Discontinuation Of Parenteral Nutrition Research Articles

Effectiveness of Fistuloclysis in Nutritional Management of Enteroatmospheric Fistulas: A Retrospective Study at Santo Tomás Hospital, Panama.

Introduction Enteroatmospheric fistulas (EAF) present significant challenges in surgical management due to their complex nature and high mortality rate. Traditional approaches often rely on prolonged parenteral nutrition, but emerging evidence suggests the potential benefits of enteral nutrition via fistuloclysis, an underappreciated enteral nutrition route. This study aims to evaluate the effectiveness of nutritional therapy, specifically fistuloclysis, in patients with EAF managed at the Trauma Unit of Santo Tomás Hospital, Panama. Methods A retrospective analysis was conducted on nine male patients diagnosed with EAF between January 2016 and December 2020. Data on demographics, fistula characteristics, and nutritional management were collected through chart review. Descriptive statistics were used for analysis. Results We analyzed nine patients, all of whom received enteral nutrition (EN) via fistuloclysis in a median of 5.5 days from the diagnosis of EAF. Seven patients required parenteral nutrition (PN) at the beginning. The use of specialized enteral formulas, supplemented with hydrolyzed proteins and medium-chain triglycerides, facilitated discontinuation of PN once 80% of nutritional requirements were met via the enteral route, and EN was continued until definitive surgery. The median duration of PN was 34 days. No adverse effects related to EN were observed, whereas complications such as central venous catheter infections were reported in all cases requiring PN. Conclusion Fistuloclysis is a viable and effective alternative to traditional PN in patients with EAF. Specialized nutritional strategies, including the use of semi-elemental formulas, contribute to improved outcomes and reduced complications. Early initiation and gradual increase in enteral nutrition via fistuloclysis demonstrate safety and efficacy, underscoring the importance of tailored nutritional approaches in optimizing patient care for complex surgical conditions.

SO,MCT,OO,FO-ILE Is Associated With Better Side Effect Profile Than SO-ILE in Critically Ill Children Receiving Parenteral Nutrition.

This study aimed to evaluate the side effect profile of soybean oil lipid injectable emulsion -(SO-ILE) and soybean oil, medium-chain triglyceride, olive oil, fish oil lipid injectable emulsion (SO,MCT,OO,FO-ILE) in critically ill children requiring parenteral nutrition (PN). This is an observational study of children admitted to our pediatric intensive care unit requiring PN for ≥7 days. Patients were divided into 2 cohorts: SO,MCT,OO,FO-ILE (n = 34) and SO-ILE (n = 111). Outcomes included development of hypertriglyceridemia (HTG), intestinal failure-associated liver disease (IFALD), length of stay, and mortality. Logistic regression was performed after controlling for duration and maximum dose of lipids. The median maximum lipid dose was significantly higher in the SO,MCT,OO,FO-ILE cohort (2.7 vs 3 g/kg; p = 0.01). Prevalence of baseline HTG was similar in both cohorts. After excluding patients with baseline HTG, incidence of HTG upon PN introduction was higher in the SO-ILE cohort (51.2% vs 26.7%; p = 0.02). The SO-ILE cohort also had significantly higher triglyceride concentrations at peak and upon discontinuation of PN (p < 0.05). Direct bilirubin and C-reactive protein were significantly higher in the SO-ILE cohort after stopping PN. Five patients (3.4%) developed IFALD, 4 of whom were in the SO-ILE cohort (p = 0.85). Upon logistic regression, mortality rate and incidence of HTG remained significantly higher in the SO-ILE cohort (adjusted odds ratio, 2.3 [95% CI, 1.1-5.3]; p = 0.04; and adjusted odds ratio, 2.0 [95% CI, 1.3-5.1]; p = 0.03, respectively). In critically ill children requiring PN, SO-ILE was associated with a higher risk of HTG, -elevated direct bilirubin, inflammatory markers and mortality compared with SO,MCT,OO,FO-ILE.

The Rate of Discontinuing Ready-to-Use Multi-Chamber Bag Parenteral Nutrition Secondary to High Serum Electrolyte Levels.

