Long-term teduglutide associated with improved response in pediatric short bowel syndrome-associated intestinal failure.

OBJECTIVES: Patients with short bowel syndrome-associated intestinal failure (SBS-IF) require long-term parenteral nutrition and/or intravenous fluids (PN/IV) to maintain fluid or nutritional balance. We report the long-term safety, efficacy, and predictors of response in pediatric patients with SBS-IF receiving teduglutide over 96 weeks. METHODS: This was a pooled, post hoc analysis of two open-label, long-term extension (LTE) studies (NCT02949362 and NCT02954458) in children with SBS-IF. Endpoints included treatment-emergent adverse events (TEAEs) and clinical response (≥20% reduction in PN/IV volume from baseline). A multivariable linear regression identified predictors of teduglutide response; the dependent variable was mean change in PN/IV volume at each visit over 96 weeks. RESULTS: Overall, 85 patients were analyzed; 78 patients received teduglutide in the parent and/or LTE studies (any teduglutide [TED] group), while seven patients did not receive teduglutide in either the parent or LTE studies. Most TEAEs were moderate or severe in intensity in both groups. By week 96, 82.1% of patients from the any TED group achieved a clinical response, with a mean fluid decrease of 30.1 mL/kg/day and an energy decrease of 21.6 kcal/kg/day. Colon-in-continuity, non-White race, older age at baseline, longer duration of teduglutide exposure, and increasing length of remaining small intestine were significantly associated with a reduction in mean PN/IV volume requirements. CONCLUSIONS: In pediatric patients with SBS-IF, teduglutide treatment resulted in long-term reductions in PN/IV requirements. The degree of PN/IV volume reduction depended on the duration of teduglutide exposure, underlying bowel anatomy, and demographics.

Teduglutide improves liver chemistries in short bowel syndrome-associated intestinal failure: Post hoc analysis.

BACKGROUND: Chronic hepatic complications are common in patients with short bowel syndrome-associated intestinal failure (SBS-IF). Teduglutide, a glucagon-like peptide-2 analogue, demonstrated efficacy in reducing parenteral nutrition and/or intravenous fluid dependence among patients with SBS-IF in phase 3 clinical studies. METHODS: This was a post hoc analysis of pooled data from two separate randomized, double-blind, placebo-controlled, multinational phase 3 clinical studies. Adult patients with SBS-IF with parenteral nutrition and/or intravenous fluid dependence without liver disease at baseline were randomized to treatment with the glucagon-like peptide-2 analogue teduglutide (0.05 or 0.10 mg/kg/day) or placebo subcutaneously once daily for 24 weeks. Mixed-effects models assessed the baseline predictors of change in liver chemistries. RESULTS: Between baseline and week 24, teduglutide treatment (n = 109) was associated with least squares mean reductions in aspartate aminotransferase (-7.51 IU/L; P = 0.014), alanine aminotransferase (-12.15 IU/L; P = 0.002), and bilirubin (-5.03 µmol/L [-0.057 mg/dl]; P < 0.001) compared with that of the placebo (n = 59). These values were independent of reductions in parenteral nutrition and/or intravenous fluid dependence. CONCLUSION: Teduglutide treatment was associated with reductions in liver chemistries by week 24, which is beneficial for patients with SBS-IF beyond improvements in parenteral nutrition and/or intravenous fluid dependence. Future studies should examine how long-term teduglutide might mitigate the risk of liver disease in patients with SBS-IF.

Effects of Teduglutide on Diarrhea in Pediatric Patients with Short Bowel Syndrome-Associated Intestinal Failure.

