinical roles from across North America completed the survey. Most respondents recommend Pedialyte® (60/66) and the second most commonly recommended ORS is the homemade World Health Organization (WHO) ORS recipe (31/66). Regardless of the type of ORS, the most common reasons identified for poor patient compliance are poor taste and cost, with taste being a greater issue for the homemade solutions and cost being a greater issue for the commercially available products. Insurance coverage for ORS is limited (only reported for Pedialyte® and Ceralyte®). The recommended ORS products contain variable sodium concentrations and variable sugar sources. The effect of these alternative sugar sources on intestinal absorption in SBS is not yet fully understood. Improved insurance coverage for ORS should be considered as this is cited as a barrier to the use of ORS in pediatric SBS.