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Background: Short bowel syndrome is considered a low prevalence disease. The scant information available about intestinal failure in Latin America was the driving force to expand this registry., Methods: A prospective, multicenter observational registry was created for patients with chronic intestinal failure short bowel at specialized centers in Latin America. Demographics, clinical characteristics, nutrition assessment, parenteral nutrition management, intestinal rehabilitation, related complications, clinical outcome, and survival were analyzed., Results: From May 2020 to July 2023, 167 patients (115 adults, 52 children) from 20 centers were enrolled. For the adults, the mean age was 37.2 ± 18 years, 48% were female, and the mean follow-up was 22.6 ± 18.3 months. The main etiology was surgical resections (postsurgical complications: 37%; ischemia: 25%); the mean intestinal length was 73 ± 55 cm. The complications were as follows: infections: 0.4/1000 catheter-days; thrombosis: 0.24/1000 catheter-days; liver disease: 2.6%. The outcomes were as follows: 28% were rehabilitated, 15% died, 9.6% were lost to follow-up, 0.9% underwent transplant, and 45.6% continued follow-up. For the children, the mean age 48 ± 52 months, 48% were female, 52% were premature. The mean follow-up was 17.2 ± 5.6 months; the mean remaining intestinal length was 38 ± 45 cm. The leading etiologies were atresia (25%), NEC (23%), and gastroschisis (21%). The complication were as follows: infections: 2/1000 catheter-days; thrombosis: 2.22/1000 catheter-day; 25% developed liver disease. The outcomes were as follows: 7.7% died, 3.8% were rehabilitated, and 88.5% continued follow-up., Conclusion: The RESTORE amendment served as a registry and educational tool for the participating teams. The aspiration is to objectively show current aspects of intestinal failure in the region and carry them to international standards. Including all Latin American countries and etiologies of chronic intestinal failure besides short gut would serve to complete this registry., (© 2024 American Society for Parenteral and Enteral Nutrition.)

Background: Serial transverse enteroplasty is used to treat patients with chronic intestinal failure owing to short bowel syndrome. Current literature lacks discussion of its role for other etiologies of intestinal failure and its impact on adult patients' nutrition support needs and quality of life., Methods: We performed a case series on adults with parenteral nutrition (PN) dependence who underwent serial transverse enteroplasty at Emory University Hospital, a quaternary referral center between 2011 and 2022. Data collected included demographics, operative technique, and preoperative and postoperative PN requirements. A phone survey was administered to evaluate the impact of PN and the operation on quality of life., Results: Ten patients underwent the procedure of interest during the study period. Indications included short bowel syndrome following multiple abdominal operations or intra-abdominal catastrophe and chronic partial bowel obstruction with dysmotility. Bowel length increased by a median of 83%. All patients were discharged home after a median hospital stay of 21 days. At 1-year follow-up, survival was 100%, two (20%) patients fully weaned from PN, three others (30%) reduced PN frequency, and six (60%) decreased their daily parenteral energy requirement. Two additional patients fully weaned from PN by 18 months postoperatively., Conclusion: This represents one of the largest case series of serial transverse enteroplasty in adults. Small intestinal length nearly doubled, and PN dependence was reduced in most patients. Given the low morbidity and good quality of life observed in this series, this procedure should be more widely investigated for patients with chronic intestinal failure., (© 2024 American Society for Parenteral and Enteral Nutrition.)

Background: Pediatric patients with intestinal failure require long-term parenteral nutrition owing to impaired enteral nutrition absorption. A potential complication is essential fatty acid deficiency (EFAD), resulting from decreased linoleic and α-linolenic acid concentrations and defined by an increased triene:tetraene ratio (TTR; Mead acid:arachidonic acid). Historically, soybean oil lipid emulsion (SOLE) was the only commercially available parenteral lipid in the United States. Recently, a composite lipid emulsion (CLE) and fish oil lipid emulsion (FOLE) received US Food and Drug Administration approval. This study investigated whether lipid emulsion regimen impacts EFAD incidence in pediatric patients with intestinal failure., Methods: This study was a 10-year retrospective cohort study of pediatric patients with intestinal failure who received parenteral SOLE, CLE, or FOLE. The primary outcome was EFAD incidence, defined as a TTR ≥ 0.2. Secondary outcomes included TTR ≥ 0.05, cholestasis incidence, lipid dose effect on EFAD incidence, and fatty acid parameter differences., Results: A total of 144 fatty acid profiles from 47 patients were reviewed. EFAD did not occur in any lipid emulsion group. There were no differences in the incidence of TTR ≥ 0.05 or cholestasis. The effect of dose could not be evaluated because of no EFAD incidence. Lastly, although each group had varied fatty acid parameters, none saw decreased essential fatty acid levels., Conclusion: This study found that, with close monitoring, the lipid emulsion regimen did not impact EFAD incidence. This suggests that FOLE and CLE do not increase EFAD risk compared with SOLE in pediatric patients with intestinal failure., (© 2024 American Society for Parenteral and Enteral Nutrition.)

Background: Patients with chronic intestinal failure (CIF) are at increased risk of developing renal impairment. The aim of this study was to evaluate the occurrence of chronic kidney disease (CKD) in patients dependent on home parenteral nutrition (HPN) and assess risk factors for renal impairment, including patients with all mechanisms of CIF., Methods: This was a cohort study of patients initiated on HPN between March 1, 2015, and March 1, 2020, at a national UK IF Reference Centre. Patients were followed from their first discharge with HPN until HPN cessation or the end of follow-up on December 31, 2021., Results: There were 357 patients included in the analysis. Median follow-up time was 4.7 years. At baseline, >40% of patients had renal impairment, with 15.4% fulfilling the criteria for CKD. Mean estimated glomerular filtration rate (eGFR) decreased significantly during the first year after initiation of HPN from 93.32 ml/min/1.73 m 2 to 86.30 ml/min/1.73 m 2 at the first year of follow-up (P = 0.002), with sequential stabilization of renal function. Increased age at HPN initiation and renal impairment at baseline were associated with decreased eGFR. By the end of follow-up, 6.7% patients developed renal calculi and 26.1% fulfilled the criteria for CKD., Conclusion: This is the largest study of renal function in patients receiving long-term HPN. After the first year following HPN initiation, the rate of decline in eGFR was similar to that expected in the general population. These findings should reassure patients and clinicians that close monitoring of renal function can lead to good outcomes., (© 2024 American Society for Parenteral and Enteral Nutrition.)

Background: Home parenteral nutrition (HPN) is a life-saving therapy required for the management of type III intestinal failure, one of the rarest organ failures. It requires a multidisciplinary approach to manage the complexity of the underlying medical, surgical, and nutrition issues, but the current levels of healthcare funding in Australia are unknown. This study aimed to quantify the caseload, staffing, and capacity of existing HPN centers nationally., Methods: This was a cross-sectional survey inviting centers known to provide HPN care. The survey was designed to capture metrics related to the national framework for the delivery of HPN. These centered on staffing levels, patient load, capacity to audit key outcomes, and service challenges., Results: A total of 24 (89%) of 27 invited centers responded to the survey. There were 17 (71%) adult centers and 7 (29%) pediatric centers. Adult centers managed a median of 12 (interquartile range [IQR]: 6-25) patients vs 16 (IQR: 9-17) in pediatric centers. Several centers did not have dedicated funding for core team members. The total funded clinician time each week per patient was 7 min (IQR: 0-12 min) in adult centers and 14 min (IQR: 10-21 min) in pediatric centers. Fewer than half of centers reported having sufficient resources to regularly audit key metrics., Conclusion: The availability of dedicated expertise to manage the highly complex needs of people living with type III intestinal failure is lacking in Australia. Current funding of HPN services falls well short of being sufficient to meet the requirements outlined in the national quality framework., (© 2024 The Author(s). Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: Chronic intestinal failure (CIF) refers to the long-lasting reduction of gut function below the minimum necessary to absorb macronutrients, water, and/or electrolytes. Patients with CIF likely develop various forms of malnutrition and dehydration, yet studies that focus primarily on body composition are lacking. Therefore, this study aimed to evaluate the body composition of adult patients with CIF., Methods: This retrospective descriptive cohort study was performed at the Radboud University Medical Center, a tertiary referral center for CIF treatment in the form of home parenteral nutrition. We collected available bioelectrical impedance analysis (BIA) data from routine care between 2019 and 2023. The primary outcome was body composition, which was evaluated by assessing body mass index (BMI), fat-free mass index (FFMI), and fat percentage (fat%)., Results: Overall, 147 adult patients with CIF were included with a median (interquartile range) age of 58 (25-68) years; 69% were female. The mean (SD) BMI was 22.1 (4.3) kg/m 2 , FFMI was 14.2 (1.9) kg/m 2 in females and 17.0 (2.0) kg/m 2 in males, and fat% was 33.7% (6.8%) in females and 24.6% (6.4%) in males. 63% had an FFMI below references, and 48% had a high fat%., Conclusion: This study found that most adult patients with CIF have an unfavorable body composition characterized by a high fat% and low FFMI despite having a normal mean BMI. These results highlight the necessity for in-depth nutrition assessment, including BIA measurement. Moreover, future studies should focus on exercise interventions to increase FFMI and improve body composition and function., (© 2024 The Author(s). Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

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)., Conclusion: 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., (© 2024 American Society for Parenteral and Enteral Nutrition.)