Background: Multi-chamber bag with electrolytes (MCB-E) parenteral nutrition (PN) formulations are gaining acceptance for nutritional support for safety and economic advantages. However, their use is hampered by serum electrolyte abnormalities. No data exist regarding MCB-E PN interruption due to high serum electrolyte levels. We assessed the rate of MCB-E PN discontinuation in surgical patients secondary to persistently high serum electrolyte levels. Methods: This prospective, cohort study included surgical patients (aged ≥18 years) who received MCB-E PN at King Faisal Specialist Hospital and Research Centre-Riyadh, from February 28, 2020, until August 30, 2021. Patients were followed for 30-day for the discontinuation of MCB-E PN secondary to persistent hyperphosphatemia, hyperkalemia, hypermagnesemia, or hypernatremia for two consecutive days. The association of discontinuing MCB-E PN with various factors was assessed using univariable and multivariable Poisson regression analysis. Results: Seventy-two patients were enrolled in the study, 55 (76.4%) completed MCB-E PN, whereas 17 (23.6%) patients discontinued MCB-E PN due to a persistent hyperphosphatemia (n = 13, 18%) and persistent hyperkalemia (n = 4, 5.5%). Hyperphosphatemia and hyperkalemia were observed at a median of 9 (IQR 6-15) days and 9.5 (IQR 7-12) days of MCB-E PN support, respectively. According to adjusted multivariable analysis, developing an episode of hyperphosphatemia or hyperkalemia was associated with MCB-E PN discontinuation (RR 6.62, 1.95-22.49; P = .002) and (RR 4.73, 1.30-17.24; P = .018), respectively. Conclusion: In surgical patients receiving short-term MCB-E PN, developing hyperphosphatemia was the most frequent high electrolyte abnormality assocaited with MCB-E PN discontinuation, followed by hyperkalemia.

39 Early Higher Parenteral Lipid Intake Decreases Extra-Uterine Growth Restriction in Very Low Birth weight Infants: A Randomized Controlled Trial

Abstract Background Approximately 43-65% of very low birth weight (VLBW) infants develop extra-uterine growth restriction (EUGR). EUGR is associated with a significant increase in the risk of neurodevelopmental impairment. Inadequate early postnatal nutrition results in excessive weight loss that cannot be explained by the physiologic contraction of body water alone. EUGR and postnatal growth failure are usually associated with negative early energy and nitrogen balance in the first week of life. Growth trajectories after initial weight loss have similar slopes regardless of gestational age, which indicates that the early excessive weight loss is a lead cause for EUGR. Objectives To study whether an early and higher parenteral lipid intake in the first week after birth would decrease the percentage of weight loss and subsequently the incidence of EUGR. Design/Methods This was a randomized, open-label, control trial of appropriate-for-gestational age VLBW infants admitted to our level III NICU. Lipid intake in the control group started at 0.5-1 g/kg/day and was increased daily by 0.5-1 g/kg/day until 3 g/kg/day was reached. The intervention group was started on 2 g/kg/day then increased to 3 g/kg/day the following day. Triglyceride levels were measured the day after the start and after each increase in lipid intake. Results Among the 176 infants assessed for eligibility, eighty-three were included in the trial. There were no significant differences between the control and the intervention group in mean gestational age (27.3 ± 2.4 vs. 27.1 ± 2.3 weeks respectively) or birth weight (1011 ± 250 vs. 1019 ± 271 g respectively). Infants in the intervention group were started on lipid earlier (13.8±7.8 vs. 17.5±7.8 h; p=0.03) and had higher cumulative lipid intake in the first 7 days of age (13.5±4.2 vs. 10.9±3.5 g/kg; p=0.004) that led to a protein to energy ratio; closer to the recommended values. Total fluid intake was similar between the two groups. Infants in the intervention group had a lower percentage of weight loss (10.4±3.6 vs. 12.7±4.6; p=0.02). The mean triglyceride level was higher in the intervention group (1.91± 0.79 vs. 1.49±0.54 mmol/L; p= 0.01), however, hypertriglyceridemia was similar between the two groups at 2 and 3 g/kg/day of lipid intake. Enteral energy and protein intake calculated weekly between the time of parenteral nutrition discontinuation and 36 weeks corrected gestational age (CGA) were similar between the 2 groups. EUGR at 36 weeks CGA was significantly lower in the intervention group (38.6% vs. 67.6%; p=0.01). Conclusion In VLBW infants, the provision of an early and higher dose of parenteral lipid in the first week of life results in less weight loss and lower incidence of EUGR.

