Ethan A. Mezoff

Objective: Essential fatty acid deficiency (EFAD) occurs when daily nutrition provisions lack sufficient linoleic acid or a‐linolenic acid. Signs, symptoms, and sequelae are nonspecific but impact health and quality of life. The objective of this study was to determine the incidence of EFAD among pediatric patients with chronic, complex healthcare needs. Methods: This single‐center, cross‐sectional study included individuals from a specialty clinic designed for children with medical complexity. Clinically stable, exclusively tube‐fed patients aged 4–21 years were prospectively enrolled to collect a serum fatty acid panel. Individual fatty acids, anthropometrics and diet history were analyzed with descriptive statistics and linear regression analysis. Results: Among 77 patients, the median triene:tetraene (T:T) ratio was 0.022 (interquartile range 0.019–0.028). Overall, linoleic, a‐linolenic, and mead acid levels were within standard reference ranges with median levels of 3266, 101, and 22 nmol/mL, respectively. Two patients met criteria for biochemical EFAD with T:T ratios of 0.052 and 0.062. There was no difference in T:T ratio by formula type. There was a negative correlation with daily calorie intake and T:T ratio. Patients identified as overweight had slightly higher average T:T ratios. Conclusions: The incidence of EFAD in this cohort was low at 2.6%. Fatty acid profiles do not need to be collected routinely. However, they should be considered if signs and symptoms of deficiency are present or in the setting of other identified risk factors, specifically obesity or additional reasons for calorie restriction below 40 kilocalories per kilogram daily.

Objectives: Preterm infants confront multiple coexistent health risks and may not receive human milk (HM), which contains human milk oligosaccharides (HMOs), among other biologically active, beneficial components. 2′-Fucosyllactose (2′-FL) is the most abundant HMO in most HM. Interventional studies of formula with added HMOs have demonstrated multiple beneficial outcomes in healthy, term infants. Methods: This double-blind, randomized, placebo-controlled pilot study (NCT03306316) evaluated the effect of a 2′-FL supplement on growth, tolerance, and neurobehavior, as assessed by the Neonatal Intensive Care Unit (NICU) Network Neurobehavioral Scale (NNNS), in preterm infants. Eligible neonates born between 26 0/7 and 31 6/7 weeks gestational age (GA) were enrolled from three NICUs in the United States. Preterm infants consuming mother’s own milk or donor human milk were randomized to receive a supplement of 0.135 g/mL of 2′-FL or 0.05 g/mL dextrose (placebo) twice daily until 36 weeks corrected GA, NNNS assessment, or hospital discharge, whichever came first. Results: Of 42 participating preterm infants, 23 (55%) received 2′-FL and demonstrated no significant differences in growth in weight, length, or head circumference over 5 weeks. There were also no differences in stool frequency, adverse (AEs) or serious adverse events (SAEs), or neurobehavior (NNNS) between groups. Conclusions: This pilot study found that 2′-FL supplementation in preterm infants through 36 weeks corrected GA was safe and well-tolerated and supported adequate growth and neurobehavior.

Background: Multidisciplinary care under intestinal rehabilitation programs (IRPs) improves survival in pediatric intestinal failure (IF). Professional societies recommend the management of pediatric patients with IF by an IRP. Whether these recommendations are followed in cases of neonatal IF is currently unclear. The objective of this study was to describe the current state of neonatal IF care across North America through the Children's Hospitals Neonatal Consortium. Methods: A web‐based survey was sent in July 2023. This survey consisted of adaptive questioning and contained 10–54 questions. The survey covered topics regarding IF care: diagnosis, rehabilitation program management, protocol use, and follow‐up. To determine correlation of respondent role and answer given, Spearman correlation was used to analyze a portion of responses. Results: There was a response rate of 93% (42/45). A total of 79% (33/42) of centers report having an IRP caring for neonatal patients. The composition and care provided by the program varied by center, with 60% (25/42) having protocols for neonatal IF management. A total of 76% (32/42) of centers report a multidisciplinary intestinal rehabilitation follow‐up. Conclusions: Neonatal IF care varies across North America. Although the presence of a rehabilitation program is known to improve pediatric patient outcomes, a relevant minority of centers in this consortium do not have access to this care. Future studies comparing neonatal specific IF care strategies are critically important to optimize outcomes.