Background: Children with chronic IF require long-term home parenteral nutrition (HPN), administered through a central venous catheter. Catheter-related bloodstream infection (CRBSI) with Staphylococcus aureus is known to be a serious infection with a high mortality rate and risk of complications. A standardized protocol on the management of S aureus CRBSIs in children receiving HPN is lacking. The aim of this study is to evaluate the effectiveness and safety of the current management in an HPN expertise center in the Netherlands., Methods: We performed a retrospective descriptive cohort study between 2013 and 2022 on children 0-18 years of age with chronic IF requiring long-term HPN. Our primary outcomes were the incidence of S aureus CRBSI per 1000 catheter days, catheter salvage attempt rate, and successful catheter salvage rate. Our secondary outcomes included complications and mortality., Results: A total of 74 patients (39 male; 53%) were included, covering 327.8 catheter years. Twenty-eight patients (38%) had a total of 52 S aureus CRBSIs, with an incidence rate of 0.4 per 1000 catheter days. The catheter salvage attempt rate was 44% (23/52). The successful catheter salvage rate was 100%. No relapse occurred, and no removal was needed after catheter salvage. All complications that occurred were already present at admission before the decision to remove the catheter or not. No patients died because of an S aureus CRBSI., Conclusion: Catheter salvage in S aureus CRBSIs in children receiving HPN can be attempted after careful consideration by a multidisciplinary team in an HPN expertise center., (© 2024 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: Catheter-related bloodstream infections (CRBSIs) in patients receiving home parenteral nutrition (HPN) for chronic intestinal failure (CIF) are associated with significant morbidity and financial costs. Taurolidine is associated with a reduction in bloodstream infections, with limited information on the cost-effectiveness as the primary prevention. This study aimed to determine the cost-effectiveness of using taurolidine-citrate for the primary prevention of CRBSIs within a quaternary hospital., Methods: All patients with CIF receiving HPN were identified between January 2015 and November 2022. Data were retrospectively collected regarding patient demographics, HPN use, CRBSI diagnosis, and use of taurolidine-citrate. The direct costs associated with CRBSI-associated admissions and taurolidine-citrate use were obtained from the coding department using a bottom-up approach. An incremental cost-effective analysis was performed, with a time horizon of 4 years, to compare the costs associated with primary and secondary prevention against the outcome of cost per infection avoided., Results: Forty-four patients received HPN within this period. The CRBSI rates were 3.25 infections per 1000 catheter days before the use of taurolidine-citrate and 0.35 infections per 1000 catheter days after taurolidine-citrate use. The incremental cost-effectiveness ratio indicates primary prevention is the weakly dominant intervention, with the base case value of $27.04 per CRBSI avoided. This held with one-way sensitivity analysis., Conclusion: Taurolidine-citrate in the primary prevention of CRBSIs in patients with CIF receiving HPN is associated with reduced hospital costs and infection rates., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: Achievement of enteral autonomy (EA) is the ultimate treatment goal in pediatric intestinal failure (IF). We aimed to assess predictors of EA in pediatric short bowel syndrome (SBS) and explore the impact of residual small bowel (SB) and large bowel (LB) length on EA., Methods: A retrospective cohort study was performed on infants aged <12 months (n = 367, six centers) with SBS referred between 2010 and 2015. The cohort was stratified based on the achievement of EA. Statistical testing was completed using t-test, chi-square, Cox proportional hazards regression model, and Kaplan-Meier analysis., Results: EA was achieved in 229 patients. In the multivariable analysis, the percentage of residual LB (hazard ratio [HR] = 1.02; 95% CI = 1.01-1.02) and SB (HR = 1.01; 95% CI = 1.01-1.02) length, presence of the ileocecal valve (HR = 2.02; 95% CI=1.41-2.88), and not coming from a high-volume transplantation center (HR = 2.42; 95% CI = 1.68-3.49) were positively associated with EA, whereas a negative association was seen with the presence of stoma at the time when shortest remnant was documented (HR = 0.72; 95% CI = 0.52-1.00). EA achievement was significantly different between the anatomical subgroups (log-rank test P < 0.001) with an EA rate of 80.4% in infants with ≥50% SB and LB (median time 209 days); 62.5% with ≥50% SB and <50% LB (397 days); 58.3% with <50% SB and ≥50% LB (1192 days), and 25.9% with <50% SB and LB. Necrotizing enterocolitis (NEC) was not associated with a better achievement of EA (NEC vs other etiologies: log-rank test P = 0.33)., Conclusions: Overall, 62% of infants with IF secondary to SBS achieved EA over a mean time of follow-up of 2.3 years. A colon length of >50% can compensate for the loss of small bowel (<50%) and account for similar EA rates as those in children with residual SB > 50%., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: There is inequal access to treatment and scarce evidence on how the disease burden in chronic intestinal failure (CIF) compares to other chronic nonmalignant types of organ failure. Therefore, we compared the health-related quality of life (HRQOL) of people with CIF with that of people with end-stage kidney disease (ESKD) receiving hemodialysis (HD). These groups were selected for comparison as they have similar treatment characteristics. We hypothesized that people treated with HD and people with CIF had similarly poor HRQOL., Methods: HRQOL was evaluated and compared in a cross-sectional study of adult people with CIF and people with ESKD HD at a tertiary hospital in Denmark, using the Short-Form 36 (SF-36)., Results: One hundred forty-one people with CIF and 131 people with ESKD receiving HD were included in the analysis. Both groups reported low scores (<50) for HRQOL on general health, vitality, and role limitation-physical. People with ESKD receiving HD had significantly lower scores than people with CIF regarding physical functioning, general health, and vitality when adjusted for sex and age. No significant difference was found for any other SF-36 domain., Conclusion: HRQOL was similarly and significantly reduced in people with CIF and in people with ESKD receiving HD. People with ESKD receiving HD had significantly poorer HRQOL than people with CIF in some aspects of physical and mental health. Access to home parenteral support treatment varies among countries that typically provide HD, suggesting an inequality in healthcare based on the type of organ failure., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Introduction: Reported outcomes for parenteral nutrition (PN)-related complications in older adult patients with acute intestinal failure who are receiving PN in the acute hospital setting are limited. Our study aims to compare PN-related complications between older and younger adult patients., Methods: A retrospective descriptive study of inpatients who were administered PN from January 1, 2019, to December 31, 2019, was performed. Patients were categorized into older (≥65 years old) and younger (<65 years old) adult groups., Results: Two hundred thirty-five patients were included. There were 103 patients in the older adult group (mean age: 73.9 [SD: 6.9] years) and 132 patients in the younger adult group (mean age: 52.4 [SD: 12.5] years). There was a significantly higher Charlson Comorbidity Index score and lower Karnofsky score in the older adult group. The older adult group received significantly lower total energy (20.8 [SD: 7.8] vs 22.8 [SD: 6.3] kcal/kg/day), dextrose (3.1 [SD: 1.4] vs 3.6 [SD: 1.4] g/kg/day), and protein (1.1 [SD: 0.4] vs 1.2 [SD: 0.3] g/kg/day) than the younger group received. The mean length of stay was significantly shorter in the older adult group (35.9 [SD: 21.3] vs 59.8 [SD: 55.3]; P < 0.05). There was no significant difference in PN-related complications and clinical outcomes (catheter-related bloodstream infections, hypoglycemia or hyperglycemia, fluid overload, or inpatient mortality) between the two groups., Conclusion: Despite more comorbidities in the older adult, the usage of PN in older adult patients with acute intestinal failure was associated with neither an increased rate of PN-related complications nor worse clinical outcomes when compared with that of younger patients., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: Central line-associated bloodstream infections (CLABSI) are a serious complication in children with intestinal failure. This study assessed the incremental costs of 4% tetrasodium ethylenediaminetetraacetic acid (EDTA) compared with taurolidine lock and heparin lock per quality-adjusted life-year (QALY) gained in children with intestinal failure from the healthcare payer and societal perspective., Methods: A Markov cohort model of a 1-year-old child with intestinal failure was simulated until the age of 17 years (time horizon), with a cycle length of 1 month. The health outcome measure was QALYs, with results expressed in terms of incremental costs and QALYs. Model parameters were obtained from published literature and institutional data. Deterministic, probabilistic, and scenario sensitivity analyses were performed., Results: 4% Tetrasodium EDTA was dominant (more effective and less expensive) compared with taurolidine and heparin, yielding an additional 0.17 QALYs with savings of CAD$88,277 compared with heparin, and an additional 0.06 QALYs with savings of CAD$52,120 compared with taurolidine lock from the healthcare payer perspective. From the societal perspective, 4% tetrasodium EDTA resulted in savings of CAD$90,696 compared with heparin and savings of CAD$36,973 compared with taurolidine lock., Conclusions: This model-based analysis indicates that 4% tetrasodium EDTA can be considered the optimal strategy compared with taurolidine and heparin in terms of cost-effectiveness. The decision uncertainty