BACKGROUND: Selection of central venous catheter (CVC) lock solution impacts catheter mechanical complications and central line-associated bloodstream infections (CLABSIs) in pediatric patients with intestinal failure. Disadvantages of the current clinical standards, heparin and ethanol lock therapy (ELT), led to the discovery of new lock solutions. High-risk pediatric patients with intestinal failure who lost access to ELT during a recent shortage were offered enrollment in a compassionate use trial with 4% tetrasodium EDTA (T-EDTA), a lock solution with antimicrobial, antibiofilm, and antithrombotic properties.
METHODS: We performed a descriptive cohort study including 14 high-risk pediatric patients with intestinal failure receiving 4% T-EDTA as a daily catheter lock solution. CVC complications were documented (repairs, occlusions, replacements, and CLABSIs). Complication rates on 4% T-EDTA were compared with baseline rates, during which patients were receiving either heparin or ELT (designated as heparin/ELT).
RESULTS: Patients initiated 4% T-EDTA at the time they were enrolled in the compassionate use protocol. Use of 4% T-EDTA resulted in a 50% reduction in CVC complications, compared with baseline rates on heparin/ELT (incidence rate ratio: 0.50; 95% CI, 0.25-1.004; P = 0.051).
CONCLUSIONS: In a compassionate use protocol for high-risk pediatric patients with intestinal failure, the use of 4% T-EDTA reduced composite catheter complications, including those leading to emergency department visits, hospital admissions, additional procedures, and mortality. This outcome suggests 4% T-EDTA has benefits over currently available lock solutions.

BACKGROUND: Although parenteral nutrition (PN) significantly improves mortality rates in pediatric short bowel syndrome (SBS), long-term PN has many possible complications and impacts quality of life. Bowel lengthening procedures (BLPs) increase the contact surface of food and the intestinal mucosa and enable the better absorption of nutrients and liquids, possibly leading to a PN decrease.
METHODS: We retrospectively reviewed the data of patients with short bowel syndrome who underwent BLPs in the period from January 2016 to January 2022. Overall, eight patients, four male, five born prematurely, underwent BLPs.
RESULTS: There was a significant decrease in the percentage of total caloric intake provided via PN and PN volume after the BLPs. The more evident results were seen 6 months after the procedure and at the last follow-up, which was, on average, 31 months after the procedure. Two patients were weaned off PN after their BLPs. Patients remained well nourished during the follow-up.
CONCLUSIONS: The BLP led to a significant decrease in PN needs and an increase in the food intake; however, significant changes happened more than 6 months after the procedure.

Facing with an increasing demand for transition to adult care management, our home parenteral nutrition (HPN) team designed an adolescent therapeutic educational program (ATEP) specifically intended for adolescents on long-term HPN. The aim of this study was to report on the first sessions of this program.
UNASSIGNED: The ATEP is designed in three sessions of five consecutive days, during school holidays over the year. It includes group sessions on catheter handling, disconnecting and connecting the PN and catheter dressing, dealing with unforeseen events (e.g., fever or catheter injury), but also sessions with psychologist, social worker, sports teacher, fashion specialist, meeting with adults who received HPN since childhood. Specific course for the accompanying parents were also provided. Six months after the last session, a 3-day trip to the attraction park \"le Futuroscope,\" Poitiers, France, was organized without any parental presence.
UNASSIGNED: After 3 ATEP courses, a total of 16 adolescents have been enrolled. They were aged between 13 and 17 years (median 14 IQR: 14-16.25). All were on long term HPN started during the neonatal period except for four who started PN at a median age of 10 years old (IQR: 1-10). At the time of the ATEP, their median PNDI was 105% (IQR: 95.5-120.8) while receiving a median of six infusions per week (IQR: 5-7). Thirteen received Taurolidine lock procedure. After the ATEP, 11 adolescents could be considered as fully autonomous, 4 as partially autonomous and one failed to gain any autonomy. Course evaluation by adolescents or parents was good to excellent.
UNASSIGNED: Through the holistic and multiprofessional approach of this training and the group cohesion, the adolescents were not only able to handle catheter care and PN connections but were able to understand and accept better their illness and project themselves into their own future.

