Parenteral nutrition (PN) is a complex and specialized form of nutrition support that has revolutionized the care for both pediatric and adult patients with acute and chronic intestinal failure (IF). This has led to the development of multidisciplinary teams focused on the management of patients receiving PN: nutrition support teams (NSTs). In this review we aim to discuss the historical aspects of IF management and NST development, and the practice, composition, and effectiveness of multidisciplinary care by NSTs in patients with IF. We also discuss the experience of two IF centers as an example of contemporary NSTs at work. An NST usually consists of at least a physician, nurse, dietitian, and pharmacist. Multidisciplinary care by an NST leads to fewer complications including infection and electrolyte disturbances, and better survival for patients receiving short- and long-term PN. Furthermore, it leads to a decrease in inappropriate prescriptions of short-term PN leading to significant cost reduction. Complex care for patients receiving PN necessitates close collaboration between team members and NSTs from other centers to optimize safety and effectiveness of PN use.

Introduction The aims of the study are to systematically assess and critically appraise the evidence concerning two surgical techniques to lengthen the bowel in children with short bowel syndrome (SBS), namely, the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP), and to identify patient characteristics associated with a favorable outcome. Materials and Methods MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception till December 2019. No language restriction was used. Results In all, 2,390 articles were found, of which 40 were included, discussing 782 patients. The median age of the patients at the primary bowel lengthening procedure was 16 months (range: 1–84 months). Meta-analysis could not be performed due to the incomparability of the groups, due to heterogeneous definitions and outcome reporting. After STEP, 46% of patients weaned off parenteral nutrition (PN) versus 52% after LILT. Mortality was 7% for STEP and 26% for LILT. Patient characteristics predictive for success (weaning or survival) were discussed in nine studies showing differing results. Quality of reporting was considered poor to fair. Conclusion LILT and STEP are both valuable treatment strategies used in the management of pediatric SBS. However, currently it is not possible to advise surgeons on accurate patient selection and to predict the result of either intervention. Homogenous, prospective, outcome reporting is necessary, for which an international network is needed.

Objectives: The aim of the study was to describe the longitudinal development of health‐related quality of life (HRQOL) and fatigue in children with chronic intestinal failure (CIF) on home parenteral nutrition (PN) and compare these children to the general population.Methods: Prospective, observational study conducted over 7 years in patients suffering from CIF receiving home PN from 2 tertiary hospitals in the Netherlands. Every 6 months, parents (if child <8 years old) or patients (if child ≥8 years old) completed 2 questionnaires: Pediatric Quality of Life Inventory 4.0 (PedsQL) Generic and Fatigue on the KLIK (kwaliteit van leven in kaart [Dutch Acronym for Quality of Life in Clinical Practice]) Patient Reported Outcome Measures portal, which were compared with the general population. Linear mixed models (LMMs) were constructed to investigate the course of HRQOL over time.Results: Thirty‐five patients were included (40% girls). At time of last KLIK contact, patients received HPN for a median of 5.3 years (interquartile range [IQR]: 2.9–9.7). In total, 272 questionnaires were completed. PedsQL generic total score for ages 5 to 7 and 8 to 12 years was significantly lower than the general population (P < 0.01 for both age groups) with effect sizes of 0.73 and 0.71, respectively. PedsQL fatigue total score for ages 5 to 7 years was also significantly lower (P = 0.01; effect size 0.70). LMMs for PedsQL Generic and Fatigue total score 2 to 7 and 8 to 18 years showed no significant coefficient for duration of home PN.Conclusions: Children suffering from CIF receiving home PN ages 5 to 12 years report lower HRQOL scores than the general population. HRQOL and fatigue do not change during long‐term treatment with home PN in these children.

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. Therefore, the aim of this study is to create a core outcome set (COS) to be used in studies on pediatric chronic intestinal failure.Methods: Candidate outcomes were selected from a recent systematic review. A three‐round Delphi study among key stakeholders and a consensus meeting with an expert panel were undertaken to achieve consensus on the COS.Results: Seventy‐two stakeholders (79%) completed all three rounds of the Delphi process. Ninety‐eight outcomes were assessed, and five new outcomes were added after the first round. Ten outcomes were included in the final COS: weaning from parenteral nutrition, growth, mortality, central line–related infection, central line longevity, sepsis not related to central line infection, central line–related thrombosis, intestinal failure–associated liver disease, (serious) adverse events, and health‐related quality of life.Conclusion: This pediatric chronic intestinal failure COS consists of 10 outcomes important for all key stakeholders. Usage of this set in future research should minimize outcome heterogeneity and enhance the value of evidence synthesis. This will lead to better management in this field of rare gastrointestinal conditions.

Pediatric chronic intestinal failure (PIF) is a rare and heterogeneous condition characterized by the inability of the patient’s intestine to adequately absorb the required fluids and/or nutrients for growth and homeostasis. As a result, patients will become dependent on home parenteral nutrition (HPN). A MEDLINE search was performed in May 2024 with keywords “intestinal failure”, “parenteral nutrition” and “pediatric”. Different underlying conditions which may result in PIF include short bowel syndrome, intestinal neuromuscular motility disorders and congenital enteropathies. Most common complications associated with HPN are catheter-related bloodstream infections, catheter-related thrombosis, intestinal failure-associated liver disease, small intestinal bacterial overgrowth, metabolic bone disease and renal impairment. Treatment for children with PIF has markedly improved with a great reduction in morbidity and mortality. Centralization of care in specialist centers and international collaboration between centers is paramount to further improve care for this vulnerable patient group. A recently promising medical therapy has become available for children with short bowel syndrome which includes glucagon-like peptide 2, a naturally occurring hormone which is known to delay gastric emptying and induce epithelial proliferation. Despite advances in curative and supportive treatment, further research is necessary to improve nutritional, pharmacological and surgical care and prevention of complications associated with parenteral nutrition use.

Background: Children with intestinal failure (IF) are at risk of loss of vascular access because of catheter‐related venous thrombosis. Whether primary prophylactic anticoagulation is effective and safe in preventing catheter‐related thrombosis is largely unknown. Our aim was to assess the incidences of catheter‐related venous thrombosis and bleeding complications in children with IF receiving home parenteral nutrition (HPN) treated with primary prophylactic anticoagulation.Methods: All children, aged 0–18 years, treated with HPN at the Emma Children's Hospital/Amsterdam UMC were followed from January 2007 to July 2019. All patients were offered primary prophylactic anticoagulation from the start of HPN. The primary outcomes were catheter‐related venous thrombosis and bleeding on prophylactic anticoagulation.Results: In total, 55 (76%) of 74 patients received primary prophylactic anticoagulation. The median age at the start of prophylaxis was 8.4 (interquartile range [IQR], 5.0–55.7) months. Patients were followed for a median of 31.2 (IQR, 10.7–53.5) months, with a total of 65,463 catheter days. The incidence of catheter‐related thrombosis on prophylactic anticoagulation was 0.2 per 1000 catheter days. In total, the incidence of clinically relevant bleeding was 0.1 per 1000 catheter days. The median time to first event was 1268 (IQR, 149–2014) days for thrombosis and 389 (IQR, 227–2912) days for clinically relevant bleeding. Cumulative event‐free survival after 5 years was 78% for thrombosis.Conclusions: Our study shows a low rate of catheter‐related venous thrombosis and a slightly elevated rate of clinically relevant bleeding in children receiving HPN and primary prophylactic anticoagulation.