Objective To characterize the natural history of intestinal failure (IF) among 14 pediatric centers during the intestinal transplantation (ITx) era. Study design The Pediatric Intestinal Failure Consortium performed a retrospective analysis of clinical and outcome data for a multi-center cohort of infants with IF. Entry criteria included infants <12 mo 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 (n, %). Results 272 infants with a gestational age of 34 wks (30, 36) and birth weight of 2.1 kg (1.2, 2.7) were followed for 25.7 mo (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 PPIs (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 1,000 catheter days. The cumulative incidences for enteral autonomy, death, and ITx were 47%, 27%, and 26%, respectively. Enteral autonomy continued into the 5th year after study entry. Conclusions 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.

Parenteral nutrition (PN) represents one of the most notable achievements of modern medicine, serving as a therapeutic modality for all age groups across the healthcare continuum. PN offers a life-sustaining option when intestinal failure prevents adequate oral or enteral nutrition. However, providing nutrients by vein is an expensive form of nutrition support, and serious adverse events can occur. In an effort to provide clinical guidance regarding PN therapy, the Board of Directors of the American Society for Parenteral and Enteral Nutrition (ASPEN) convened a task force to develop consensus recommendations regarding appropriate PN use. The recommendations contained in this document aim to delineate appropriate PN use and promote clinical benefits while minimizing the risks associated with the therapy. These consensus recommendations build on previous ASPEN clinical guidelines and consensus recommendations for PN safety. They are intended to guide evidence-based decisions regarding appropriate PN use for organizations and individual professionals, including physicians, nurses, dietitians, pharmacists, and other clinicians involved in providing PN. They not only support decisions related to initiating and managing PN but also serve as a guide for developing quality monitoring tools for PN and for identifying areas for further research. Finally, the recommendations contained within the document are also designed to inform decisions made by additional stakeholders, such as policy makers and third-party payers, by providing current perspectives regarding the use of PN in a variety of healthcare settings. (JPEN J Parenter Enteral Nutr. 2017;41:324-377) The etiology-based nutrition diagnoses in adults in clinical practice settings are as follows: Starvation-related malnutrition: Chronic starvation without inflammation (eg, anorexia nervosa). Chronic disease-related malnutrition: Inflammation is chronicand of mild to moderate degree (eg, organ failure, pancreatic cancer, rheumatoid arthritis, sarcopenic obesity). Acute disease or injury-related malnutrition:Inflammation is acute and of severe degree (eg, major infection burns, trauma, closed head injury). 2,3Malnutrition, pediatric: An imbalance between nutrient requirement and intake, resulting in cumulative deficits of energy, protein, or micronutrients that may negatively affect growth, development, and other relevant outcomes. It is recommended that growth charts based on a standard deviation z score system be used to track and assess nutrition status in children. 4,5Nutritionally-at-risk: Consider the individual nutritionally-atrisk if any of the following is present. Nutritionally-At-Risk Adult Summary of RecommendationsThese consensus recommendations are designed to identify best practices, guide day-to-day clinical decisions, reduce variations in practice, and enhance patient safety. They are not intended to supersede the judgment of the healthcare professional based on the circumstances of the individual patient.

Objectives 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. Study design A multicenter retrospective cohort analysis from the Pediatric Intestinal Failure Consortium (PIFCon) was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with PN dependence >60 days. Enteral autonomy was defined as PN discontinuation >3 months. Results 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 NEC [OR 95% CI: 2.42 (1.33, 4.47)], care at an IF site without an associated intestinal transplant (ITx) program [OR 2.73 (1.56, 4.78)] and an intact ileocecal valve (ICV) [OR 2.80 (1.63, 4.83)] as independent risk factors for enteral autonomy. A second model (n=144) including only patients with intra-operatively measured residual small bowel length (RSB) found NEC [OR 3.44 (1.36, 8.71)], care at a non-ITx center [OR 6.56 (2.53, 16.98)] and RSB (cm) [OR 1.04 (1.02, 1.06)] to be independently associated with enteral autonomy. Conclusions A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ICV 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.

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A best-practice CCABSI prevention bundle that included ethanol lock prophylaxis in both the hospital and home was successfully implemented, well tolerated, and demonstrated a significant and sustained reduction in preventable harm in the form of CCABSIs in children with intestinal failure.

Histoplasmosis is an important complication of treatment with TNF-alpha neutralizing agents. Children with IBD treated with aTNF therapy who develop the infection may present with minimal pulmonary symptoms. While discontinuation of aTNF therapy is important initially, few data exist to determine when and how aTNF therapy can be reinstituted. Recognition of Histoplasma capsulatum is often delayed due to the overlap of symptoms with some of the extraintestinal manifestations of IBD and other more prevalent infectious complications.

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Ultrasound-guided percutaneous liver biopsy performed by experienced pediatric interventional radiologists in a children's hospital setting is as safe and effective as biopsy performed by pediatric gastroenterologists.