Evaluation and Management of Iron Deficiency in Children Undergoing Intestinal Rehabilitation-A Position Paper From the NASPGHAN Intestinal Rehabilitation Special Interest Group.

Iron deficiency (ID) is the most common nutritional deficiency affecting children undergoing intestinal rehabilitation (IR). Patients may be asymptomatic or present with nonspecific symptoms including fatigue, irritability, and dizziness. The diagnosis of ID in this population can be complicated by the coexistence of systemic inflammation or other nutritional deficiencies which may mimic ID. Many routinely available laboratory tests lack specificity and no consensus on screening is available. Success in oral and enteral treatment is impeded by poor tolerance of iron formulations in a population already challenged with intolerance. Newer parenteral iron formulations exhibit excellent safety profiles, but their role in repletion in this population remains unclear. The following report, compiled by a multidisciplinary group of providers caring for children undergoing IR and representing the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Special Interest Group for Intestinal Rehabilitation, seeks to address these challenges. After discussing iron physiology and population-specific pathophysiology, we make recommendations on iron intake, iron status assessment, and evaluation for alternative causes of anemia. We then provide recommendations on iron supplementation and treatment of ID anemia specific to this nutritionally vulnerable population.

The 21st Century CURES Act in Pediatric Gastroenterology: Problems, Solutions, and Preliminary Guidance.

ABSTRACT: The information blocking (IB) prohibition component of the 21st Century CURES Act (21CCA) comes into effect April 5, 2021, which gives patients and their families near-instant access to almost all clinical notes, lab results, and health data. Exceptions to IB prohibition include risk of harm and patient privacy, but violations can be punished by a fine of up to $1,000,000.00. A committee of pediatric gastroenterologists reviewed the 21CCA regulation and compared local practice policies. Pediatric practitioners need to understand how age will affect local information release policies and to know which note types are released, paying special consideration to trainee notes and confidential information. Extraneous detail should be removed from notes, emotional labeling be avoided, and objective statements be made when referring to the care of other providers. Awareness of the 21CCA provides pediatric gastroenterologists with the opportunity to adapt their medical documentation practices to accommodate the new law.

Management of Central Venous Access in Children With Intestinal Failure: A Position Paper From the NASPGHAN Intestinal Rehabilitation Special Interest Group.

ABSTRACT: Intestinal failure requires the placement and maintenance of a long-term central venous catheter for the provision of fluids and/or nutrients. Complications associated with this access contribute to significant morbidity and mortality, while the loss of access is an increasingly common reason for intestinal transplant referral. As more emphasis has been placed on the prevention of central line-associated bloodstream infections and new technologies have developed, care for central lines has improved; however, because care has evolved independently in local centers, care of central venous access varies significantly in this vulnerable population. The present position paper from the Intestinal Failure Special Interest Group of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) reviews current evidence and provides recommendations for central line management in children with intestinal failure.

Single High-dose Vitamin D3 Supplementation in Pediatric Patients With Inflammatory Bowel Disease and Hypovitaminosis D.

OBJECTIVES: The primary aim was to determine the effectiveness of a single high-dose of oral vitamin D3 (stoss therapy) in children with inflammatory bowel disease (IBD) and hypovitaminosis D. Our secondary aim was to examine the safety of stoss therapy. METHODS: We conducted a randomized, prospective study of 44 patients, ages 6 to 21 years, with IBD and 25-hydroxyvitamin D (25-OHD) concentrations <30 ng/mL. Patients were randomized to receive 50,000 IU of vitamin D3 once weekly for 6 weeks (standard of care, SOC group) or 300,000 IU once (stoss group). Serum 25-OHD levels were obtained at baseline, 4 and 12 weeks. Safety monitoring labs were performed at week 4. RESULTS: Thirty-nine of 44 enrolled patients (19 stoss, 20 SOC) completed the study. Baseline vitamin D levels were not significantly different between the groups. Stoss therapy resulted in a substantial rise in 25-OHD levels at week 4, equivalent to the weekly regimen (53.6 ±â€Š17.3 vs 54.6 ±â€Š17.5 ng/mL). At week 12, serum 25-OHD levels decreased in both groups, significantly lower in the stoss group, but remained close to 30 ng/mL (29.8 ±â€Š7.1 vs 40.4 ±â€Š11.9 ng/mL, P = 0.04). A significant interaction with treatment group over time was observed (P = 0.0003). At the week-4 time point, all patients who received stoss therapy had normal serum calcium and PTH levels. Eighty percentage of patients preferred stoss therapy to the weekly regimen. CONCLUSIONS: Stoss therapy was safe and effective in raising 25-OHD in children with IBD commensurate to that of the weekly regimen.