OBJECTIVES: This post-hoc analysis evaluated the effect of teduglutide treatment on diarrhea in patients with short bowel syndrome-associated intestinal failure (SBS-IF). METHODS: Data from 2 open-label, multicenter, phase 3 pediatric SBS-IF clinical trials of teduglutide (NCT01952080 and NCT02682381) were pooled where possible. The primary objective was to evaluate the change in stool consistency, frequency, and volume from baseline to weeks 12 and 24 of treatment in patients who received any teduglutide dose from both studies ("total teduglutide"). Safety assessments included gastrointestinal adverse event reporting. RESULTS: Overall, 101 patients were analyzed. Among the total teduglutide group (n = 87), there were significant changes from baseline to weeks 12 and 24 in mean (standard error) Bristol Stool Form Scale (BSFS) score [-1.8 (0.26; P < 0.0001) and -2.2 (0.27; P < 0.0001), respectively], parenteral nutrition and/or intravenous fluid (PN/IV) volume [-16.9 (1.7; P < 0.0001) and -20.1 (2.3; P < 0.0001) mL/kg/day, respectively], and enteral nutrition volume [9.2 (1.7; P < 0.0001) and 9.6 (2.3; P = 0.0002) mL/kg/day, respectively]. Among patients in the standard of care group (n = 14) there were numerical changes in BSFS score, and enteral nutrition volume at weeks 12 and 24; significant changes in PN/IV volume [-6.9 (1.5) mL/kg/day; P = 0.0041] were observed at 24 weeks, but not at 12 weeks. CONCLUSION: In this post-hoc analysis, short-term treatment with teduglutide was associated with improved stool consistency, as well as trends towards reductions in PN/IV requirements and advancements in enteral nutrition volume in children with SBS-IF. Further research assessing the impact of patient-level factors on stool characteristics when using teduglutide is warranted.

Management of short-bowel syndrome: A survey of unmet educational needs among healthcare providers.

BACKGROUND: Management of short-bowel syndrome with intestinal failure (SBS-IF) is complex and requires a multidisciplinary approach. Because of the rarity of SBS-IF, healthcare professionals (HCPs) often lack clinical experience with the disease and may benefit from education regarding SBS-IF and its management. This study identified unmet educational needs related to the management of patients with SBS-IF. METHODS: This was a prospective, web-based survey (December 2019-January 2020) in which a series of clinical questions were posed to US HCPs after presenting three standardized SBS-IF cases to assess current practice patterns. HCPs were then asked a series of questions to identify potential knowledge gaps and unmet educational needs relating to SBS-IF management. RESULTS: Overall, 558 HCPs completed the survey, with 12%-38% having a formal SBS-IF multidisciplinary team currently available to make treatment decisions within their institution. Clinicians involved in care included gastroenterologists (93%), registered dietitians (79%), gastroenterology nurse practitioners and physician assistants (37%), registered nurses (43%), social workers (45%), and psychologists/psychiatrists (27%). There was underuse of published guidelines and limited understanding of the course of intestinal adaptation. Responses to the clinical scenarios highlighted disparities in SBS-IF care delivery, including diagnosis, management goals, medications prescribed, and nutrition practices. CONCLUSIONS: Future SBS-IF educational interventions for HCPs should aim to improve awareness and understanding of the disease, facilitate timely diagnosis, and standardize management practices to ensure patients receive optimal interdisciplinary care as widely as possible.

Pediatric Gastrointestinal Histopathology in Patients With Tetratricopeptide Repeat Domain 7A (TTC7A) Germline Mutations: A Rare Condition Leading to Multiple Intestinal Atresias, Severe Combined Immunodeficiency, and Congenital Enteropathy.