can be reduced by conducting further research on the model input parameters. An expected value of perfect information analysis can identify what model input parameters would be most valuable to focus on., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: To determine variables associated with outcomes in infants with intestinal failure (IF) and ostomy following reanastomosis (RA)., Methods: A single-center, descriptive cohort study of 120 infants with IF and a stoma from January 2011 to December 2020 with subsequent RA during initial hospitalization. The primary outcome was achievement of enteral autonomy (EA) following RA. Other outcomes were duration of hospital stay, and mortality. Penalized logistic regression and linear regression were used for data analysis., Results: The median gestational age was 26 weeks, and the median birth weight was 890 g. Three infants died. The median duration between ostomy creation and RA was 80 days (interquartile range; 62.5, 100.5). For each additional day of discontinuity, the odds of EA decreased by 2% (odds ratio [OR] = 0.980; 95% confidence interval [CI]: 0.962, 0.999; P = 0.038), and death increased by 4.2% (OR = 1.042; 95% CI: 1.010, 1.075; P = 0.009). For each additional mL/kg/day of enteral feeds at RA, the odds of EA increased by 7.5% (OR = 1.075; 95% CI: 1.027, 1.126, P = 0.002) and duration of hospital stay decreased by 0.35 days (slope coefficient = -0.351; 95% CI: -0.540, -0.163; P < 0.001)., Conclusion: Shorter duration of intestinal discontinuity and enteral nutrition before RA could positively influence EA and duration of stay in infants with IF and ostomy following RA., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: Enteral drug therapy is challenging in short bowel syndrome with intestinal failure (SBS-IF) because of unpredictable absorption. SEFA-6179 is an enterally administered medium-chain fatty acid analogue under development for intestinal failure-associated liver disease. We investigate the pharmacokinetics of two SEFA-6179 formulations in two large-animal models of SBS-IF, including a new pseudojejunostomy model., Methods: Twenty Yucatan minipigs were obtained. Half underwent pre-resection pharmacokinetic study with single-dose SEFA-6179 administration. All minipigs then underwent 90% jejunoileal resection, with either a jejunoileal anastomosis or bypass of the intraperitoneal colon with anastomosis just proximal to the rectum (pseudojejunostomy). On postoperative day 3, a single-dose pharmacokinetic study was performed., Results: Both SBS-IF models were well tolerated. Compared with the jejunoileal anastomosis minipigs, pseudojejunostomy minipigs had a more severe malabsorptive phenotype with weight loss by postoperative day 4 (+0.1 vs -0.9 kg, P = 0.03) and liquid diarrhea (Bristol 5 vs Bristol 7, P = 0.0007). Compared with pre-resection minipigs, both jejunoileal and pseudojejunostomy minipigs had lower total plasma exposure of SEFA-6179 measured by area under the curve (jejunoileal: 37% less, P = 0.049; pseudojejunostomy: 74% less, P = 0.0001). Peak plasma concentration was also lower in the pseudojejunostomy group compared with pre-resection (65% less, P = 0.04), but not lower in the jejunoileal group (P = 0.47)., Conclusion: In two SBS-IF minipig models, SEFA-6179 had substantially decreased absorption compared with pre-resection minipigs. Dose optimization for different intestinal anatomy and function may be required. We describe a new SBS-IF pseudojejunostomy model that may improve the translation of preclinical research to patients with SBS-IF who have enterostomies., (© 2023 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Introduction: The International Intestinal Failure Registry (IIFR) is an international consortium to study intestinal failure (IF) outcomes in a large contemporary pediatric cohort. We aimed to identify predictors of early (1-year) enteral autonomy., Methods: We included IIFR pilot phase patients. IF was defined by a parenteral nutrition need for at least 60 days due to a primary gastrointestinal etiology. The primary outcome was time to enteral autonomy achievement. We built a mixed-effects Weibull accelerated failure time model with random effects by center to analyze variables associated with enteral autonomy achievement with a primary outcome of time ratio (TR)., Results: We included 189 patients (82% with short bowel syndrome) representing 11 international centers. Cumulative incidence of early enteral autonomy was 51.6%, and death was 6.5%. In multivariable analysis, ostomy presence (TR, 2.63; 95% CI, 1.41-4.90) was associated with increased time to enteral autonomy achievement, and Asian/Indian (TR, 0.28; 95% CI, 0.10-0.81) and Pacific Islander race (TR, 0.34; 95% CI, 0.13-0.90) were associated with decreased time to enteral autonomy achievement. In a second model in the subset with measured percentage of bowel length remaining, ostomy presence (TR, 4.21; 95% CI, 1.90-9.33) was associated with increased time to enteral autonomy achievement, whereas greater percentage of bowel remaining (TR, 0.96; 95% CI, 0.94-0.98) was associated with decreased time to enteral autonomy achievement., Conclusions: Minimizing bowel resection at initial surgery and establishing bowel continuity by ostomy reversal can effectively decrease the time to early enteral autonomy achievement in children with IF., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: Reports of essential fatty acid deficiency (EFAD) in patients receiving parenteral nutrition (PN) and a composite lipid (mixed oil intravenous lipid emulsion [MO ILE]) are predominantly when managed by lipid restriction. The objective of this study was to determine the prevalence of EFAD in patients with intestinal failure (IF) who are PN dependent without lipid restriction., Methods: We retrospectively evaluated patients, ages 0-17 years, followed by our intestinal rehabilitation program between November 2020 and June 2021 with PN dependency index (PNDI) of >80% on a MO ILE. Demographic data, PN composition, PN days, growth, and plasma fatty acid profile were collected. A plasma triene-tetraene (T:T) ratio >0.2 indicated EFAD. Summary statistics and Wilcoxon rank sum test evaluated to compare between PNDI category and ILE administration (grams/kilograms/day). P < 0.05 was considered significant., Results: Twenty-six patients (median age, 4.1 years [interquartile range (IQR) = 2.4-9.6]) were included. The median duration of PN was 1367 days (IQR = 824-3195). Sixteen patients had a PNDI of 80%-120% (61.5%). Fat intake for the group was 1.7 g/kg/day (IQR = 1.3-2.0). The median T:T ratio was 0.1 (IQR = 0.1-0.2) with no values >0.2. Linoleic and arachidonic acid were low in 85% and 19% of patients, respectively; however, Mead acid was normal in all patients., Conclusion: This report is the largest to date on the EFA status of patients with IF on PN. These results suggest that, in the absence of lipid restriction, EFAD is not a concern when using MO ILEs in children receiving PN for IF., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: Patients with intestinal failure (IF) often present with abnormal body composition characterized by high fat mass. However, the distribution of fat and its association with the development of IF-associated liver disease (IFALD) remain unclear. This study aims to investigate the body composition and its relationship with IFALD in older children and adolescents with IF., Methods: This retrospective case-control study enrolled patients with IF receiving parenteral nutrition (PN) at Keio University Hospital who initiated PN before the age of 20 years (cases). The control group included patients with abdominal pain, with available computed tomography (CT) scan and anthropometric data. CT scan images of the third lumbar vertebra (L3) were used for body composition analysis and compared between the groups. Liver histology was compared with CT scan findings in IF patients who underwent biopsy., Results: Nineteen IF patients and 124 control patients were included. To account for age distribution, 51 control patients were selected. The median skeletal muscle index was 33.9 (29.1-37.3) in the IF group and 42.1 (39.1-45.7) in the control group (P < 0.01). The median visceral adipose tissue index (VATI) was 9.6 (4.9-21.0) in the IF group and 4.6 (3.0-8.3) in the control group (P = 0.018). Among the 13 patients with IF who underwent liver biopsies, 11 (84.6%) had steatosis, and there was a tendency for fibrosis to correlate with VATI., Conclusion: Patients with IF exhibit low skeletal muscle mass and high visceral fat, which may be related to liver fibrosis. Routine monitoring of body composition is recommended., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: The objective of the study was to compare bioelectrical impedance analysis (BIA) and skinfolds with dual energy x-ray absorptiometry (DXA) in the assessment of body composition of children with intestinal failure. DXA is the reference method for body composition assessment in clinical settings., Methods: Children aged 1-18 years with intestinal failure whohave DXA as part of routine clinical monitoring were eligible. BIA measured total body water on the same day as DXA. Skinfold measurements were taken at four sites: triceps, biceps, subscapular, and suprailiac. Percentage of fat mass (%FM) and fat-free mass (%FFM) were derived from resistance and reactance measured by BIA by using age-specific equations. Percentage of FM was calculated from skinfold measures by using age-specific equations. Data on patient characteristics, intestinal failure-related factors, and feeding method were collected. Paired t test examined differences in %FM and %FFM and Bland-Altman analysis determined the agreement between BIA, skinfolds, and DXA. Marginal linear model assessed the effect of age, sex, and feeding method on the difference in body composition obtained between DXA and BIA and between DXA and skinfolds., Results: Sixty-eight children with intestinal failure, mean age 8.9 ± 4.2 years, were studied. There was no difference between %FFM and %FM obtained by DXA and BIA (P = 0.26), with a mean bias (95% CI) of -0.69 (-1.9 to 0.5) for %FFM. Sex and age were individually and jointly associated with the bias observed between DXA and BIA (P < 0.05). Skinfold and DXA measurements were significantly different (P < 0.05)., Conclusions: BIA is an acceptable clinical tool for assessing body composition in pediatric intestinal failure., (© 2023 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