A Procedural and Educational Experience Following Creation of an Advanced Pediatric Endoscopy Service.

Advanced endoscopic procedures occur infrequently enough in pediatric patients to preclude effective maintenance of competence among all pediatric gastroenterologists. A recent study suggests that fellows are largely unable to achieve the prescribed case volume recommended to achieve competence. We sought to describe the procedural and educational experience following the creation of an advanced pediatric endoscopy service in response to declining confidence among practice members regarding advanced procedures. We found most advanced endoscopy cases (90%) were accomplished during routine business hours with little seasonal variation. Esophageal dilations occurred far more than all other procedures provided by this service. Control of nonvariceal bleeding, feeding tube placement, enteroscopy, and needle knife therapy, among others, were performed exclusively but relatively infrequently by members of this advanced endoscopy service. Fellows were present for many cases, although they participated in relatively few. We conclude that the creation of an advanced endoscopy service permits distillation of rare but technically demanding cases to few providers, ensuring maintenance of skills, although the role of fellows remains in question.

The human milk oligosaccharide 2'-fucosyllactose augments the adaptive response to extensive intestinal.

Intestinal resection resulting in short bowel syndrome (SBS) carries a heavy burden of long-term morbidity, mortality, and cost of care, which can be attenuated with strategies that improve intestinal adaptation. SBS infants fed human milk, compared with formula, have more rapid intestinal adaptation. We tested the hypothesis that the major noncaloric human milk oligosaccharide 2'-fucosyllactose (2'-FL) contributes to the adaptive response after intestinal resection. Using a previously described murine model of intestinal adaptation, we demonstrated increased weight gain from 21 to 56 days (P < 0.001) and crypt depth at 56 days (P < 0.0095) with 2'-FL supplementation after ileocecal resection. Furthermore, 2'-FL increased small bowel luminal content microbial alpha diversity following resection (P < 0.005) and stimulated a bloom in organisms of the genus Parabacteroides (log2-fold = 4.1, P = 0.035). Finally, transcriptional analysis of the intestine revealed enriched ontologies and pathways related to antimicrobial peptides, metabolism, and energy processing. We conclude that 2'-FL supplementation following ileocecal resection increases weight gain, energy availability through microbial community modulation, and histological changes consistent with improved adaptation.

The winding road to understanding the neonatal origins of inflammatory gastrointestinal disorders.

Beginning with the observation that birth weight correlates with increased risk of cardiovascular disease, the concept of neonatal programming, that the environmental influence on fetal and neonatal development results in modification of the risk profile for adult disease, has begun to emerge as an important component to understanding the origin of chronic diseases of many different organ systems. Until recently, the gastrointestinal system has not been considered. Our understanding of the pathogenesis of many intestinal inflammatory disorders is still incomplete; however, a brief review of what is known reveals several opportunities for the early intraluminal environment to affect the development of the intestinal immune system. Early clinical observations such as the increased risk of celiac disease observed in those born by cesarean section and the protective effect of breast-feeding against inflammatory bowel disease and celiac disease support the role of neonatal programming in the development of chronic inflammatory gastrointestinal disease. Additional, more robust clinical studies are needed to confirm this role. Furthermore, examination of the possible mechanisms of immune phenotype modification is necessary.

Should Clinicians Prescribe Non-FDA Regulated Dietary Supplements When Caring for Children With Hypovitaminosis D?

Hypovitaminosis D is a prevalent micronutrient deficiency that can be severe and hard to treat in children with short bowel syndrome, a condition treated with substantial bowel resection. Surgically altered bowel anatomy then results in iatrogenic digestion and absorption limitations that require short- and long-term management and follow-up. Care of children with hypovitaminosis D standardly includes prescription dietary micronutrient supplementation, sometimes in irregularly high doses. This commentary responds to a pediatric case of vitamin D toxicity and suggests micronutrient-prescribing risk mitigation strategies in light of the absence of regulatory oversight of over-the-counter dietary supplements, inadequate insurance coverage, and easily available commercial retail products.