P1.36: Essential fatty acids profile in pediatric patients receiving an intravenous lipid emulsion containing 15% fish oil

Composite intravenous lipid emulsion (ILE) containing soybean (30%), medium chain triglycerides (MCT) (30%), olive (25%) and fish (15%) oil (SMOFlipid®) is now widely used in the world for prematures, newborns and children on parenteral nutrition (PN). Long-term use in children on home-PN (HPN) is not documented. The aim was to assess the fatty acids (FA) profile of such HPN children. Population: 26 children with chronic intestinal failure (CIF) were assessed [SBS = 10, congenital enteropathy = 9, total aganglionosis = 4, CIPOS = 3] aged 6 months-16 years on home-PN for 12 months-16 years, highly dependent on PN (low plasma citrulline levels and high PN intake). All received SMOFlipid® as source of ILE for 12 - 38 months at the dose of 2.1 ± 0.39 g/kg/day, 6.7 ± 0.7 days/week. They were compared to 26 SBS children aged 7.8±3.9 years, weaned off PN for > 2 years. Methods: Sampling performed after ≥ 24h fat free PN and 6-8h after PN discontinuation. Red blood cell (RBC) fatty acids (FA) profiles were established by using gas-chromatography. Citrulline plasma levels, ratio non protein energy PN-intake(NPE)/resting energy expenditure (REE) - Schofield equation-, growth parameters in Z-score and total bilirubin were assessed.. FA profiles and Holman ratio (triene/tetraene) were compared to those obtained from the control group. Results: NSD: No significant difference. Conclusion: Long-term administration of an ILE rich in fish oil (15%) in highly PN dependent children (PN/REE:130±20 %) is well tolerated. Bilirubin plasma levels remained low and growth was normal. The RBC-FA profile, reflects the n-3/n-6 FA acid composition of this new fish oil rich ILE, without EFA deficiency as assessed by the Holman ratio. This “new” RBC-FA profile, followed on the long term, is not accompanied with any harmful clinical effect and can be considered as safe and probably beneficial for preventing IFALD.

Effect of Early Parenteral Nutrition Discontinuation on Time to Regain Birth Weight in Very Low Birth Weight Infants: A Randomized Controlled Trial.

Peripherally inserted central catheters (PICCs) are used to administer parenteral nutrition (PN) in very low birth weight infants (VLBW; <1500 g). Clinicians try to optimize early nutrition but also minimize the risks associated with intravascular devices. The objective of this study was to examine the early nutrition impact of discontinuing PN at different enteral feed volumes in VLBW infants. In this unmasked, multicenter, randomized controlled trial, patients were randomly assigned to PICC removal and PN discontinuation at an enteral feed volume of 100mL/kg/day (intervention) or 140mL/kg/day (control). Clinically stable VLBW infants with a PICC in situ who were receiving PN were eligible for inclusion. Infants with major congenital anomalies were excluded. A total of 139 patients were enrolled; 69 and 70 patients were randomized to the intervention and control groups, respectively. The primary outcome measure was the mean difference in time (days) to regain birth weight. The groups were well matched at study entry. Patients in the intervention group regained birth weight more slowly (mean difference 1.5 days CI: 0.3-2.7 days, P = 0.01). The mean difference in time to regain birth weight for infants <1000g was 2.8 days (95% CI: 0.8-4.8 days, P = 0.008). In VLBW infants, early PICC removal at an enteral feed volume of 100mL/kg/day compared with later removal at 140mL/kg/day resulted in a significant delay in time to regain birth weight, and this delay was more pronounced in infants <1000 g.

Standardized Nutrition Protocol for Very Low-Birth-Weight Infants Resulted in Less Use of Parenteral Nutrition and Associated Complications, Better Growth, and Lower Rates of Necrotizing Enterocolitis.