Mutations in the tetratricopeptide repeat domain 7A (TTC7A) gene are a rare cause of congenital enteropathy that can result in significant morbidity. TTC7A deficiency leads to disruption of the intestinal epithelium. The histopathology of this condition has been partly described in case reports and clinical studies. This manuscript describes an in-depth investigation of the pediatric gastrointestinal pathology of the largest histologically examined cohort with confirmed TTC7A mutations reported to date and, for the first time, compared the findings to age-matched and sex-matched control patients with intestinal atresia not thought to be associated with TTC7A mutations. Hematoxylin and eosin-stained slides of endoscopically obtained mucosal biopsies and surgical resection specimens from 7 patients with known TTC7A mutations were examined retrospectively. The microscopic findings were found to be on a spectrum from atresia-predominant to those with predominantly epithelial abnormalities. Several unique histopathologic characteristics were observed when compared with controls. These included neutrophilic colitis and prominent lamina propria eosinophilia throughout the gastrointestinal tract. Striking architectural abnormalities of the epithelium were observed in 4 of the 7 patients. The 5 patients with intestinal atresia demonstrated hypertrophy and disorganization of the colonic muscularis mucosae accompanied by bland spindle cell nodules within the intestinal wall. The components of the latter were further elucidated using immunohistochemistry, and we subsequently hypothesize that they represent obliterated mucosa with remnants of the muscularis mucosae. Finally, atrophic gastritis was noted in 4 patients. In conclusion, the unique histopathologic characteristics of TTC7A mutation-associated enteropathy described herein more fully describe this novel disease entity in infants who present with congenital enteropathy or enterocolitis.

Learning Gaps and Family Experience, Nurse-Facilitated Home Parenteral Nutrition Simulation-Based Discharge Training: Proof-of-Concept Study.

BACKGROUND: Home parenteral nutrition (HPN) is a life-sustaining therapy for children and adults suffering with severe digestive diseases, yet complications are commonplace, and predischarge trainings are variable. High-fidelity simulation training provides participants with an immersive experience using realistic equipment, supplies, and scenarios. Simulation training is rapidly becoming a potential gold standard for healthcare but is currently underutilized for families and caregivers. METHODS: We prospectively collected data on pediatric patients managed at a single HPN program from September 1, 2016, to September 30, 2018. Participants in a pilot simulation-based training program (orientation, high-fidelity mannequin, realistic homelike space, standardized clinical scenarios, and structured debriefing) were compared with historical controls. We excluded patients with short-term HPN use and strictly palliative goals of care. RESULTS: Nineteen (90%) families participated in the pilot initiative with a median (interquartile range) age of 0.9 (3.7) years and diagnosis of short-bowel syndrome in 14 (74%). During teaching scenarios, learning gaps were identified for aseptic needleless changes (53%), HPN equipment setup (84%) with specific difficulty adding multivitamin (32%), and dressing changes (63%). Thirty-day readmission rates in simulation-based training group vs historical cases were 42% vs 63% (P = not significant). There was no difference in length of stay between groups. All (100%) simulation-based training group participants would recommend this learning experience to others. CONCLUSION: HPN discharge training is a novel use for high-fidelity simulation to address family/caregiver satisfaction and to identify learning gaps. Further studies are needed to refine predischarge training materials and examine the impact on postdischarge outcomes.

One-year Experience With Composite Intravenous Lipid Emulsion in Children on Home Parenteral Nutrition.

BACKGROUND: Composite lipid emulsion (CLE) composed of soybean oil, medium-chain triglycerides, olive oil, and fish oil is approved in the US for parenterally fed adults. For stable children discharged on home parenteral nutrition (HPN) without cholestasis (direct bilirubin > 2.0 mg/dL), CLE has theoretical benefits over soybean-based intravenous lipid emulsion due to reduced phytosterol exposure with higher calorie support to permit reduced glucose infusion rates (GIRs), omega-3 supplementation, and supplemental α-tocopherol. METHODS: In this prospective, single-center open-label research study, safety and efficacy outcomes were evaluated in patients on HPN younger than 18 years treated with CLE at 1 to 3 g ·â€Škg-1 ·â€Šday-1 over 12 months. The primary outcome was change in anthropometrics and GIRs compared with baseline. Secondary outcomes were changes in fatty acid profiles and liver function and enzyme tests compared with baseline. RESULTS: Fifty-seven subjects were treated with a median age of 7 years. The diagnosis was short bowel syndrome in 72%. Change in practice was associated with a decrease in mean GIRs from 17 to 14 mg ·â€Škg-1 ·â€Šh-1 at 4 to 6 months postbaseline and beyond with a coincidental decline in mean arachidonic acid and stable growth parameters. No significant adverse events were noted. CONCLUSIONS: CLE was safe and well-tolerated in stable children on HPN at 1 year, but further studies are needed in this population to appreciate long-term outcomes.