As the majority of children with short bowel syndrome (SBS) and intestinal failure (IF) are now surviving into adulthood, there is a paradigm shift from short-term management to long-term outcomes and a growing need to focus on healthcare transition (HCT). It is imperative that adolescents and young adults with SBS and IF receive disease education, empowerment, and support as they navigate the transition from pediatric to adult care. Furthermore, both pediatric and adult healthcare providers who manage these patients should be aware of the challenges faced by this population, barriers to their HCT, and strategies to overcome them. This article reviews the literature on HCT in children with chronic illnesses, discusses barriers to HCT in SBS/IF, identifies the important constituents of the transition process in SBS/IF, and provides recommendations for the successful and smooth transition of the pediatric patient to the adult healthcare environment. Structured and multicomponent HCT programs should become the standard of care to ensure uninterrupted high-quality care across the life span for patients with SBS/IF., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: Longitudinal changes in bone health in children with intestinal failure (IF) are unclear. We aimed to better understand the trajectory of bone mineral status over time in children with IF and identify clinical factors that influence the trajectory., Methods: Clinical records of patients attending the Intestinal Rehabilitation Center of Cincinnati Children's Hospital Medical Center between 2012 and 2021 were reviewed. Children diagnosed with IF before age 3 years with at least two lumbar spine dual-energy x-ray absorptiometry scans were included. We abstracted information on medical history, parenteral nutrition, bone density, and growth. We calculated bone density z scores with and without adjustment for height z scores., Results: Thirty-four children with IF met inclusion criteria. Children were shorter than average with a mean height z score of -1.5 ± 1.3. The mean bone density z score was -1.5 ± 1.3 with 25 of the cohort having a z score < -2.0. After height adjustment, the mean bone density z score was -0.42 ± 1.4 with 11% below -2.0. Most dual-energy x-ray absorptiometry scans (60%) had a feeding tube artifact. Bone density z scores increased slightly with age and lower parenteral nutrition dependency and were higher in scans without an artifact. Etiologies of IF, line infections, prematurity, and vitamin D status were not associated with height-adjusted bone density z scores., Conclusion: Children with IF were shorter than expected for age. Deficits in bone mineral status were less common when adjusting for short stature. Etiologies of IF, prematurity, and vitamin D deficiency were not associated with bone density., (© 2023 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: Ethanol lock therapy (ELT) decreases central line-associated bloodstream infections; however, the effect on mechanical catheter complications is unclear. In recent years, ELT has become unavailable for many patients, often resulting in high-risk patients switching back to heparin locks. We investigated the impact of ELT on mechanical catheter complications during this period., Methods: We performed a retrospective cohort study of the Boston Children's Hospital intestinal rehabilitation program from January 1, 2018, to December 31, 2020. Pediatric patients with a central venous catheter requiring parenteral support for 3 months were included. The primary outcome was the composite rate of mechanical catheter complications (repairs and replacements)., Results: The pediatric intestinal failure cohort consisted of 122 patients. Forty-four percent received ELT for the entirety of the study period, 29% used only heparin locks, and 27% used ELT and heparin locks at different periods. During ELT use, there was 1.65 times the risk of mechanical catheter complications (composite outcome of repairs and replacements) compared with heparin locks (adjusted incidence rate ratio [aIRR] = 1.65, 95% CI = 1.18-2.31). Current ELT use was associated with 2.3 times the risk of catheter repairs (aIRR = 2.30, 95% CI = 1.36-3.89) but no significant increase in catheter replacement risk (aIRR = 1.41, 95% CI = 0.91-2.20)., Conclusion: In the largest pediatric intestinal failure cohort evaluated to date, the use of ELT, compared with heparin locks, increased the risk of mechanical catheter complications. Mechanical complications carry morbidity requiring urgent clinic or emergency department visits and additional procedures. The investigation of alternative lock solutions is warranted., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: Intestinal failure-associated liver disease (IFALD) occurs in up to 50% of neonates treated with prolonged parenteral nutrition. Preventative strategies for IFALD include soybean oil lipid emulsion (SOLE) minimization and use of mixed-oil intravenous lipid emulsions (ILE). We conducted a pilot study prospectively comparing these two ILE strategies in the prevention of IFALD in neonates who required abdominal surgery., Methods: We randomized eligible neonates to SOLE at 1 g/kg/day (SOLE Min) or mixed-oil ILE containing fish oil (MOLE) at 3 g/kg/day. These treatment groups were also compared with historic controls who received SOLE at 2-3 g/kg/day (SOLE Historic). We defined IFALD as a direct bilirubin >2 mg/dl on two measurements. Secondary outcomes included laboratory, growth, clinical, and nutrition outcomes., Results: A total of 24 prospective and 24 historic patients were included. There was no difference in the rate of IFALD. However, there was a difference in the weekly change of direct bilirubin levels (SOLE Historic +0.293 mg/dl/week vs MOLE, P < 0.001; SOLE Min +0.242 mg/dl/week vs MOLE, P < 0.001). The MOLE group also had a lower direct bilirubin at study completion (SOLE Historic, 1.7 ± 1.7 mg/dl; SOLE Min, 1.6 ± 1.4 mg/dl; MOLE, 0.4 ± 0.4 mg/dl; P = 0.002) and received greater total calories (P = 0.008)., Conclusion: The rate of IFALD did not differ when comparing ILE strategies in neonates requiring abdominal surgery. However, the MOLE group maintained significantly lower direct bilirubin levels over time while receiving increased calories. This pilot study highlights the need for further randomized controlled trials comparing these ILE strategies., (© 2023 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: Improved survival rates for children with intestinal failure (IF) have resulted in an increased population of children receiving long-term parenteral nutrition (PN). Our objective was to determine burden on caregivers of children with IF receiving long-term PN., Methods: We performed a cross-sectional study of caregivers of children with IF receiving long-term PN in our intestinal rehabilitation program. A healthy comparison group matched on age of the child was enrolled. All participants completed standardized questionnaires, including the Parental Stress Index - Short Form (PSI-SF), Hospital Anxiety and Depression Scale (HADS), and PedsQL Family Impact Module (PedsQL FIM). Univariate analysis was completed using a Student t test and chi-square, with an alpha value of <0.05 considered significant., Results: Thirty-eight caregivers of children with IF and 29 caregivers of healthy children consented, with response rates of 89% and 96.5%, respectively. Our study demonstrated increased stress for caregivers compared with comparison parents (PSI-SF total score of 83 [SD = 26.8] vs 62.9 [SD = 13.5]; P < 0.01). Caregivers had increased anxiety (HADS anxiety score of 9.3 [SD = 4.8] vs 6.7 [SD = 3.2]; P = 0.02) and higher depression scores (HADS depression score of 6.3 [SD = 4.3] vs 4.1 [SD = 2.6]; P = 0.02) compared with the comparison group. Caregivers of children with IF demonstrated decreased health-related quality of life (HRQoL) (reduced PedsQL FIM total score of 50.6 [SD = 18.2] vs 84.1 [SD = 20.5]; P < 0.01)., Conclusions: Our results demonstrated significant burden of care in caregivers of children with IF receiving long-term PN, with elevated stress, anxiety, and depression and decreased HRQoL., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Most data on anemia in children with intestinal failure (IF) have been obtained from studies in which the data were collected at a single point in time. We aimed to identify the frequency of anemia and factors associated with hemoglobin levels in children with IF during their course of home parenteral nutrition., Methods: We performed a longitudinal cohort study of patients with IF followed up at a pediatric intestinal rehabilitation center. Outcome variables were hemoglobin levels and prevalence of anemia during the follow-up period. The exposure variables were age, duration of parenteral nutrition, chronic disease, and serum concentrations of C-reactive protein, iron, copper, selenium, vitamins A, D, B 12 , and folic acid., Results: Twenty-five children with a median time of receiving parenteral nutrition of 40.7 months were included. A median (and interquartile range) of 40.7 (25.2-58) hemoglobin measurements were performed per patient. Mean (SD) hemoglobin was 10.7 (1.8) g/dL at baseline and 11.6 (0.9) g/dL in the last observation (paired t test, P = 0.07); 32% of patients had mean hemoglobin values below the lower limit for age. In a multivariable predictive model, having C-reactive protein >1 mg/dL was associated with a decrease of 0.57 g/dL in hemoglobin (95% CI, -0.90 to -0.24, P = 0.01), and an increase of 1 mg/L in vitamin A concentration was associated with the increase of 0.93 g/dL in Hb level (95% CI, 0.24-1.61; P = 0.008)., Conclusion: Anemia affects almost one-third of children with IF and its frequency decreases during the follow-up period. Hemoglobin levels are associated with inflammatory response and serum micronutrient concentrations., (© 2023 American Society for Parenteral and Enteral Nutrition.)