Bacterial overgrowth assessment and treatment among pediatric intestinal rehabilitation and nutrition support providers: An international survey of clinical practice patterns.

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.

Cost-effectiveness of ethanol lock prophylaxis to prevent central line-associated bloodstream infections in children with intestinal failure in the United States.

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.

Elimination of hospital-acquired central line-associated bloodstream infection on a mixed-service pediatric unit.

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.

Biochemical Parameters in Extremely Preterm Infants Receiving Mixed Lipid Emulsions.

OBJECTIVE: A mixture of soybean, medium-chain triglycerides, olive, and fish oils (SMOF) contains higher α-tocopherol and n-3 polyunsaturated fatty acids and lower phytosterol content compared with conventional soybean oil lipid emulsions (SOLE). We sought to characterize plasma total fatty acid profiles (FAPs) and assess the tolerability of long-term SMOF therapy in extremely preterm infants. METHODS: We retrospectively evaluated infants born <28 weeks gestational age who received at least 30 consecutive days of SMOF between July 2016 and June 2019. We evaluated monthly FAPs and biochemical tolerance to SMOF using direct bilirubin (DB) and triglyceride (TG) levels. Growth parameters were evaluated longitudinally until discharge. RESULTS: Sixteen patients with median gestational age 24 weeks (IQR, 23-25 weeks) received SMOF for median 76 days (IQR, 52-130 days). Fourteen patients had necrotizing enterocolitis (NEC) requiring surgical intervention and 15 patients received SOLE for median 19 days (IQR, 14-26 days) prior to switching to SMOF. Median docosahexaenoic acid (DHA) and eicosapentaenoic acid (EPA) levels were elevated, whereas the remaining fatty acid levels fell within reported reference ranges. There were no incidents of essential fatty acid deficiency (triene to tetraene ratio >0.2) or hypertriglyceridemia (TG >200 mg/dL) with a general downtrend in DB after the first month on SMOF. All growth Z-scores declined throughout hospital stay. CONCLUSIONS: Infants who received SMOF had a more pronounced elevation in DHA than EPA, of which the clinical significance remains unknown. Growth Z-scores declined with SMOF but were confounded by a high prevalence of surgically treated NEC.

Case Report: Acute Abdominal Pain as Presentation of Pneumonia and Acute Pancreatitis in a Pediatric Patient With COVID-19.

Abdominal pain, nausea, and vomiting are known gastrointestinal symptoms of symptomatic SARS-CoV-2 infection (COVID-19 disease) in pediatric patients.1 There is little literature regarding pancreatitis in COVID-19. We describe a 16-year-old male diagnosed with acute pancreatitis in the setting of a SARS-COV-2 infection and associated fluid balance considerations.

Intestinal Failure: A Description of the Problem and Recent Therapeutic Advances.

Pediatric intestinal failure occurs when gut function is insufficient to meet the nutrient and hydration needs of the growing child. The commonest cause is short bowel syndrome with maldigestion and malabsorption following massive bowel loss. The remnant bowel adapts during the process of intestinal rehabilitation. Management promotes the achievement of enteral autonomy while mitigating the risk of comorbid disease. The future of care is likely to see expansion of pharmacologic methods for augmenting bowel adaptation, tissue engineering techniques enabling immune suppression-free autologous bowel transplant, and the development of electronic health record tools for efficient, collaborative study and care improvement.

Gastrointestinal Endoscopy in the Neonate.

Gastrointestinal endoscopy permits direct observation of the alimentary tract, acquisition of mucosal tissue for histopathologic examination, and other diagnostic and therapeutic maneuvers. Endoscopes of appropriate size for many neonates and an expanding array of compatible tools and accessories have broadened what is possible, although few neonatal data exist to guide use. Evaluation and treatment of gastrointestinal bleeding, evaluation and dilation of fibromuscular congenital esophageal stenosis, and the bedside placement of gastrostomy tube have been described. Careful consideration of risks, benefits, and discussions between involved specialties permit patient-specific application of these tools and techniques to augment care of this vulnerable population.