Growth in Infants and Children With Intestinal Failure-associated Liver Disease Treated With Intravenous Fish Oil.

BACKGROUND: Infants with intestinal failure (IF) and IF-associated liver disease (IFALD) are at risk for poor somatic growth because of increased metabolic demands, inadequate intake, intestinal malabsorption, chronic liver disease and other comorbidities. There are limited data on the nutritional adequacy of intravenous fish oil lipid emulsion (FOLE) compared with standard soybean oil lipid emulsion (SOLE) in the setting of intestinal failure. AIMS: To describe growth patterns in a large cohort of infants with IFALD treated with FOLE. METHODS: We compared growth data from infants enrolled in a single-center, prospective FOLE study to published norms, as well as to a multicenter, historical cohort of infants with IF treated with SOLE. RESULTS: One hundred thirty-eight infants with IFALD were treated with FOLE and 108 with SOLE. Compared with normative growth curves from WHO and published preterm data, infants in both groups from 6 to 11 months postmenstrual age exhibited declines in mean weight- and length-for-age z scores. At 24 months postmenstrual age compared with WHO growth data, infants treated with FOLE had a mean (95% confidence interval [CI]) weight-for-age z-score of 0.13 (-0.18 to 0.45) and length-for-age z-score of 0.07 (-0.33 to 0.47). In comparison, at 24 months postmenstrual age, infants treated with SOLE had a mean weight for age z-score of -0.93 (-1.20 to -0.67) and mean length for age z-score of -2.33 (-2.75 to -1.91). Independent predictors of higher weight, length and head circumference z-scores included older postmenstrual age at baseline, fewer central line-associated blood stream infections, resolution of cholestasis, type of intravenous fat emulsion (FOLE vs SOLE) and female sex. CONCLUSIONS: Infants with IFALD treated with FOLE showed comparable somatic growth to those treated with SOLE in early infancy, and improved somatic growth up to 24 months of age, supporting its wider use in this patient population.

Antibiotic Susceptibility and Therapy in Central Line Infections in Pediatric Home Parenteral Nutrition Patients.

BACKGROUND: Patients receiving home parenteral nutrition (HPN) are at high-risk for central line-associated bloodstream infections (CLABSI). There are no published management guidelines, however, for the antibiotic treatment of suspected CLABSI in this population. Historical microbiology data may help inform empiric antimicrobial regimens in this population. OBJECTIVE: The aim of the study was to describe antimicrobial resistance patterns and determine the most appropriate empiric antibiotic therapy in HPN-dependent children experiencing a community-acquired CLABSI. METHODS: Single-center retrospective cohort study evaluating potential coverage of empiric antibiotic regimens in children on HPN who developed a community-acquired CLABSI. RESULTS: From October 1, 2011 to September 30, 2017, there were 309 CLABSI episodes among 90 HPN-dependent children with median age 3.8 years old.Fifty-nine percent of patients carried the diagnosis of surgical short bowel syndrome. Organisms isolated during these infections included 60% Gram-positive bacteria, 34% Gram-negative bacteria, and 6% fungi. Among all staphylococcal isolates, 51% were methicillin sensitive. Among enteric Gram-negative organisms, sensitivities were piperacillin-tazobactam 71%, cefepime 97%, and meropenem 99%. Organisms were sensitive to current institutional standard therapy with vancomycin and piperacillin-tazobactam in 69% of cases compared with vancomycin and cefepime or vancomycin an meropenem in 85% and 96% of cases (both P < 0.01). CONCLUSIONS: Empiric antimicrobial therapy for suspected CLABSI in HPN-dependent children should include therapy for methicillin-resistant staphylococci as well as enteric Gram-negative organisms. Future studies are needed to evaluate clinical outcomes based upon evidence-based antimicrobial regimens.

Caregiver Training for Pediatric Home Parenteral Nutrition: A 5-Session Discharge Curriculum.