From the time of their initial surgery, children with short bowel syndrome (SBS) begin rehabilitating their remaining bowel in a quest towards reaching enteral autonomy. Even in the earliest experiences from 1987, nearly 98% of children with intestinal failure (IF) attend school during this process and 50% of children were shown to adapt defined as achieving independence from parenteral nutrition (PN)

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.

Introduction: Hospital‐acquired central line‐associated bloodstream infections (CLABSI) are “never events” in U.S. healthcare. National efforts to improve CLABSI rates are ongoing. Efforts are important for all patients with a central venous catheter (CVC) and critical to children with intestinal failure (IF) who depend on long‐term, daily use of a CVC and undergo extended hospitalizations. We describe outcomes of a multidisciplinary CLABSI elimination effort on a 24‐bed medical‐surgical unit caring for children with IF.Methods: Unit CLABSI events from 1/9/2012 to 4/16/2020 were evaluated with multiple improvement interventions. We leveraged prospectively maintained clinical registries and National Healthcare Safety Network (NHSN) reporting data to extract patient and unit demographics, ethanol lock utilization, and unit CVC days. Interventions were developed utilizing expert consensus and CDC guidelines with active frontline staff engagement. Descriptive statistics and tests of non‐parametric data were employed for analysis.Results: Ninety‐five patients with IF and 862 non‐IF patients experienced a total of 1,629 admissions with 20,372 CVC days. Twelve hospital‐acquired CLABSI events occurred during the study period, including 7 following NHSN definition change on 1/1/2015 (0.56 per 1,000 CVC days). After the last unit CLABSI on 12/5/2016, there were 7,117 CVC days through study conclusion.Conclusions: Described interventions with an enhanced culture of collaborative care profoundly improved hospital‐acquired CLABSI occurrence. Success in a specific population translated to all other unit patients with a CVC. Findings suggest elimination is not the result of a single new product or practice, but also includes support and empowerment of those caring for the patient and their CVC.

Background: Ethanol lock use has been associated with significantly lower rates of central line–associated bloodstream infection (CLABSI) in children with intestinal failure. Concerns have been raised among intestinal rehabilitation program providers regarding the impact of recent changes in cost and availability of ethanol locks in the US.Methods: We conducted a survey among the members of the North American Society For Pediatric Gastroenterology, Hepatology & Nutrition Intestinal Rehabilitation Special Interest Group (NASPGHAN IR‐SIG) regarding practice changes among providers to tackle this issue and the anticipated effect on CLABSI rates.Results: The results show that the vast majority of US participants use ethanol locks in their population with intestinal failure, with most anticipating or already experiencing reduction in access to ethanol locks. Most worrisome is that more than half of participating programs expect an increase in CLABSI rates in this vulnerable patient population as a consequence of limited access to ethanol locks.Conclusion: Further multicenter prospective studies to assess the efficacy of alternative locking agents, besides ethanol, are needed in order to have readily available and affordable options for CLABSI prevention in the future.

Introduction: Central line–associated bloodstream infections (CLABSIs) lead to significant morbidity and mortality in children with intestinal failure (IF). Ethanol lock prophylaxis (ELP) greatly reduces CLABSI frequency with minimal side effects. However, in the United States, a recently approved orphan drug designation for dehydrated alcohol has greatly increased 70% ethanol cost from about $10/day to $1000/day. We examined the cost‐effectiveness of ELP in relation to these changes.Methods: We simulated a previously developed IF Markov model over 1 year. Costs were measured in 2020 US dollars and effectiveness in quality‐adjusted life‐years (QALYs). CLABSI rate with and without ELP was estimated from the largest available comparative observational study. The primary outcome was incremental cost‐effectiveness ratio (ICER) between treatments. Secondary outcomes included CLABSI frequency. Sensitivity analyses on all model parameters were performed.Results: In the base model, children with IF not using ELP accumulated $131,815 in costs and 0.32 QALYs per patient compared with $437,884 and 0.33 QALYs per patient in those using ELP. The ICER was nearly $17 million/QALY gained. ELP resulted in a 40% reduction in CLABSI frequency. ELP became cost‐effective at $68/day and cost‐saving at $63/day. Sensitivity analysis identified no other plausible parameter variation to reach the benchmark of $100,000/QALY gained.Conclusions: At the current price, ELP is not cost‐effective for CLABSI prevention in children with IF in the United States. This study highlights the critical need for the approval of an affordable lock therapy option to prevent CLABSIs in these children.