We assessed the impact of a standardized nutrition initiative for very low-birth-weight (VLBW) infants on their nutrition and clinical outcomes. This was a prospective analysis of VLBW infants born before and after the initiation of a nutrition protocol. This protocol included trophic feeds, feeding advancement, fortification guidelines, parameters on the concentration of parenteral nutrition (PN), and the discontinuation of PN and central lines. Gastric residual monitoring was discontinued. Statistical analyses were performed with Fisher's exact and Student's t-tests. Primary outcome measures were days receiving PN, days made nil per os (NPO) after feeding initiation, necrotizing enterocolitis, and growth parameters. Secondary outcome measures were central-line days, sepsis, blood transfusions, cholestasis, osteopenia, chronic lung disease, and retinopathy of prematurity. 136 VLBW infants were analyzed, including 77 in the preprotocol group and 59 in the postprotocol group. Infants postprotocol were found to have reduced PN days (26.1 versus [vs] 18.4, P < .01), fewer days made NPO after feeding initiation (7.2 vs 4.0, P = .02), NEC (7.8% vs 0%, P = 0.038), central-line days (26.5 vs 18.6, P < .01), cholestasis (16% vs 3%, P=.02), and blood transfusions (5.3 vs 3.1, P = .028). Growth, defined by change in z-score from birth to discharge, improved for weight (-1.3 vs -0.8, P < .01), length (-1.5 vs -1.0, P = .033), and head (-1.1 vs -0.6, P = .024). Initiation of a standardized nutrition initiative for VLBW infants significantly improved growth, reduced PN use, and improved patient outcomes.

Long-Term Outcomes in Children With Intestinal Failure-Associated Liver Disease Treated With 6 Months of Intravenous Fish Oil Followed by Resumption of Intravenous Soybean Oil.

Intravenous soybean oil (SO) is a commonly used lipid emulsion for children with intestinal failure (IF); however, it is associated with IF-associated liver disease (IFALD). Studies have demonstrated that intravenous fish oil (FO) is an effective treatment for IFALD. However, there is a lack of long-term data on children who stop FO and resume SO. This study's objective was to investigate our institution's outcomes for children with IFALD treated with 6months of FO and who then restarted SO. Inclusion criteria for FO included children with IFALD. Parenteral nutrition (PN)-dependent children resumed SO after FO and were prospectively followed for 4.5 years or until death, transplant, or PN discontinuation. The primary outcome was the cumulative incidence rate (CIR) for cholestasis after FO. Forty-eight subjects received FO, and conjugated bilirubin decreased over time (-0.22mg/dL/week; 95% confidence interval [CI]: -0.25, -0.19; P< .001). The CIR for cholestasis resolution after 6months of FO was 71% (95% CI: 54%, 82%). Twenty-seven subjects resumed SO and were followed for a median of 16months (range 3-51months). While the CIR for enteral autonomy after 3 years of follow-up was 40% (95% CI: 17%, 26%), the CIR for cholestasis and transplant was 26% (95% CI: 8%, 47%) and 6% (95% CI: 0.3%, 25%), respectively. In this study, FO effectively treated cholestasis, and SO resumption was associated with cholestasis redevelopment in nearly one-fourth of subjects. Long-term FO may be warranted to prevent end-stage liver disease.

Long Term Outcomes after Intestinal Transplantation from a Single South-American Center, Lessons Learned

Introduction In many countries, intestinal failure patients (IF pts) had the only option of parenteral nutrition (PN) as available treatment until intestinal transplantation (ITx) evolves as part of a multidisciplinary team. We would like to report the long-term outcomes of a series of ITx performed in children and adults at a single center, 9 years after its creation. Material and Methods retrospective analysis of all consecutive ITx performed between May 2006 and Oct 2017. Diagnoses, pre ITx mean time on PN, indication for ITx, time on the waiting list (WL), type of ITx, mean total ischemia time (TIT), and warm ischemia time (WIT), time for PN discontinuation, 5-year actuarial patient survival are reported. Results 44 patients received 47 ITx. The mean time on PN for the Tx group was 1604 days*. The main indication for ITx was: lack of central venous accesses followed by PN associated liver disease, and catheter related infectious complications. The mean time on the WL was 222 days (SD: 195 days). ITx were performed in 28 children and 16 adults. Thirty-seven procedures were isolated ITx; 10 multiorgan (MTO) (4 combined, 7 multivisceral - 2 with kidney); 4 (8,5%) were re-transplants: 3 isolated, 1 multivisceral; 8 included right colon. Sixteen pts (36%) received the abdominal rectus fascia. All procedures were performed by the same team; TIT was 7:36±2:10 hs, WIT was 39.1±10.5 min. Mean length of implanted intestine: 325 ± 59.5 cm, BC ileostomy were performed in 59.5% of the cases. 18/47 Tx required early re-operations. The overall mean follow up time is 49±45.2 months; the mean time to be off PN was 68 days (* p: 0,001); the overall 5 year pt survival is 55.1%; 60.5% for Isolated ITx vs 35% for MTO (p=0,01); 60.3% for ped recipient’s vs 44.9% for adults (p=NS). Conclusions After 11 years of establishing a dedicated adult and ped IF program, we proved that ITX is a valid therapeutic alternative for pediatric and adult candidates. Long term results are comparable to a large series reported from centers of develop countries or the ITR.