To maximize safety and the patient experience, caregivers require intensive training to administer home parenteral nutrition (HPN) before initial hospital discharge. This article provides the rationale, best practices, and a template for caregiver predischarge HPN education provided by nurses. The standardized HPN discharge curriculum is outlined over 5 didactic and hands-on sessions.

Essential Fatty Acid Status in Surgical Infants Receiving Parenteral Nutrition With a Composite Lipid Emulsion: A Case Series.

Infants requiring prolonged parenteral nutrition (PN) may receive intravenous (IV) lipid in the form of soybean oil, fish oil, or a composite lipid emulsion (CLE) (i.e., SMOFlipid®). Soybean oil lipid-dose restriction is a popular method of treating and reducing the risk of intestinal failure-associated liver disease (IFALD) that may influence dosing strategies of other IV fat emulsions. Here we present 4 infants receiving PN with SMOFlipid® as their IV lipid source and examine trends in essential fatty-acid status, triglycerides, and dosing strategy. The infants on restricted doses of CLE developed biochemical essential fatty-acid deficiency (EFAD) that resolved with a dosage increase or by transition to a pure fish-oil lipid emulsion. Three of the 4 infants originally prescribed CLE were diagnosed with IFALD and started a pure fish-oil lipid emulsion after treatable causes of cholestasis were excluded. One of the 4 infants presented with hypertriglyceridemia that resolved upon transition to pure fish-oil lipid emulsion. Misapplication of lipid restriction protocols to CLE regimens render infants at risk for EFAD. CLE should be dosed within recommended ranges to prevent EFAD. Restricted protocols warrant close monitoring of essential fatty-acid status in infants receiving prolonged PN, particularly in those with minimal or no enteral intake. Hypertriglyceridemia and cholestasis are known adverse effects of CLE and require monitoring.

Virtual Telemedicine Visits in Pediatric Home Parenteral Nutrition Patients: A Quality Improvement Initiative.

BACKGROUND: Despite being less costly than prolonged hospitalization, home parenteral nutrition (HPN) is associated with high rates of post-discharge complications, including frequent readmissions and central line-associated bloodstream infections (CLABSIs). Telemedicine has been associated with improved outcomes and reduced healthcare utilization in other high-risk populations, but no studies to date have supported effectiveness of telemedicine in pediatric HPN. METHODS: We prospectively collected data on pediatric patients managed at a single HPN program who participated in postdischarge telemedicine visits from March 1, 2014 to March 30, 2016. We excluded patients with a history of HPN and strictly palliative care goals. Univariate analysis was performed for primary outcomes: Community-acquired CLABSI and 30-day readmission rate. RESULTS: Twenty-six families participated in the pilot initiative with median (interquartile range) patient age 1.5 (5.7) years old, diagnosis of short bowel syndrome in 16 (62%), and in-state residence in 17 (55%). Ishikawa (fishbone) diagram identified causes of post-discharge HPN complications. Areas of focus during telemedicine visit included central venous catheter care methods, materials, clinical concerns, and equipment. Compared to historical comparison group, the telemedicine group experienced CLABSI rates of 1.0 versus 2.7 per 1,000 line days and readmission rates of 38% versus 17% (p = 0.03, 0.02, respectively). CONCLUSIONS: Telemedicine visits identified opportunities for improvement for families newly discharged on HPN. In a small cohort of patients who experienced telemedicine visits, we found lower CLABSI rates alongside higher readmission rates compared with a historical comparison group. Further studies are needed to optimize telemedicine in delivering care to this high-risk population.

Analysis of Healthcare Institutional Costs of Pediatric Home Parenteral Nutrition Central Line Infections.