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., (© 2022 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: Long-term parenteral nutrition (PN) can lead to intestinal failure-associated liver disease (IFALD). Omega-3 (n-3) polyunsaturated fatty acids (PUFAs) eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) were shown to prevent IFALD. EPA-derived and DHA-derived oxylipins could contribute to this protective effect., Methods: We analyzed the effect of parenteral fish oil on oxylipins in patients with chronic intestinal failure receiving PN (n = 8). Patients first received no fish oil for 8 weeks and then switched to PN with 25% of fat as fish oil for another 8 weeks. Fatty acid profiles of red blood cells, PUFA-derived oxylipins generated by cyclooxygenase, lipoxygenase (LOX), and cytochrome P450 (CYP) pathways, inflammatory markers, and liver function were assessed before and during fish-oil PN., Results: EPA plus DHA in erythrocytes (the Omega-3 Index) was high with a median of 11.96% at baseline and decreased to 9.57% without fish oil in PN. Addition of fish oil in PN increased the median Omega-3-Index to 12.75%. EPA-derived and DHA-derived CYP-dependent and LOX-dependent metabolites increased significantly with fish oil in PN, with less pronounced changes in arachidonic acid and its oxylipins. There were no significant changes of inflammation and liver function parameters., Conclusions: This study shows that fish oil-containing PN leads to primarily CYP- and LOX-dependent n-3 PUFA-derived inflammation-dampening oxylipins arising from EPA and DHA. Within this short (16-week) study, there were no significant changes in inflammation and clinical readout parameters., (© 2022 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: Short bowel syndrome is the most common cause of intestinal failure (IF) in infants. We aimed to evaluate growth, nutrition intakes, and predictors of weaning from parenteral nutrition (PN) of infants with IF., Methods: Clinical parameters, nutrition intakes, body weight and length z-scores were compared monthly from the 1st to 12th and at 18 and 24 months among infants receiving PN and those weaned. Logistic regression analysis was conducted to explore the predictors of weaning., Results: We included 23 infants (10/23 weaned). Median [range: minimum; maximum] birth weight and gestational age were 1620 [590; 3490] g and 31 [24; 39] weeks, respectively. All infants showed growth retardation with similar median delta weight z-score from birth to discharge: -1.48 [-1.92; -0.94] in not-weaned and -1.18 [-2.70; 0.31] in weaned infants (P = 0.833) and a subsequent regain after the discharge: 0.20 [-3.47; 3.25] and 0.84 [-0.03; 2.58], respectively (P = 0.518). No differences in length z-score were found. After the sixth month, infants weaned from PN received lower PN energy and protein intakes compared with those not-weaned. Infants weaned from PN showed lower PN dependency index (PNDI%) from 5 months onward (45% for weaned and 113% for not-weaned infants at 5 months: P < 0.001). The Belza score, a predictor of enteral autonomy computed at 6 months, is associated with being weaned from PN within 24 months (odds ratio: 1.906; P = 0.039)., Conclusion: Infants weaned and not-weaned showed similar growth patterns. Our findings support the clinical relevance of Belza score and PNDI% as predictors of weaning from PN., (© 2022 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Intestinal failure (IF) is a rare disease that requires ongoing intravenous supplementation to sustain growth and health. Advancements in parenteral nutrition (PN) and central venous access in the 1960s and 1970s transformed a life-limiting disease to a chronic one in which patients are able to administer hydration, electrolytes, micronutrients, and macronutrients in the comfort of their home. However, despite ongoing advancements in the field of home PN (HPN), complications-whether related to central venous catheters or PN itself-remain common and can be associated with significant morbidity and mortality. Central venous access can be associated with thrombosis, central line-associated bloodstream infection, or damage and can result in loss of access over time. PN can be associated with IF-associated liver disease or hyperglycemia. The key to preserving central venous access and quality of life and maintaining health for patients with chronic IF (CIF) is education focused on prevention and prompt management of CIF complications as they arise. This education typically takes place at the time of initiation of HPN, either in the hospital setting or in the patient's home. The present manuscript describes the historical progression of HPN, prevalence and characteristics of CIF, and an in-depth discussion of the most common catheter-related and PN-related complications and their management, along with a discussion of our education and training process., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Introduction: Patients with chronic intestinal failure (IF) have a low degree of physical activity, decreased muscle mass, and decreased muscle strength, leading to a high risk of sarcopenia. We aimed to test the prevalence of sarcopenia by the use of SARC-F and EWGSOP and to investigate the association between the two at baseline and after 12 weeks of an exercise intervention., Methods: Thirty-one patients with chronic IF completed 12 weeks of three weekly home-based individualized exercise sessions. Body composition was measured by bioimpedance analysis and physical function by handgrip strength (HGS) and timed up-and-go (TUG). Sarcopenia was assessed by SARC-F and EWGSOP. Multiple regression analysis was used to test for the association between the two tools., Results: The prevalence of sarcopenia measured by EWGSOP was 59%. This prevalence did not change after the intervention. At baseline, 38.8% of patients were screened as at risk for sarcopenia by SARC-F. This decreased to 29.0% after the intervention (P < 0.001). A statistically significant increase was achieved in muscle mass (P = 0.017) and muscle mass index (P = 0.016). Furthermore, both TUG (P = 0.033) and HGS (P = 0.019) improved., Conclusions: Sarcopenia is prevalent in patients with chronic IF. EWGSOP finds more patients to be at risk of sarcopenia than SARC-F but was not sufficiently sensitive to measure changes induced by the physical intervention. The significant change in SARC-F may illustrate that patients, themselves, find an improvement in self-perceived health., (© 2022 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: A fungal-related catheter-related bloodstream infection (CRBSI) is less frequent than those induced by bacteria. In the past, a single episode of fungal CRBSI has been used as a marker of home parenteral nutrition (HPN) failure and thus a possible indication for intestinal transplantation., Methods: Survival outcomes were assessed from a prospectively maintained database of patients initiated on HPN for underlying chronic intestinal failure between 1993 and 2018, with a censoring date of December 31, 2020. Cox regression was performed to assess predictors of mortality with univariable and multivariable analysis., Results: A total of 1008 patients were included in the study, with a total of 1 364 595 catheter days. There were 513 CRBSI events recorded in 262 patients, equating to a CRBSI rate of 0.38/1000 catheter days. A total of 38/262 (14.5%) patients had at least one episode of fungal CRBSI, whereas 216/262 (82.4%) had at least one bacterial but no fungal CRBSI. The median time between HPN initiation and the first CRBSI episode was 20.6 months (95% confidence interval, 16.5-24.1). Episodes of fungal or bacterial CRBSI and the number of CRBSI episodes were not associated with increased mortality. Overall, 15 CRBSI-related deaths were observed in the observation period (0.01 CRBSI deaths/1000 catheter days), two of these were fungal in origin., Conclusion: The occurrence of a fungal CRBSI does not increase the risk of death compared with patients who have bacterial CRBSI or those without a CRBSI event., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: The proadaptive effects of glucagon-like peptide-2 (GLP-2) include stimulation of intestinal mucosal growth as well as intestinal blood flow and angiogenesis. We have recently reported that daily subcutaneous injections of glepaglutide, a long-acting GLP-2 analog, improved intestinal absorptive function in patients with short bowel syndrome (SBS). As secondary and exploratory end points, the effects of glepaglutide on intestinal morphology and perfusion are reported., Methods: The following assessments were done in 18 patients with SBS in a randomized, crossover, dose-finding, phase 2 trial before and after three weeks of treatment with glepaglutide: plasma citrulline and mucosa biopsies to assess changes in (1) intestinal morphology by immunohistochemistry and (2) gene expressions associated with absorption, proliferation, and markers of tight-junction integrity by quantitative polymerase chain reaction. Intestinal perfusion was assessed in stoma nipples by laser speckle contrast