Background and aims: Home parenteral nutrition (HPN) is a life-saving treatment for patients affected by chronic intestinal failure (CIF). Both this clinical condition and its therapy require radical lifestyle modifications, affecting life quality and psychological balance in patients as well as family members. Patient psychological burden has rarely been taken into consideration, not to mention that of caregivers. This study aims to evaluate the levels of anxiety in CIF patients on HPN, and their caregivers, consequently determining their impact on the psychological and physical aspects. Methods: After a brief introductory interview, adult patients on HPN for CIF and their caregivers were asked to fill in the HAMA-A questionnaire. Results: Fifty patients and their respective caregivers were enrolled. Mean HAMA-A scores were similar in patients and caregivers and testified the presence of a mild to severe impact of CIF and HPN in both groups, with a significantly higher impact on female patients and caregivers. After adjusting age, education level, duration of CIF and HPN dependence, and degree of kinship, no differences were revealed in the scores. Conclusions: The study confirms that CIF patients on HPN and their caregivers have a significant anxiety burden independently from the duration of the disease, therefore needing appropriate support.

BACKGROUND: Intestinal failure, defined as the loss of gastrointestinal function to the point where nutrition cannot be maintained by enteral intake alone, presents numerous challenges in children, not least the timing of consideration of intestine transplantation.
OBJECTIVE: To describe the evolution of care of infants and children with intestinal failure including parenteral nutrition, intestine transplantation, and contemporary intestinal failure care.
METHODS: The review is based on the authors\' experience supported by an in-depth review of the published literature.
RESULTS: The history of parenteral nutrition, including out-patient (home) administration, and intestine transplantation are reviewed along with the complications of intestinal failure that may become indications for consideration of intestine transplantation. Current management strategies for children with intestinal failure are discussed along with changes in need for intestine transplantation, recognizing the difficulty in generalizing recommendations due to the high level of heterogeneity of intestinal pathology and residual bowel anatomy and function.
CONCLUSIONS: Advances in the medical and surgical care of children with intestinal failure have resulted in improved transplant-free survival and a significant fall in demand for transplantation. Despite these improvements a number of children continue to fail rehabilitative care and require intestine transplantation as life-saving therapy or when the burden on ongoing parenteral nutrition becomes too great to bear.

BACKGROUND: Home parenteral nutrition (HPN) is the primary treatment modality for patients with chronic intestinal failure, one of the least common organ failures. This article provides a retrospective analysis of the data collected on HPN patients in the Czech Republic over the past 30 years.
METHODS: National registry data were collected using a standardised online form based on the OASIS registry (Oley - A.S.P.E.N. Information System) across all centres providing HPN in the Czech Republic. Data collected prospectively from adult patients in the HPN program were analysed in the following categories: epidemiology, demographics, underlying syndrome, diagnosis, complications, and teduglutide therapy prevalence.
RESULTS: The registry identified a total of 1,838 adult patient records, reflecting almost 1.5 million individual catheter days. The prevalence of HPN has risen considerably over the last few decades, currently reaching 5.5 per 100,000 population. The majority of patients have short bowel syndrome and GI obstruction, with cancer being the most prevalent underlying disease. Catheter-related bloodstream infections have been the most prevalent acute complication. However, the incidence in 2022 was only 0.15 per 1,000 catheter days. The study also observed an increase in the prevalence of patients on palliative HPN over the last decade.
CONCLUSIONS: This study presents a thorough analysis of data from the Czech REgistr Domaci NUtricni Podpory (REDNUP) registry. It shows an increasing prevalence of HPN, namely, in the palliative patient group. The sharing of national data can improve understanding of this rare condition and facilitate the development of international guidelines.

OBJECTIVE: The intestinal rehabilitation program (IRP) is a specialized approach to managing patients with intestinal failure (IF). The goal of IRP is to reduce the patient\'s dependence on parenteral nutrition by optimizing nutrition intake while minimizing the risk of complications and providing individualized medical and surgical treatment. We aimed to provide a thorough overview of our extensive history in adult IRP.
METHODS: We reviewed the medical records of adults with IF treated at our center\'s IRP over the past two decades. We collected data on demographic and clinical results, such as the causes of IF, the current status of the remaining bowel, nutritional support, and complications or mortality related to IF or prolonged parenteral nutrition.
RESULTS: We analyzed a total of 47 adult patients with a median follow-up of 6.7 years. The most common cause of IF was massive bowel resection due to mesenteric vessel thrombosis (38.3%). Twenty-eight patients underwent rehabilitative surgery, including 12 intestinal transplants. The 5-year survival rate was 81.9% with 13 patients who expired due to sepsis, liver failure, or complication after transplantation. Of the remaining 34 patients, 18 were successfully weaned off from parenteral nutrition.
CONCLUSIONS: Our results of IRP over two decades suggest that the individualized and multidisciplinary program for adult IF is a promising approach for improving patient outcomes and achieving nutritional autonomy.