BACKGROUND: Although previous literature suggests home parenteral nutrition (HPN)-dependent children experience frequent complications like community-acquired central line-associated bloodstream infections (CLABSI), few studies have characterized the cost. OBJECTIVE: The aim of this study was to evaluate institutional cost of community-acquired CLABSI in pediatric patients with HPN. METHODS: This is a single-center retrospective review of institutional costs for patients with HPN with community-acquired CLABSI at a tertiary care children's hospital. Inclusion was age 18 years or less between October 2011 and April 2016. Exclusions were death during hospitalization and readmission within 2 days of discharge. Patient-level factors were compared between high-cost group and all others using Welch 2-sample t test and analysis of variance. Multivariable logistic regression was used to determine predictors of higher cost. RESULTS: There were 176 CLABSI admissions among 68 patients during the study period (median 2 hospitalizations per patients). The mean cost and length of stay per hospital admission are $28,375 (2015 US dollars) and 8 days, and both were associated with intensive care unit admission (ICU), central venous catheter removal, private insurance, and age <2 years at admission. Nine percent of patients were classified as "super-utilizers" whose 54 hospitalizations accounted for 28% of total institutional costs. CONCLUSIONS: Among pediatric patients with HPN, community-acquired CLABSI is associated with significant cost and length of stay. Healthcare utilization is disproportionately concentrated in a small number of patients. These study findings may help inform cost analysis for future CLABSI prevention strategies.

Innovative Discharge Process for Families with Pediatric Short Bowel Syndrome: A Prospective Nonrandomized Trial.

BACKGROUND: Home parenteral nutrition (HPN) is a life-sustaining therapy for short bowel syndrome (SBS) and other severe digestive diseases, but complications are common. We evaluated a predischarge HPN hands-on training course to reduce complications in children with SBS, including hospital readmissions. METHODS: We conducted a prospective, nonrandomized controlled research study between April 1, 2014, and April 30, 2017. Eligible participants were children aged <18 years old with SBS and anticipated HPN dependence duration ≥6 months. Excluded participants had a previous history of discharge with a central venous catheter (CVC), HPN, or intravenous fluids or strictly palliative goals of care. An intervention group practiced hands-on HPN within the hospital room for 24 hours using infusion equipment. The groups received standard teaching (CVC care, home infusion pump operation, HPN preparation and administration). RESULTS: Nine children were assigned to the intervention group and 12 served as controls. The median age was 8.4 months, and length of stay (LOS) was 82 days. All participants experienced ≥1 event, with a total of 47 issues related to HPN. There were no significant associations between group assignment and 30-day postdischarge events. Each additional week of LOS was associated with 11% increase in the odds of an emergency department visit (OR 1.11; 95% CI, 1.01-1.26) and 16% increase in the odds of readmission (OR 1.16; 95% CI, 1.04-1.37). CONCLUSIONS: Postdischarge events remained widespread despite HPN bedside interventions offered by this pilot intervention. With refinement of HPN discharge processes, quality benchmarks are needed.

Intestinal Rehabilitation Programs in the Management of Pediatric Intestinal Failure and Short Bowel Syndrome.

Intestinal failure is a rare, debilitating condition that presents both acute and chronic medical management challenges. The condition is incompatible with life in the absence of the safe application of specialized and individualized medical therapy that includes surgery, medical equipment, nutritional products, and standard nursing care. Intestinal rehabilitation programs are best suited to provide such complex care with the goal of achieving enteral autonomy and oral feeding with or without intestinal transplantation. These programs almost all include pediatric surgeons, pediatric gastroenterologists, specialized nurses, and dietitians; many also include a variety of other medical and allied medical specialists. Intestinal rehabilitation programs provide integrated interdisciplinary care, more discussion of patient management by involved specialists, continuity of care through various treatment interventions, close follow-up of outpatients, improved patient and family education, earlier treatment of complications, and learning from the accumulated patient databases. Quality assurance and research collaboration among centers are also goals of many of these programs. The combined and coordinated talents and skills of multiple types of health care practitioners have the potential to ameliorate the impact of intestinal failure and improve health outcomes and quality of life.

Home Parenteral Nutrition and Intravenous Fluid Errors Discovered Through Novel Clinical Practice of Reconciling Compounding Records: A Case Series.