imaging and quantitative fluorescence angiography with indocyanine green., Results: In the 1- and 10-mg dose groups, glepaglutide significantly increased plasma citrulline by 15.3 µmol/L (P = 0.001) and 15.6 µmol/L (P = 0.001), respectively. Trends toward an increase in villus height, crypt depth, and epithelium height were seen in the same groups. No significant changes were seen in gene expressions or intestinal perfusion., Conclusion: The increase in plasma citrulline and the morphological improvements may partly account for improvement in the intestinal absorptive function. However, the finding of a stability in perfusion after three weeks of treatment with glepaglutide may have been preceded by a more profound acute-phase increase in intestinal perfusion at treatment initiation., (© 2022 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: Both fish-oil lipid injectable emulsion (FO-ILE) and mixed-oil lipid injectable emulsion (MO-ILE) are key components of parenteral nutrition and require importation into Japan, and they are easily oxidized after opening. Given the small daily volumes of these lipids dispensed in infants and children with intestinal failure (IF), the purpose of the study was to identify the optimal storage method., Methods: Lipids were prepared in polypropylene syringes in the following manner: air-sealing and photoprotection, air-sealing only, photoprotection only, and uncovered. Samples were stored for 14 days at 4°C or 26°C. The degree of oxidative degradation was evaluated by measuring malondialdehyde (MDA) concentration and pH and comparing them to the values measured immediately after opening., Results: For FO-ILE, the increase in MDA concentration for 14 days was insignificant in air-sealed samples, regardless of photoprotection (+0.45 μM, P = 1.0) or no photoprotection (+0.52 μM, P = 1.0). This trend was more pronounced at 4°C than at 26°C (P < 0.01). The maximum pH decrease was 0.08 at 4°C. MO-ILE exhibited an insignificant increase in MDA concentration for 14 days with air-sealed samples, regardless of photoprotection (+0.36 μM, P = 0.11) or no photoprotection (+0.33 μM, P = 0.76). This trend was more pronounced at 4°C than at 26°C (P < 0.01). The maximum pH decrease was 0.12 at 4°C. For soybean-oil lipid injectable emulsion, the trend was similar with no considerable deterioration., Conclusion: Syringe-dispensed FO-ILE and MO-ILE stored under airtight refrigeration remained undeteriorated for 14 days. Our results are considered clinically valuable when supplying these expensive resources for infants with IF., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Small bowel bacterial overgrowth (SBBO) is a common, but difficult to diagnose and treat, problem in pediatric short bowel syndrome (SBS). Lack of clinical consensus criteria and unknown sensitivity and specificity of bedside diagnosis makes research on this potential SBS disease modifier challenging. The objective of this research was to describe clinical care of SBBO among international intestinal rehabilitation and nutrition support (IR&NS) providers treating patients with SBS., Methods: A secure, confidential, international, electronic survey of IR&NS practitioners was conducted between March 2021 and May 2021. All analyses were conducted in the R statistical computing framework, version 4.0., Results: Sixty percent of respondents agreed and 0% strongly disagreed that abdominal pain, distension, emesis, diarrhea, and malodorous stool, were attributable to SBBO. No more than 20% of respondents strongly agreed and no more than 40% agreed that any sign or symptom was specific for SBBO. For a first-time diagnosis, 31 practitioners agreed with use of a 7-day course of a single antibiotic, with a majority citing grade 5 evidence to inform their decisions (case series, uncontrolled studies, or expert opinion). The most common first antibiotic used to treat a new onset SBBO was metronidazole, and rifaximin was the second most commonly used. One hundred percent of respondents reported they would consider a consensus algorithm for SBBO, even if the algorithm may be divergent from their current practice., Conclusion: SBBO practice varies widely among experienced IR&NS providers. Development of a clinical consensus algorithm may help standardize care to improve research and care of this complex problem and to identify risks and benefits of chronic antibiotic use in SBS., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Home parenteral nutrition (HPN) is the recommended treatment in patients with chronic intestinal failure (CIF). HPN is associated with a worsening of patients' quality of life and has a dramatic impact on personal and familial daily life. Little is known about the informal caregiver burden of patients receiving HPN., Aim: To assess informal caregiver burden and the factors associated with moderate-to-severe caregiver burden in patients treated with HPN., Methods: Informal caregivers of consecutive patients treated with HPN in a French nutrition referral center were invited to participate in a survey between January 2021 and June 2021. They had to fill out an anonymous standardized self-questionnaire evaluating burden by the Zarit Burden Interview (ZBI) and depression and anxiety symptoms by the Hospital Anxiety and Depression Scale (HADS) score since HPN introduction., Results: Among the 87 HPN patients having informal caregivers, 53 (61%) completed the questionnaire. Thirty (57%) informal caregivers were women. The caregiver's median age was 62 years (IQR, 21.0). Twelve (22%) expressed moderate to severe burden (ZBI score, 41-88). In a multivariate analysis, the caregiver's male gender (odds ratio [OR], 16.45; 95% CI, 2.30-238.75; P = 0.014) and the number of simultaneous infusions per day >1 (OR, 9.92; 95% CI, 1.35-121.60; P = 0.0038) were associated with a moderate to severe burden. Twenty caregivers reported anxious and depressive symptoms with an elevated HADS score., Conclusion: In this prospective survey, a minority of informal caregivers of patients with CIF being treated with HPN expressed moderate to severe burden. The caregiver's male gender was associated with a higher burden in patients with CIF., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Iron supplementation is required for pediatric patients with intestinal failure (IF). There is a paucity of literature on optimal iron formulation and outcomes in this patient population that requires ongoing supplementation. The aim of this study was to assess outcomes in pediatric patients with IF receiving iron sucrose (IS) vs ferric carboxymaltose (FCM) iron infusions., Methods: This was a single-center observational cohort study of pediatric patients with IF requiring ongoing intravenous iron supplementation. Patients were transitioned from IS to FCM as iron therapy. Longitudinal linear mixed-effects models and generalized estimating equations were used to compare outcomes, including hematologic, iron, and growth parameters for 12-month treatment duration on each iron formulation. Adverse effects were descriptively summarized., Results: Twenty-three patients were included. Sixteen received IS and later switched to FCM, five received IS only, and two received FCM only. Most patients' etiology of IF was short bowel syndrome (FCM: 81%, IS: 83%). No differences were seen over time for iron, hematologic, and growth metrics between IS and FCM. The median number of infusions over 12 months for those taking IS was 15 (interquartile range [IQR] = 13-26) and 2 for FCM (IQR = 1-2). Asymptomatic hypophosphatemia was noted in both groups. Similar central line-associated bloodstream infection rates were noted., Conclusion: IS and FCM infusions both maintained hematologic and iron parameters with no significant difference noted between the two types of iron, though the number of FCM infusions was significantly less. No significant adverse effects were noted., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Introduction: Catheter-related bloodstream infections (CRBSIs) remain the commonest complication associated with home parenteral nutrition (HPN). Although the management outcomes of CRBSIs have been extensively reported by specialized intestinal failure (IF) centers, there are minimal data reporting CRBSI outcomes for HPN-dependent patients admitted to nonspecialized hospitals., Method: This was an observational study from a prospectively maintained database of CRBSIs in HPN-dependent patients managed outside of a specialized IF center., Results: Three hundred and six patients from a total cohort of 1066 HPN-dependent patients suffered from 489 CRBSI events from 2003 to 2021; after 2017, 71 of these events were managed at the patient's local, nonspecialized hospital and the remainder at the specialized IF center. From 2017 to 2021, salvage of the central venous catheter (CVC) with antimicrobial therapy was attempted in 32 out of 71 (45.1%) patients admitted to the nonspecialized hospital, with successful salvage recorded in 23 (71.8%) cases. Notably, CVC salvage was attempted more commonly (77 out of 103 [74.8%]; P = 0.004 vs nonspecialized hospital), with a better