The significance of organ prolapse in gastroschisis

PurposeThe aim of this study was to evaluate the incidence and importance of organ prolapse (stomach, bladder, reproductive organs) in gastroschisis. MethodsThis is a retrospective review of gastroschisis patients from 2000 to 2014 at a single tertiary institution. Statistical analysis was performed using a chi-square test, Student's t test, log-rank test, or Cox regression analysis models. All tests were conducted as two-tailed tests, and p-values <0.05 were considered statistically significant. ResultsOne hundred seventy-one gastroschisis patients were identified. Sixty-nine (40.6%) had at least one prolapsed organ besides bowel. The most commonly prolapsed organs were stomach (n=45, 26.3%), reproductive organs (n=34, 19.9%), and bladder (n=15, 8.8%). Patients with prolapsed organs were more likely to have simple gastroschisis with significant decreases in the rate of atresia and necrosis/perforation. They progressed to earlier enteral feeds, discontinuation of parenteral nutrition, and discharge. Likewise, these patients were less likely to have complications such as central line infections, sepsis, and short gut syndrome. ConclusionsGastroschisis is typically described as isolated bowel herniation, but a large portion have prolapse of other organs. Prolapsed organs are associated with simple gastroschisis, and improved outcomes most likely due to a larger fascial defect. This may be useful for prenatal and postnatal counseling of families. Type of studyCase Control/Retrospective Comparative Study. Level of evidenceLevel III.

Early versus Late Enteral Feeding in Preterm Intrauterine Growth Restricted Neonates with Antenatal Doppler Abnormalities: An Open-Label Randomized Trial.

Enteral feeding in preterm neonates with intrauterine growth restriction (IUGR) and absent or reversed end diastolic flow (AREDF) on umbilical artery (UA) Doppler is delayed owing to an increased risk of necrotizing enterocolitis (NEC). Delaying enteral feeding with longer duration of parenteral nutrition (PN) carries an increased risk of sepsis. To study early versus late feeding in preterm IUGR neonates for time required to attain sufficient feed volume to discontinue PN and increased risk of NEC or feed intolerance (FI). Open-label randomized controlled trial. Tertiary care neonatal unit and fetal-maternal medicine unit in India. Preterm intrauterine growth restricted neonates' ≤32 weeks with AREDF on UA Doppler enrolled from 1 January 2014 to 31 July 2015. Randomized to receive early or late feeding using mothers own or donor breast milk as per a feed initiation and advancement protocol. Time in days required to attain sufficient feed volume allowing discontinuation of PN and incidence of NEC in neonates fed early versus late. There were 77 eligible neonates. Sixty-two neonates were included and stratified as extreme preterm (27-29 weeks) (n = 20) and very preterm (30-32 weeks) (n = 42). Ten extreme preterm and 21 very preterm neonates were randomized to each early feeding and late feeding arm. There was a significantly faster attainment of sufficient feeds in the early feeding arm of both the stratified groups [extreme preterm: median 14 days (Interquartile range IQR: 12-15) compared with 18 days (IQR: 18-20), hazard ratio (HR): 1.59, 95% CI: 0.626-4.078; very preterm: 12 days (IQR: 10-14) as compared with 16 days (IQR 15-17), HR: 1.89, 95% CI: 1.011-3.555]. There was no difference in the incidence of NEC, FI and combined outcome of NEC and FI. Early feeding in preterm IUGR neonates with AREDF on antenatal UA Doppler allowed earlier discontinuation of PN, allowing birth weight to be regained earlier and did not increase the incidence of NEC and FI.

Mucous fistula refeeding decreases parenteral nutrition exposure in postsurgical premature neonates

