Chylomicron retention disease: a rare aetiology of failure to thrive.

The aetiology of failure to thrive (FTT) in children is broad, of which some conditions are extremely rare. It is important to consider these rarer conditions, especially in the setting of other concerning signs/symptoms or when there is no improvement with conventional treatment. In this case report we highlight such a rare condition-chylomicron retention disease (CRD) as an aetiology of FTT. CRD often presents with non-specific symptoms, resulting in delayed diagnosis which is established by genetic workup and histology from small intestinal biopsies. Despite being rare, CRD needs to be considered as one of the differential diagnoses after ruling out the more common causes of FTT.

SO,MCT,OO,FO-ILE Is Associated With Better Side Effect Profile Than SO-ILE in Critically Ill Children Receiving Parenteral Nutrition.

OBJECTIVES: This study aimed to evaluate the side effect profile of soybean oil lipid injectable emulsion -(SO-ILE) and soybean oil, medium-chain triglyceride, olive oil, fish oil lipid injectable emulsion (SO,MCT,OO,FO-ILE) in critically ill children requiring parenteral nutrition (PN). METHODS: This is an observational study of children admitted to our pediatric intensive care unit requiring PN for ≥7 days. Patients were divided into 2 cohorts: SO,MCT,OO,FO-ILE (n = 34) and SO-ILE (n = 111). Outcomes included development of hypertriglyceridemia (HTG), intestinal failure-associated liver disease (IFALD), length of stay, and mortality. Logistic regression was performed after controlling for duration and maximum dose of lipids. RESULTS: The median maximum lipid dose was significantly higher in the SO,MCT,OO,FO-ILE cohort (2.7 vs 3 g/kg; p = 0.01). Prevalence of baseline HTG was similar in both cohorts. After excluding patients with baseline HTG, incidence of HTG upon PN introduction was higher in the SO-ILE cohort (51.2% vs 26.7%; p = 0.02). The SO-ILE cohort also had significantly higher triglyceride concentrations at peak and upon discontinuation of PN (p < 0.05). Direct bilirubin and C-reactive protein were significantly higher in the SO-ILE cohort after stopping PN. Five patients (3.4%) developed IFALD, 4 of whom were in the SO-ILE cohort (p = 0.85). Upon logistic regression, mortality rate and incidence of HTG remained significantly higher in the SO-ILE cohort (adjusted odds ratio, 2.3 [95% CI, 1.1-5.3]; p = 0.04; and adjusted odds ratio, 2.0 [95% CI, 1.3-5.1]; p = 0.03, respectively). CONCLUSIONS: In critically ill children requiring PN, SO-ILE was associated with a higher risk of HTG, -elevated direct bilirubin, inflammatory markers and mortality compared with SO,MCT,OO,FO-ILE.

Factors Affecting the Gut Microbiome in Pediatric Intestinal Failure.

BACKGROUND: There is little data on gut microbiome and various factors that lead to dysbiosis in pediatric intestinal failure (PIF). This study aimed to characterize gut microbiome in PIF and determine factors that may affect microbial composition in these patients. METHODS: This is a single-center, prospective cohort study of children with PIF followed at our intestinal rehabilitation program. Stool samples were collected longitudinally at regular intervals over a 1-year period. Medical records were reviewed, and demographic and clinical data were collected. Medication history including the use of acid blockers, scheduled prophylactic antibiotics, and bile acid sequestrants was obtained. Gut microbial diversity among patients was assessed and compared according to various host characteristics of interest. RESULTS: The final analysis included 74 specimens from 12 subjects. Scheduled prophylactic antibiotics, presence of central line associated bloodstream infection (CLABSI) at the time of specimen collection, use of acid blockers, and ≥50% calories delivered via parenteral nutrition (PN) was associated with reduced alpha diversity, whereas increasing age was associated with improved alpha diversity at various microbial levels ( P value <0.05). Beta diversity differed with age, presence of CLABSI, use of scheduled antibiotics, acid blockers, percent calories via PN, and presence of oral feeds at various microbial levels ( P value <0.05). Single taxon analysis identified several taxa at several microbial levels, which were significantly associated with various host characteristics. CONCLUSION: Gut microbial diversity in PIF subjects is influenced by various factors involved in the rehabilitation process including medications, percent calories received parenterally, CLABSI events, the degree of oral feeding, and age. Additional investigation performed across multiple centers is needed to further understand the impact of these findings on important clinical outcomes in PIF.