salvage success rate (64 out of 77 [83.1%] P = 0.01 vs nonspecialized hospital) in patients who were admitted to the specialized IF center., Conclusion: In some instances, CRBSIs can be effectively managed when patients presenting to a nonspecialized hospital; however, overall salvage is more likely to be successful in the specialized setting. Further development of clinical and educational networks between IF centers and patients' local hospitals aimed at standardizing care may lead to improved CRBSI outcomes., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Newer intravenous lipid emulsions (ILEs), such as fish oil-based intravenous lipid emulsions (FO-ILEs) and soybean oil, medium-chain triglycerides, olive oil, and fish oil-based intravenous lipid emulsions (SMOF-ILEs), provide alternatives to soybean oil-based intravenous lipid emulsions (SO-ILEs). We explored current ILE practice patterns among intestinal rehabilitation and transplant centers., Methods: A survey was developed addressing ILE availability, ILE preference in clinical scenarios, and factors influencing ILE choice. This survey was reviewed locally and by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Intestinal Rehabilitation Special Interest Group, the Intestinal Rehabilitation and Transplant Association scientific committee, and the American Society of Parenteral and Enteral Nutrition pediatric intestinal failure section research committee. We recruited providers nationally and internationally from centers with and without intestinal transplant programs., Results: We included 34 complete responses, 29 from the United States. Sixteen centers performed intestinal transplants. All centers had access to SMOF-ILEs, 85% had access to FO-ILEs, and 91% had access to SO-ILEs. In new patients, 85% use SMOF-ILEs as the first choice ILE. In those with new intestinal failure-associated liver disease (IFALD), FO-ILE was preferred to SMOF-ILE (56% vs 38%). In those developing IFALD on SMOF-ILE, 65% switched to FO-ILE, whereas 24% remained on SMOF-ILE., Conclusions: Centers have routine access to alternative ILEs, and these are quickly replacing SO-ILEs in all circumstances. Future work should focus on how this shift in practice affects outcomes to provide decision support in specific clinical scenarios., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Apraglutide is a novel long-acting glucagon-like peptide-2 (GLP-2) analog designed for once-weekly subcutaneous dosing, with the potential to increase fluid and nutrient absorption by the remnant intestine of patients who have short bowel syndrome (SBS) with intestinal insufficiency (SBS-II) or intestinal failure (SBS-IF). This trial investigated the safety and effects on intestinal absorption of apraglutide in patients with SBS-II and SBS-IF., Methods: In this open-label, phase 1 and 2 trial, adult patients with SBS-II (n = 4) or SBS-IF (n = 4) and a fecal output of ≥1500 g/day received once-weekly subcutaneous 5 mg apraglutide for 4 weeks. Safety was the primary end point. Secondary end points included change from baseline in intestinal absorption of wet weight (indicative of fluid absorption), electrolytes, and energy (by bomb calorimetry) measured by inpatient metabolic balance studies., Results: Common treatment-related adverse events were decreased gastrointestinal (GI) stoma output (n = 6), stoma complications (n = 6), GI stoma complications (n = 5), nausea (n = 5), flatulence (n = 4), abnormal GI stoma output (n = 4), polyuria (n = 3), and abdominal pain (n = 3). The only treatment-related serious adverse event (experienced in one patient) was abdominal pain. Apraglutide significantly increased wet weight and energy absorption by an adjusted mean of 741 g/day (95% CI, 194 to 1287; P = 0.015) and 1095 kJ/day (95% CI, 196 to 1994; P = 0.024), respectively. Sodium and potassium absorption significantly increased by an adjusted mean of 38 mmol/day (95% CI, 3 to 74; P = 0.039) and 18 mmol/day (95% CI, 4 to 32; P = 0.020), respectively., Conclusion: Once-weekly 5 mg apraglutide was well tolerated in patients with SBS-II and SBS-IF and significantly improved the absorption of fluids, electrolytes, and energy., (© 2022 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: Although international guidelines support the use of commercially available multichamber bags (MCBs), the majority of home parenteral nutrition (HPN) in the United Kingdom has been compounded PN, tailored to the patient. However, national capacity limitations in aseptic facilities have necessitated the increased use of MCBs. There are no studies evaluating the appropriateness or benefits of using a "hybrid" regimen incorporating both MCBs and compounded PN in patients already established on compounded HPN., Methods: This was a cross-sectional audit evaluation conducted on September 1, 2021, at a national United Kingdom reference center. All HPN-dependent adults prescribed HPN for chronic intestinal failure were assessed by a multidisciplinary team for their potential of being switched to a "hybrid" regimen of MCBs and compounded PN., Results: Of 180 patients currently receiving compounded HPN that included intravenous nitrogen with glucose ± lipid, 65 (36.1%) were deemed clinically suitable for a hybrid PN regimen, with minimal variance in PN constituents per week (volume 0%, non-nitrogen kilocalories 0%, nitrogen 0%, fat -0.2%, glucose 0%, sodium 0%, potassium 0%, calcium 0%, magnesium 0%, and phosphate -0.1%) and requiring no additional central venous catheter manipulations. The potential reduction in compounded PN would reduce by 3627 bags per year, equating to a cost saving of £141,453 per year (equivalent to $178,885)., Conclusion: Wider use of hybrid MCB/compounded HPN regimens could lead to a reduction in the need for compounded PN to be produced by aseptic facilities. Further evaluation of acceptability and tolerance of hybrid regimens by patients already receiving compounded HPN is required., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Catheter-related venous thrombosis (CRVT) is a severe complication of home parental nutrition. Although primary prevention of CRVT is crucial, there is no consensus on anticoagulant use to prevent this adversity. The aim was to compare CRVT risk in patients with chronic intestinal failure (CIF) in the presence or absence of anticoagulants, and to identify CRVT risk factors., Methods: This retrospective cohort study comprised adult patients with CIF with a central venous access device (CVAD) between 2010 and 2020 that were treated at our national CIF referral center. Analyses were performed at a CVAD level., Results: Overall, 1188 CVADs in 389 patients were included (540.800 CVAD days). Anticoagulants were used in 403 CVADs. In total, 137 CRVTs occurred in 98 patients, resulting in 0.25 CRVTs/1000 CVAD days (95% CI, 0.22-0.29). Anticoagulant use was associated with a decreased CRVT risk (odds ratio [OR] = 0.53; 95% CI, 0.31-0.89; P = 0.02). Left-sided CVAD insertion (OR = 2.00; 95% CI, 1.36-2.94), a history of venous thrombosis (OR = 1.73; 95% CI, 1.05-2.84), and a shorter period postinsertion (OR = 0.78; 95% CI, 0.65-0.92) were independently associated with an increased CRVT risk., Conclusion: Anticoagulants decreased the CRVT risk. In addition, we identified left-sided vein insertion, a history of venous thrombosis, and a shorter period post-CVAD insertion as CRVT risk factors. Further prospective studies should provide guidance whether prophylactic anticoagulant use, especially in higher-risk patients with a left-sided CVAD or a history of venous thrombosis, is justified., (© 2021 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: This study investigated the prevalence, characteristics, and management of patients with chronic intestinal failure (CIF) in the United States in 2012-2020, based on parenteral support (PS) prescription claims and healthcare utilization., Methods: Patients with CIF were identified from the Integrated DataVerse® claims database if they had at least two PS prescriptions within 6 months and a relevant diagnosis. Analysis included prevalence and characteristics of patients with CIF, their travel distance to receive PS prescriptions, and the distribution of PS providers and their prescribing history., Results: Up to 24,048 patients with CIF were identified, equivalent to 75 patients per million. CIF affected people of all ages, being more prevalent in women than in men. Many providers signed PS orders for small patient groups over short time periods, whereas few providers signed PS orders for large patient groups long term, indicating a lack of centralization. The distribution of PS providers suggested a disparity in healthcare coverage in rural vs urban areas, leading to patients traveling considerable distances to receive PS prescriptions. This may be exacerbated by a decline of providers with expertise in CIF and nutrition., Conclusions: Healthcare disparities for patients with CIF have likely been obscured by the lack of CIF-specific diagnostic and procedure codes, obliging providers to code for their patients under other codes. Effective policy changes, including