Background/PurposePremature neonates can develop intraabdominal conditions requiring emergent bowel resection and enterostomy. Parenteral nutrition (PN) is often required, but results in cholestasis. Mucous fistula refeeding allows for functional restoration of continuity. We sought to determine the effect of refeeding on nutrition intake, PN dependence, and PN associated hepatotoxicity while evaluating the safety of this practice. MethodsA retrospective review of neonates who underwent bowel resection and small bowel enterostomy with or without mucous fistula over 2years was undertaken. Patients who underwent mucous fistula refeeding (RF) were compared to those who did not (OST). Primary outcomes included days from surgery to discontinuation of PN and goal enteral feeds, and total days on PN. Secondary outcomes were related to PN hepatotoxicity. ResultsThirteen RF and eleven OST were identified. There were no significant differences among markers of critical illness (p>0.20). In the interoperative period, RF patients reached goal enteral feeds earlier than OST patients (median 28 versus 43days; p=0.03) and were able to have PN discontinued earlier (median 25 versus 41days; p=0.04). Following anastomosis, the magnitude of effect was more pronounced, with RF patients reaching goal enteral feeds earlier than OST patients (median 7.5 versus 20days; p≤0.001) and having PN discontinued sooner (30.5 versus 48days; p=0.001). ConclusionsRF neonates reached goal feeds and were able to be weaned from PN sooner than OST patients. A prospective multicenter trial of refeeding is needed to define the benefits and potential side effects of refeeding in a larger patient population in varied care environments.

Outcome of acute intestinal failure.

Type 2 acute intestinal failure is characterized by the need for parenteral nutrition (PN) for several months, and is typically caused by complications of abdominal surgery with enteric fistulas or proximal stomas. This study aimed to evaluate clinical management according to quality indicators established by the Association of Surgeons of Great Britain and Ireland. Consecutive patients with type 2 intestinal failure referred to a specialized centre were analysed. Outcomes included the rate of discontinuation of PN, morbidity and mortality. Eighty-nine patients were analysed, of whom 57 had an enteric fistula, 29 a proximal stoma (6 with distal fistulas), and three had intestinal failure owing to other causes. One patient was deemed inoperable, and nine patients died from underlying illness during initial management. Before reconstructive surgery, 94 per cent (65 of 66 operated and 3 patients scheduled for surgery) spent the period of rehabilitation at home. Discontinuation of PN owing to restoration of enteral autonomy was achieved in 65 (73 per cent) of 89 patients. Seven patients developed a recurrent fistula, which was successfully managed with a further operation in four, resulting in successful fistula takedown in 41 of 44 patients undergoing fistula resection. Three patients (5 per cent) died in hospital after reconstructive surgery. The overall mortality rate in this series, including preoperative deaths from underlying diseases, was 16 per cent (14 patients). Intestinal failure care and reconstructive surgery resulted in successful discontinuation of PN in the majority of patients, although disease-related mortality was considerable.

Long-Term Outcomes of Intestinal and Multivisceral Transplantation at a Single Center in Argentina

BackgroundIntestinal failure (IF) patients received parenteral nutrition (PN) as the only available therapy until intestinal transplantation (ITx) evolved as an accepted treatment. The aim of this article is to report the long-term outcomes of a series of ITx performed in pediatric and adult patients at a single center 9 years after its creation. Patients and MethodsThis is a retrospective analysis of the ITx performed between May 2006 and January 2015. Diagnoses, pre-ITx mean time on PN, indications for ITx, time on the waiting list for types of ITx, mean total ischemia time, and warm ischemia time, time until PN discontinuation, incidence of acute and chronic rejection, and 5-year actuarial patient survival are reported. ResultsA total of 42 patients received ITx; 80% had short gut syndrome (SG); the mean time on PN was 1620 days. The main indication for ITx was lack of central venous access followed by intestinal failure–associated liver disease (IFALD) and catheter-related infectious complications. The mean time on the waiting list was 188 days (standard deviation, ±183 days). ITx were performed in 26 children and 14 adults. In all, 32 procedures were isolated ITx (IITX); 10 were multiorgan Tx (MOT; 3 combined, 7 multivisceral Tx (MVTx), 1 modified MVTx and 2 with kidney); 2 (4.7 %) were retransplantations: 1 IITx, 1 MVTx, and 5 including the right colon. Thirteen patients (31%) received abdominal rectus fascia. All procedures were performed by the same surgical team. Total ischemia time was 7:53 ± 2:04 hours, and warm ischemia time was 40.2 ± 10.5 minutes. The mean length of implanted intestine was 325 ± 63 cm. Bishop–Koop ileostomy was performed in 67% of cases. In all, 16 of 42 Tx required early reoperations. The overall mean follow-up time was 41 ± 35.6 months. The mean time to PN discontinuation after Tx was 68 days (P = .001). The total number of acute cellular rejection (ACR) episodes until the last follow-up was 83; the total number of grafts lost due to ACR was 4; and the total graft lost due to chronic rejection was 3. At the time of writing, the overall 5-year patient survival is 55% (65% for IITx vs 22% for MOT; P = .0001); 60% for pediatric recipients vs 47% for adults (P = NS); 64% when the indication for ITx was SG vs 25% for non-SG (P = .002). ConclusionsAt this center, candidates with SG, in the absence of IFALD requiring IITx, showed the best long-term outcomes, independent of recipient age. A multidisciplinary approach is mandatory for the care of intestinal failure patients, to sustain a rehabilitation and transplantation program over time.

