ASPEN definitions in pediatric intestinal failure.

Pediatric intestinal failure (PIF) is a relatively rare disease entity that requires focused interdisciplinary care and specialized nutrition management. There has long been a lack of consensus in the definition of key terms related to PIF because of its rarity and a plethora of small studies rather than large trials. As such, the American Society for Parenteral and Enteral Nutrition (ASPEN) PIF Section, composed of clinicians from a variety of disciplines caring for children with intestinal failure, is uniquely poised to provide insight into this definition void. This document is the product of an effort by the Section to create evidence-based consensus definitions, with the goal of allowing for appropriate comparisons between clinical studies and measurement of long-term patient outcomes. This paper has been approved by the ASPEN Board of Directors.

Effectiveness of a central line associated blood stream infection protocol in a pediatric population.

BACKGROUND: Central line associated bloodstream infections are a common cause of bacteremia and sepsis in pediatric patients with intestinal failure, secondary to long-term CVC use. METHODS: An IRB approved retrospective chart review was conducted on TPN-dependent patients with IF who had an identified CLABSI and presented to Children's of Alabama's emergency department (ED) and were admitted to the hospital. RESULTS: Forty-four patients were included in the study, 28 in the first 18-month period and 26 in the second, with 10 in both populations. After implementation, mean time from ED presentation to antibiotic ordered and ED admission to antibiotic administered were lower. Mean time between antibiotic administration and admission to the floor was greater, and number of infectious disease consultations was greater. Floor-ICU transfers were lower, readmissions within 30 days was similar, and mean length of stay was similar. CONCLUSION: A collaborative, multidiscipline-supported protocol for the care of patients with IF presenting to the ED enhances efficiency of antibiotic ordering/administration, as well as reduces the number of unplanned floor-ICU transfers.

Effect of the COVID-19 Pandemic on Pediatric Intestinal Failure Healthcare Delivery.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic has influenced how healthcare is being provided, particularly in patients whose diagnoses require multidisciplinary care, such as pediatric intestinal failure (IF). We sought to ascertain the effects of the COVID-19 pandemic on healthcare delivery for pediatric patients with IF. METHODS: A 20-question survey was administered to members of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Intestinal Rehabilitation (IR) Special Interest Group. Input values were "yes" and "no," along with a free-text response. Following a 10-day open survey period, data were divided into cohorts based on patient population size and disease burden by state. Analysis was then performed using the χ2 test application. RESULTS: Responses from 29 centers were included in analysis. Centers that followed >50 patients on parenteral nutrition (PN) were more likely to have social workers present in telemedicine visits and observed more central line difficulties among families. Centers located in states with <40,000 reported cases of COVID-19 saw patients less frequently and were more likely to withhold changes to PN prescriptions. Additionally, the survey revealed a significant degree of financial hardship and food insecurity among families. CONCLUSION: Many aspects of pediatric IF healthcare delivery have been impacted by the COVID-19 pandemic, both for care providers and caregivers. Despite the availability of telemedicine, IR centers should remain attentive to the global needs of the pediatric IF patient, as well as their families.

Key Determinants for Achieving Enteral Autonomy and Reduced Parenteral Nutrition Exposure in Pediatric Intestinal Failure.

BACKGROUND: We sought to evaluate the relationship between determinants of intestinal failure (IF) and achieving enteral autonomy from parenteral nutrition (PN) in a large single-center cohort of children. METHODS: This is a retrospective chart review of pediatric subjects enrolled in a database for the Center for Advanced Intestinal Rehabilitation at Children's of Alabama from 1989 to 2016. IF was defined as dependence on PN for >60 days. Subjects were included if they were followed since birth or infancy for a minimum of 3 months and sufficient documentation of study variables were available. Gestational age, race, diagnosis, anatomy (percent small and large bowel remaining, presence of ileocecal valve [ICV]), county of residence (rural/urban), and days of PN use were recorded. Kaplan-Meier curves and parametric survival regression models were used to investigate the relationship between the demographic and clinical variables with the length of PN use. RESULTS: Initially, 290 subjects were available to review. After inclusion/exclusion were applied, 158 subjects remained. Gestational age, diagnosis (necrotizing enterocolitis), small-bowel length (>50%), and presence of an ICV were all positive predictors for reaching enteral autonomy. Residual colon length was associated with shorter duration of PN in days. CONCLUSION: Enteral autonomy is a key outcome among children with IF. In our cohort, we found that gestational age, diagnosis, remaining small bowel, and presence of ICV are positive predictors for reaching this important milestone. Colon length is also an important factor with respect to duration of PN in days.