Fat malabsorption is central to the pathophysiology of short bowel syndrome (SBS). It occurs in patients with insufficient intestinal surface area and/or function to maintain metabolic and growth demands. Rapid intestinal transit and impaired bile acid recycling further contribute to fat malabsorption. A significant portion of patients require parenteral nutrition (PN) for their survival but may develop sepsis and liver dysfunction as a result. Despite advancements in the treatment of SBS, fat malabsorption remains a chronic issue for this vulnerable patient population. Peer-reviewed literature was assessed on the topic of fat malabsorption in SBS. Current management of patients with SBS involves dietary considerations, PN management, antidiarrheals, glucagon-like peptide 2 agonists, and multidisciplinary teams. Clinical trials have focused on improving intestinal fat absorption by facilitating fat digestion with pancreatic enzymes. Targeting fat malabsorption in SBS is a potential pathway to improving lifestyle and reducing morbidity and mortality in this rare disease.

Patients with intestinal failure who receive long-term parenteral nutrition (PN) often develop intestinal failure-associated liver disease (IFALD). Although there are identified risk factors, the early pathogenesis is poorly understood and treatment options are limited. Here, we perform a transcriptomic analysis of liver tissue in a large animal IFALD model to generate mechanistic insights and identify therapeutic targets. Preterm Yorkshire piglets were provided PN or bottle-fed with sow-milk replacer for 14 days. Compared to bottle-fed controls, piglets receiving PN developed biochemical cholestasis by day of life 15 (total bilirubin 0.2 vs. 2.9 mg/dL, P = 0.01). RNA-Seq of liver tissue was performed. Ingenuity Pathway Analysis identified 747 differentially expressed genes (343 upregulated and 404 downregulated) with an adjusted P < 0.05 and a fold-change of > |1|. Enriched canonical pathways were identified, demonstrating broad activation of inflammatory pathways and inhibition of cell cycle progression. Potential therapeutics including infliximab, glucocorticoids, statins, and obeticholic acid were identified as predicted upstream master regulators that may reverse the PN-induced gene dysregulation. The early driver of IFALD in neonates may be inflammation with an immature liver; identified therapeutics that target the inflammatory response in the liver should be investigated as potential treatments.

BACKGROUND: D-lactic acidosis (DLA) is a serious complication of short bowel syndrome (SBS) in children with intestinal failure (IF). Malabsorbed carbohydrates are metabolized by bacteria in the intestine to D-lactate which can lead to metabolic acidosis and neurologic symptoms.
METHODS: A retrospective chart review was performed in children ≤18 years old with SBS who had one of the following criteria: unexplained metabolic acidosis, neurologic signs or symptoms, history of antibiotic therapy for small bowel bacterial overgrowth, or high clinical suspicion of DLA. Cases had serum D-lactate concentration >0.25 mmol/L; controls with concentrations ≤0.25 mmol/L.
RESULTS: Of forty-six children, median age was 3.16 (interquartile range (IQR): 1.98, 5.82) years, and median residual bowel length was 40 (IQR: 25, 59) cm. There were 23 cases and 23 controls. Univariate analysis showed that cases had significantly lower median bicarbonate (19 vs. 24 mEq/L, p = 0.001), higher anion gap (17 vs. 14 mEq/L, p < 0.001) and were less likely to be receiving parenteral nutrition, compared with children without DLA. Multivariable analysis identified midgut volvulus, history of intestinal lengthening procedure, and anion gap as significant independent risk factors. Midgut volvulus was the strongest independent factor associated with DLA (adjusted odds ratio = 17.1, 95% CI: 2.21, 133, p = 0.007).
CONCLUSIONS: DLA is an important complication of pediatric IF due to SBS. Patients with IF, particularly those with history of midgut volvulus, having undergone intestinal lengthening, or with anion gap acidosis, should be closely monitored for DLA.