Performance of Cornell Assessment of Pediatric Delirium Scale in Mechanically Ventilated Children.

Accuracy of delirium diagnosis in mechanically ventilated children is often limited by their varying developmental abilities. The purpose of this study was to examine the performance of the Cornell Assessment of Pediatric Delirium (CAPD) scale in these patients. This is a single-center, prospective, observational study of patients requiring sedation and mechanical ventilation for 2 days or more. CAPD scale was implemented in our unit for delirium screening. Each CAPD assessment was accompanied by a physician assessment using Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-V) criteria. Sensitivity analysis was performed to determine the best cut-off score in our target population. We also evaluated ways to improve the accuracy of this scale in patients with and without developmental delay. A total of 837 paired assessments were performed. Prevalence of delirium was 19%. Overall, CAPD score ≥ 9 had sensitivity of 81.8% and specificity of 44.8%. Among typically developed patients, the sensitivity and specificity were 76.7 and 65.4%, respectively, whereas specificity was only 16.5% for developmentally delayed patients. The best cut-off value for CAPD was 9 for typically developed children and 17 for those with developmental delay (sensitivity 74.4%, specificity 63.2%). Some CAPD questions do not apply to patients with sensory and neurocognitive deficits; upon excluding those questions, the best cut-off values were 5 for typically developed and 6 for developmentally delayed children. In mechanically ventilated patients with developmental delay, CAPD ≥ 9 led to a high false-positive rate. This emphasizes the need for either a different cut-off score or development of a delirium scale specific to this patient population.

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.

Warning Labels and High-Powered Magnet Exposures.

BACKGROUND AND OBJECTIVES: High-powered magnets are among the most dangerous childhood foreign bodies. Consumer advocates and physicians have called for these products to be effectively banned, but manufacturers assert warning labels would sufficiently mitigate risk. METHODS: Subjects from Injuries, Morbidity, and Parental Attitudes Concerning Tiny High-powered Magnets (IMPACT of Magnets), a retrospective, multicenter study of children with high-powered magnet exposures (ie, ingestion or bodily insertion), were contacted. Consenting participants responded to a standardized questionnaire regarding the presence and utility of warning labels, magnet product manufacturer, and attitudes around risk. RESULTS: Of 596 patients in the IMPACT study, 173 parents and 1 adult patient were reached and consented to participate. The median age was 7.5 years. Subjects reported not knowing if a warning label was present in 60 (53.6%) cases, whereas 25 (22.3%) stated warnings were absent. Warnings were present in 28 (24.1%) cases but only 13 (46.4%) reported reading them. A manufacturer was identified by families in 28 (16.1%) exposures; 25 of these were domestic and 27 had warnings. Subjects reported knowing magnets were dangerous in 58% of the cases, although 44.3% believed they were children's toys and only 6.9% knew high-powered magnets were previously removed from the United States market. CONCLUSIONS: Over 90% of subjects from the IMPACT study didn't know if warning labels were present or failed to read them if they were, whereas almost half believed high-powered magnets were children's toys. Warning labels on high-powered magnet products are, therefore, unlikely to prevent injuries in children.

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.

High-Powered Magnet Exposures in Children: A Multi-Center Cohort Study.

BACKGROUND AND OBJECTIVES: High-powered magnets were effectively removed from the US market by the Consumer Product Safety Commission (CPSC) in 2012 but returned in 2016 after federal court decisions. The United States Court of Appeals for the 10th Circuit cited imprecise data among other reasons as justification for overturning CPSC protections. Since then, incidence of high-powered magnet exposure has increased markedly, but outcome data are limited. In this study, we aim to describe the epidemiology and outcomes in children seeking medical care for high-powered magnets after reintroduction to market. METHODS: This is a multicenter, retrospective cohort study of patients aged 0 to 21 years with a confirmed high-powered magnet exposure (ie, ingestion or insertion) at 25 children's hospitals in the United States between 2017 and 2019. RESULTS: Of 596 patients with high-powered magnet exposures identified, 362 (60.7%) were male and 566 (95%) were <14 years of age. Nearly all sought care for magnet ingestion (n = 574, 96.3%), whereas 17 patients (2.9%) presented for management of nasal or aural magnet foreign bodies, 4 (0.7%) for magnets in their genitourinary tract, and 1 patient (0.2%) had magnets in their respiratory tract. A total of 57 children (9.6%) had a life-threatening morbidity; 276 (46.3%) required an endoscopy, surgery, or both; and 332 (55.7%) required hospitalization. There was no reported mortality. CONCLUSIONS: Despite being intended for use by those >14 years of age, high-powered magnets frequently cause morbidity and lead to high need for invasive intervention and hospitalization in children of all ages.