Home parenteral nutrition (HPN) and home intravenous (IV) hydration are complex, high-risk life-sustaining therapies for children and adults with severe digestive disorders. HPN compounding errors have the potential to cause serious patient harm. Here we present a retrospective case series at an interdisciplinary pediatric HPN program that includes specialized pharmacists reconciling prescriptions against commercial compounding records. Seven HPN or IV hydration patients were affected by significant errors in anion balance, copper, potassium, sodium, and infusion volume. Outpatient pharmacists' or other clinicians' review of compounding records as part of routine quality assurance process is critical for identifying and preventing errors.

Discrepancies Between Prescribed and Actual Pediatric Home Parenteral Nutrition Solutions.

BACKGROUND: Home parenteral nutrition (HPN) is increasingly prescribed for pediatric patients with complex medical conditions. Commercial vendors are widely available to compound HPN. The aim of this study was to determine the frequency of discrepancies between written HPN prescriptions and commercially compounded solutions, as well as to record the associated severity of harm from discrepancies. METHODS: From January to April 2013, 2 clinical pharmacists independently and prospectively reconciled HPN compounding records with electronic prescriptions (gold standard) during all routine ambulatory encounters to a multidisciplinary HPN program. Types, severity, and causes of discrepancies were recorded. RESULTS: Sixty-one unique patients were identified for inclusion during 117 visits. HPN solutions were compounded at 13 unique vendors across 14 states. Of all 100 compounding records, 46 (46%) contained at least 1 discrepancy, with a total of 60 discrepancies identified, affecting 34 of 61 (56%) patients. There was at least 1 discrepancy in solutions originating from 10 of 13 (77%) home infusion companies. Discrepancies were classified as Medication Error Reporting and Prevention levels C (n = 37) and D (n = 23; ie, all reaching patient but not causing harm). CONCLUSIONS: We found an alarmingly high rate of preparation discrepancies in a cohort of pediatric patients receiving HPN. Routine reconciliation of HPN compounds with intended prescriptions may be critical for ambulatory patients receiving this high-risk therapy. While home infusion commercial vendors provide an indispensable function, discrepancies and errors with potential for harm may be more common than previously appreciated.

Necrotizing enterocolitis is associated with earlier achievement of enteral autonomy in children with short bowel syndrome.

PURPOSE: Necrotizing enterocolitis (NEC) remains one of the most common underlying diagnoses of short bowel syndrome (SBS) in children. The relationship between the etiology of SBS and ultimate enteral autonomy has not been well studied. This investigation sought to evaluate the rate of achievement of enteral autonomy in SBS patients with and without NEC. METHODS: Following IRB approval, 109 patients (2002-2014) at a multidisciplinary intestinal rehabilitation program were reviewed. The primary outcome evaluated was achievement of enteral autonomy (i.e. fully weaning from parenteral nutrition). Patient demographics, primary diagnosis, residual small bowel length, percent expected small bowel length, median serum citrulline level, number of abdominal operations, status of the ileocecal valve (ICV), presence of ileostomy, liver function tests, and treatment for bacterial overgrowth were recorded for each patient. RESULTS: Median age at PN onset was 0 weeks [IQR 0-0]. Median residual small bowel length was 33.5 cm [IQR 20-70]. NEC was present in 37 of 109 (33.9%) of patients. 45 patients (41%) achieved enteral autonomy after a median PN duration of 15.3 [IQR 7.2-38.4]months. Overall, 64.9% of patients with NEC achieved enteral autonomy compared to 29.2% of patients with a different primary diagnosis (p=0.001, Fig. 1). Patients with NEC remained more likely than those without NEC to achieve enteral autonomy after two (45.5% vs. 12.0%) and four (35.7% vs. 6.3%) years on PN (Fig. 1). Logistic regression analysis demonstrated the following parameters as independent predictors of enteral autonomy: diagnosis of NEC (p<0.002), median serum citrulline level (p<0.02), absence of a jejunostomy or ileostomy (p=0.013), and percent expected small bowel length (p=0.005). CONCLUSIONS: Children with SBS because of NEC have a significantly higher likelihood of fully weaning from parenteral nutrition compared to children with other causes of SBS. Additionally, patients with NEC may attain enteral autonomy even after long durations of parenteral support.