Predictors of Enteral Autonomy in Children with Intestinal Failure: A Multicenter Cohort Study

In a large cohort of children with intestinal failure (IF), we sought to determine the cumulative incidence of achieving enteral autonomy and identify patient and institutional characteristics associated with enteral autonomy. A multicenter, retrospective cohort analysis from the Pediatric Intestinal Failure Consortium was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with dependence on parenteral nutrition (PN) >60 days. Enteral autonomy was defined as PN discontinuation >3 months. A total of 272 infants were followed for a median (IQR) of 33.5 (16.2-51.5) months. Enteral autonomy was achieved in 118 (43%); 36 (13%) remained PN dependent and 118 (43%) patients died or underwent transplantation. Multivariable analysis identified necrotizing enterocolitis (NEC; OR 2.42, 95% CI 1.33-4.47), care at an IF site without an associated intestinal transplantation program (OR 2.73, 95% CI 1.56-4.78), and an intact ileocecal valve (OR 2.80, 95% CI 1.63-4.83) as independent risk factors for enteral autonomy. A second model (n = 144) that included only patients with intraoperatively measured residual small bowel length found NEC (OR 3.44, 95% CI 1.36-8.71), care at a nonintestinal transplantation center (OR 6.56, 95% CI 2.53-16.98), and residual small bowel length (OR 1.04 cm, 95% CI 1.02-1.06 cm) to be independently associated with enteral autonomy. A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ileocecal valve, and longer bowel length are associated with achieving enteral autonomy. It is likely that variations in institutional practices and referral patterns also affect outcomes in children with IF.

Enteral Nutrition in the Management of Pediatric Intestinal Failure

Intestinal failure is an uncommon but devastating condition whose natural history has dramatically improved over the past two decades (1). Infants with intestinal failure due to severe short bowel syndrome or other diagnoses previously considered incompatible with life are now routinely being saved and cared for in cutting edge, multidisciplinary programs. Enteral nutrition plays a central role in the management of children with intestinal failure. This review provides an overview of enteral nutrition in pediatric intestinal failure, with specific emphasis on recent advances in clinical management, patient outcomes, and emerging therapies.

Necrotizing Enterocolitis (NEC) and the Risk of Intestinal Stricture: The Value of C-Reactive Protein

Necrotizing enterocolitis (NEC) is a severe complication frequently seen during the neonatal period associated with high mortality rate and severe and prolonged morbidity including Post-NEC intestinal stricture. The aim of this study is to define the incidence and risk factors of these post-NEC strictures, in order to better orient their medicosurgical care. Sixty cases of NEC were retrospectively reviewed from a single tertiary center with identical treatment protocols throughout the period under study, including systematic X-ray contrast study. This study reports a high rate of post-NEC intestinal stricture (n = 27/48; 57% of survivors), either in cases treated surgically (91%) and after the medical treatment of NEC (47%). A colonic localization of the strictures was more frequent in medically-treated patients than in those with NEC treated surgically (87% vs. 50%). The length of the strictures was significantly shorter in case of NEC treated medically. No deaths were attributable to the presence of post-NEC stricture. The mean hospitalization time in NICU and the median age at discontinuation of parenteral nutrition were longer in the group with stricture, but this difference was not significant. The median age at discharge was significantly higher in the group with stricture (p = 0.02). The occurrence of post-NEC stricture was significantly associated with the presence of parietal signs of inflammation and thrombopenia (<100 000 platelets/mm3). The mean maximum CRP concentration during acute phase was significantly higher in infants who developed stricture (p<0.001), as was the mean duration of the elevation of CRP levels (p<0.001). The negative predictive value of CRP levels continually <10 mg/dL for the appearance of stricture was 100% in our study. In conclusion, this retrospective and monocentric study demonstrates the correlation between the intensity of the inflammatory syndrome and the risk of secondary intestinal stricture, when systematic contrast study is performed following NEC.