centralized care, revision of reimbursement models, and expansion of nutrition-focused education in addition to the newly introduced International Classification of Diseases codes, are needed to provide the best care for patients., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Patients with short-bowel syndrome and intestinal failure (SBS-IF) require parenteral support (PS) and experience various symptoms and comorbidities. This survey assessed the impact of SBS-IF and PS on patients and their health-related quality of life (HRQoL)., Methods: An online survey of adult patients who had a self-reported clinician diagnosis of SBS-IF and were receiving PS was conducted in France, Germany, Italy, the UK, and the USA. Patients reported symptoms, comorbidities, and treatment satisfaction; the Work Productivity and Activity Impairment Questionnaire: Specific Health Problem (WPAI:SHP) and the Home Parenteral Nutrition-Quality of Life (HPN-QoL) questionnaire assessed impact on work and HRQoL, respectively., Results: Patients (N = 181; aged 52.0 ± 15.1 years; 56.9% women) experienced fatigue (75.1%), anemia (49.7%), and difficulty spending time with family (36.5%) and friends (30.4%). A total work productivity loss of 37.5% was calculated in patients reporting employment (29.3%). Patients typically (64.0%) reported some degree of satisfaction with their PS treatment. Almost two-thirds (59.7%) reported that their PS was either "not," "a little," or "moderately" convenient. The mean HPN-QoL scores were higher for patients who were satisfied with treatment (n = 116; 17.1 ± 21.0 [median, 16.7; interquartile range, 0.0-31.7]) than for patients who were dissatisfied/neither (n = 65; 1.7 ± 19.7 [median, 0.0; interquartile range, -13.3-13.3])., Conclusions: Patients with SBS-IF who are receiving PS experience burdensome symptoms and comorbidities and report impacts on work productivity and time spent with friends and family. This study can increase awareness of the impacts of SBS-IF and PS and how treatment satisfaction may influence patients' health and HRQoL., (© 2022 Takeda. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background: Children with intestinal failure (IF) receiving long-term parenteral nutrition (PN) require significant medical care, including high-risk procedures such as accessing a central venous catheter, with the majority provided by family caregivers in the home. This study sought to understand the experiences of family caregivers., Methods: This was a qualitative study of family caregivers of children with IF. Participants were recruited from an intestinal rehabilitation program to participate in virtual focus groups, which were recorded and transcribed. Thematic analysis was used to capture and describe experiences., Results: Thirteen caregivers providing care to 11 children participated in three virtual focus groups held between May and June 2020. Data analysis revealed five primary themes: (1) caregiving as a 24/7 commitment, (2) facing constant risk of death, (3) chronic illness creates difficult feelings and emotions, (4) effects on all aspects of family life, and (5) adapting and functioning as a family. The findings of this study pair well with the biopsychosocial model highlighting the need for multifaceted assessment and supports., Conclusion: The impact of caring for a child with IF receiving long-term PN is significant for caregivers and their families and has implications for all aspects of their lives. A better understanding of the impact on caregivers and their coping strategies lays the groundwork for the optimization of quality of life for caregivers and their family., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Short bowel syndrome (SBS) is considered a low prevalence disease. In Argentina, no registries are available on chronic intestinal failure (CIF) and SBS. This project was designed as the first national registry to report adult patients with this disease., Methods: A prospective multicenter observational registry was created including adult patients with CIF/SBS from approved centers. Demographics, clinical characteristics, nutrition assessment, home parenteral nutrition (HPN) management, surgeries performed, medical treatment, overall survival, and freedom from HPN survival were analyzed., Results: Of the 61 enrolled patients, 56 with available follow-up data were analyzed. At enrollment, the mean intestinal length was 59.5 ± 47.3 cm; the anatomy was type 1 (n = 41), type 2 (n = 10), and type 3 (n = 5). At the end of the interim analysis, anatomy changed to type 1 in 31, type 2 in 17, and type 3 in 8 patients. The overall mean time on HPN before enrollment was 33.5 ± 56.2 months. Autologous gastrointestinal reconstruction surgery was performed before enrollment on 21 patients, and afterward on 11. Nine patients (16.1%) were weaned off HPN with standard medical nutrition treatment; 12 patients received enterohormones, and 2 of them suspended HPN; one patient was considered a transplant candidate. In 23.7 ± 14.5 months, 11 of 56 patients discontinued HPN; Kaplan-Meier freedom from HPN survival was 28.9%. The number of cases collected represented 19.6 new adult CIF/SBS patients per year., Conclusion: The RESTORE project allowed us to know the incidence, the current medical and surgical approach for this pathology, as well as its outcome and complications at dedicated centers., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Registered dietitian nutritionists subjectively assess muscle loss as part of the nutrition-focused physical examination (NFPE), using guidelines to standardize malnutrition diagnosis. Computed tomography (CT) scans provide an objective measure of skeletal muscle mass and abdominal wall and visceral adipose tissue and can be used to determine skeletal muscle loss., Methods: In this retrospective review, our team compared muscle measurements including the psoas, paraspinal muscles, and abdominal wall muscle area at the level of the third lumbar vertebral body (using CT)-as well as visceral and subcutaneous adipose tissue measurements-before and after gut transplant with the malnutrition diagnosis found on the NFPE. We also examined the association between CT measurements and postoperative infection, length of stay, and mortality., Results: Forty-two patients were included in the study. Adipose tissue measurements on CT analysis were significantly lower in the malnutrition group compared with those without malnutrition (P ≤ 0.05) in both the pretransplant and posttransplant groups. Skeletal muscle size measurements were not significantly associated with malnutrition, but when adjusted for patients' height by calculating skeletal muscle index, an association between low skeletal muscle index scores and malnutrition diagnosis was found (P = 0.026). Pretransplant malnutrition diagnosis did not predict infection, length of stay, or mortality., Conclusion: Objective assessment of subcutaneous adipose tissue by CT analysis was significantly correlated with the subjective assessment of malnutrition by NFPE in both pretransplant and posttransplant patients. Skeletal muscle index scores were significantly lower in pretransplant patients who were diagnosed with malnutrition., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Extensive intestinal resection may lead to short bowel (SB) syndrome, resulting in intestinal insufficiency or intestinal failure (IF). Intestinal insufficiency and IF involve deficiency of the proglucagon-derived hormones glucagon-like peptide-1 (GLP-1) and GLP-2. Two major problems of SB are epithelial surface loss and accelerated transit. Standard treatment now targets intestinal adaptation with a GLP-2 analogue to enlarge absorptive surface area. It is possible that additional benefit can be gained from a combination of GLP-1 and GLP-2 activity, with the aim to enlarge intestinal surface area and slow intestinal transit., Methods: The GLP-1- and GLP-2-specific effects of the novel dual GLP-1 receptor (GLP-1R) and GLP-2 receptor (GLP-2R) agonist dapiglutide (rINN) were characterized in rodents. Furthermore, in a murine SB model of intestinal insufficiency with 40% ileocecal resection, the influence of dapiglutide on intestinal growth, body weight, food intake, volume status, and stool water content was tested against vehicle and sham-operated male mice., Results: Dapiglutide significantly improves oral glucose tolerance, reduces intestinal transit time, and promotes intestinal growth. In the SB mouse model, dapiglutide promotes body weight recovery, despite unchanged intake of liquid diet. Dapiglutide promotes significant intestinal growth, as indicated by significantly increased villus height as well as intestinal length. Furthermore, dapiglutide reduces stool water losses, resulting in reduced plasma aldosterone., Conclusion: Dapiglutide possesses specific and potent GLP-1R and GLP-2R agonist effects in rodents. In the murine SB model, combined unimolecular GLP-1R and GLP-2R stimulation with dapiglutide potently attenuates intestinal insufficiency and potentially also IF., (© 2021 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)