Development of venous thrombi in a pediatric population of intestinal failure.

BACKGROUND/PURPOSE: Although pediatric intestinal failure (IF) is now a survivable diagnosis, children are still at risk for complications. Loss of venous access persists as a leading indication for intestinal transplantation. The goal of this study was to identify risk factors for loss of venous access in a pediatric intestinal failure population on long-term PN. METHODS: We identified all patients who were PN dependent. RESULTS: Patients that developed venous thrombosis had significantly more lines placed in the first 2 years of life compared to those who did not develop thrombosis. Multivariate regression analysis revealed that diagnosis (NEC and gastroschisis) and parental education were significant predictors of venous thrombosis. CONCLUSION: By identifying potential risk factors for thrombus development, interventions can be developed to improve the overall outcome in pediatric IF patients. TYPE OF STUDY: Diagnostic LEVEL OF EVIDENCE: III.

Optimization of Laboratory Ordering Practices for Complete Blood Count With Differential.

Objectives: To investigate the utilization of CBC and CBC with differential (CBC w/diff) tests at University of Alabama at Birmingham Hospital, and to determine if a reduction in CBC w/diff tests could be achieved without negatively impacting patient care. Methods: The quantity of testing and distribution of repeated tests before, during, and after an educational intervention were compared. Results: CBC w/diff tests were ordered 10-fold more frequently than CBC tests. The trauma burn intensive care unit ordered the most CBC w/diff tests, with repeat tests done every 4 or 12 hours. The educational intervention reduced the number of CBC w/diff tests ordered and tests repeated every 12 hours. Conclusions: The educational intervention changed the ordering practices of CBC w/diff and CBC tests. This was sustained after the intervention and no negative effects on patient care were noted. Similar interventions may lead to optimization of ordering practices of other laboratory tests.

Increased Anti-Flagellin and Anti-Lipopolysaccharide Immunoglobulins in Pediatric Intestinal Failure: Associations With Fever and Central Line-Associated Bloodstream Infections.

BACKGROUND: Central line-associated bloodstream infections (CLABSIs) pose a significant challenge in the lives of patients with intestinal failure (IF). We hypothesized that plasma immunoglobulins against flagellin (FLiC) and lipopolysaccharide (LPS) would be able to differentiate CLABSIs from nonbacterial febrile episodes and that levels would increase with infection and decline following appropriate antibiotic treatment. MATERIALS AND METHODS: Patients with IF, due to short bowel syndrome, between the ages of 3 months and 4 years of age, were recruited at Cincinnati Children's Hospital Medical Center. Anti-FLiC and anti-LPS plasma antibody levels were measured in 13 children with IF at baseline, during febrile events, and also following treatment with antibiotics. These were also measured in 11 healthy children without IF who were recruited as controls. RESULTS: Plasma anti-FLiC IgA levels increased during febrile episodes in all patients with IF (baseline mean of 1.10 vs febrile episode mean of 1.32 optical density units, respectively; P = .046). Neither plasma anti-FLiC nor anti-LPS IgA or IgG levels distinguished CLABSI from nonbacterial febrile episodes compared with baseline levels. Compared with controls, patients with IF had significantly higher plasma levels of anti-FLiC and anti-LPS IgA at baseline. CONCLUSION: Plasma anti-FLiC IgA antibody levels rise during febrile episodes but do not differentiate between nonbacterial febrile illnesses and CLABSIs in pediatric IF. However, the upregulation of these antibodies in IF suggests the baseline systemic presence of Gram-negative bacterial products.