Large Granular Lymphocytic Leukemia: A Treatable Form of Refractory Celiac Disease

Large granular lymphocyte leukemia (LGL) is characterized by clonal expansion of CD3+ T cells or CD3(-) natural killer cells and frequently is associated with autoimmune diseases. We describe 2 patients with celiac disease who no longer responded to gluten-free diets after they developed T-cell LGL, with intestinal localization of malignant lymphocytes. Flow cytometry phenotyping of isolated intestinal intraepithelial and lamina propria cells eliminated type II refractory celiac disease, identifying large-sized CD8(+)CD57(+) T cells. Treatment with a combination of cyclosporine and methotrexate restored the patients' sensitivity to gluten-free diets. LGL therefore might be a cause of refractory celiac disease that is sensitive to immunosuppressive therapy.

Parenteral Plant Sterols and Intestinal Failure–associated Liver Disease in Neonates

We prospectively evaluated incidence of prolonged (>28 days) parenteral nutrition (PN), associated complications, and significance of parenteral plant sterols (PS) in neonatal intestinal failure-associated liver disease (IFALD) compared with children. We recruited 28 neonates (mean age 50 days, range 28-126) and 11 children (6.9 y, 2.1-16.6) in all of Finland. Patients underwent repeated measurements of serum cholesterol, noncholesterol sterols, including PS, cholestanol and cholesterol precursors, and liver biochemistry during and 1 month after discontinuation of PN. Healthy matched neonates (n=10) and children (n=22) served as controls. IFALD occurred more frequently among neonates (63%) than children (27%; P<0.05). Ratios of serum PS, including stigmasterol, sitosterol, avenasterol, and campesterol, and total PS were increased among neonates compared with healthy controls and children on PN by 2- to 22- and 2- to 5-fold (P<0.005), respectively. Neonates with IFALD had significantly higher ratios of serum PS and cholestanol compared with neonates without IFALD (P<0.05). Total duration of PN associated with serum cholestanol, stigmasterol, avenasterol, alanine aminotransferase, and aspartate aminotransferase (r=0.472-0.636, P<0.05). Cholestanol and individual serum PS, excluding campesterol, reflected direct bilirubin (r=0.529-0.688, P<0.05). IFALD persisted after discontinuation of PN in 25% of neonates with 4.2- and 2.2-times higher ratios of serum stigmasterol and cholestanol compared with neonates without IFALD (P<0.05). Frequent occurrence of IFALD among neonates on PN displays an association to duration of PN and markedly increased serum PS, especially stigmasterol, in comparison to healthy neonates and children on PN. Striking accumulation of parenteral PS may contribute to IFALD among neonates.

Natural History of Pediatric Intestinal Failure: Initial Report from the Pediatric Intestinal Failure Consortium

To characterize the natural history of intestinal failure (IF) among 14 pediatric centers during the intestinal transplantation era. The Pediatric Intestinal Failure Consortium performed a retrospective analysis of clinical and outcome data for a multicenter cohort of infants with IF. Entry criteria included infants <12 months receiving parenteral nutrition (PN) for >60 continuous days. Enteral autonomy was defined as discontinuation of PN for >3 consecutive months. Values are presented as median (25th, 75th percentiles) or as number (%). 272 infants with a gestational age of 34 weeks (30, 36) and birth weight of 2.1 kg (1.2, 2.7) were followed for 25.7 months (11.2, 40.9). Residual small bowel length in 144 patients was 41 cm (25.0, 65.5). Diagnoses were necrotizing enterocolitis (71, 26%), gastroschisis (44, 16%), atresia (27, 10%), volvulus (24, 9%), combinations of these diagnoses (46, 17%), aganglionosis (11, 4%), and other single or multiple diagnoses (48, 18%). Prescribed medications included oral antibiotics (207, 76%), H2 blockers (187, 69%), and proton pump inhibitors (156, 57%). Enteral feeding approaches varied among centers; 19% of the cohort received human milk. The cohort experienced 8.9 new catheter-related blood stream infections per 1000 catheter days. The cumulative incidences for enteral autonomy, death, and intestinal transplantation were 47%, 27%, and 26%, respectively. Enteral autonomy continued into the fifth year after study entry. Children with IF endure significant mortality and morbidity. Enteral autonomy may require years to achieve. Improved medical, nutritional, and surgical management may reduce time on PN, mortality, and need for transplantation.