Utility of Body Habitus Parameters to Determine and Improve the Accuracy of the Broselow Tape.

OBJECTIVES: The aims of this study were to determine and improve accuracy of the Broselow Tape (BT) in estimating children's weight by adding body habitus parameters. METHODS: This cross-sectional study was conducted in an urban hospital pediatric clinic. Children up to 8 years old coming in for well-child visit were included. Children with acute illness or presence of any chronic condition potentially resulting in growth disturbance and out of BT height range were excluded. The following body habitus parameters were measured using the Centers for Disease Control and Prevention guidelines: actual weight, predicted weight using BT (BTW), mid-thigh circumference (MTC), body mass index, mid-arm circumference, and waist-to-hip ratio. RESULTS: A total of 301 children were enrolled. Of these, 151 were male (50%). Hispanics constituted 160 (53.2%). There was a positive linear association between BTW and actual weight in the overall cohort (adjusted R2 = 0.9164, P < 0.001). However, there was a difference in this association among children younger than 2 years and children older than 2 years (adjusted R2 = 0.89 vs 0.4841). Incorporating MTC and/or waist circumference along with BTW in the model increased the accuracy, providing a better estimate of actual weight (adjusted R2 = 0.94, P < 0.001). CONCLUSIONS: We conclude that there might be inaccuracies in the weight predicted by BT in our patient population, especially those weighing more than 15 kg and older than 2 years. Our study also demonstrates that MTC correlates closely with the actual weight and could be used in addition to BT for more accurate weight estimation.

Anticipated impact of recent ethanol lock shortage and price increase on practice among pediatric intestinal rehabilitation programs across the United States.

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.

Factors Differentiating Multisystem Inflammatory Syndrome in Children (MIS-C) From Severe/Critical COVID-19 Infection in Children.

OBJECTIVE: To differentiate severe/critical coronavirus disease 2019 (COVID-19) infection from multisystem inflammatory syndrome in children (MIS-C). METHODS: Single-center chart review comparing characteristics of children with MIS-C and 'severe/critical' COVID-19 infection. Multivariate logistic regression was performed to create predictive models for predicting MIS-C. RESULTS: Of 68 patients, 28 (41.2%) had MIS-C while 40 (58.8%) had severe/critical COVID-19 infection. MIS-C patients had a higher prevalence of fever, mucocutaneous, cardiac and gastrointestinal involvement and a lower prevalence of respiratory symptoms (P<0.05). Significantly lower hemoglobin, platelet count, serum electrolytes, and significantly elevated inflammatory and coagulation markers were observed in MIS-C cohort. Upon multivariate logistic regression, the best model included C-reactive protein (CRP), platelet count, gastrointestinal and mucocutaneus involvement and absence of respiratory involvement (performance of 0.94). CRP>40 mg/L with either platelet count <150x109 or mucocutaneous involvement had specificity of 97.5% to diagnose MIS-C. CONCLUSION: Elevated CRP, thrombocytopenia and mucocutaneous involvement at presentation are helpful in differentiating MIS-C from severe COVID-19.

Scheduled Empiric Antibiotics May Alter the Gut Microbiome and Nutrition Outcomes in Pediatric Intestinal Failure.

BACKGROUND: In this study, we aim to determine the effect of scheduled antibiotics on gut microbiome in pediatric intestinal failure (IF) and to evaluate the effect of the gut microbiome on nutrition outcomes in IF. METHODS: Fecal samples were collected at regular intervals from pediatric patients with IF for gut microbiome comparison between 2 cohorts: (group 1) those on scheduled prophylactic antibiotics and (group 2) those who were not on scheduled antibiotics. Gut microbiome composition and diversity were compared among the 2 cohorts. The association among gut microbiome composition, diversity, and nutrition outcomes (mainly ability to decrease parenteral nutrition [PN] energy requirement and ability to attain positive growth) was also determined. RESULTS: The microbiome of patients with IF on scheduled antibiotics differed significantly from those not on scheduled antibiotics. Abundance of certain Gram-negative and pathogenic bacteria (Pseudomonas, Prevotella, and Sutterella) was higher in the scheduled cohort. Patients with decreased Enterobacteriaceae demonstrated a greater ability to demonstrate a reduction in PN requirement, as well as attain positive growth. CONCLUSION: Scheduled antibiotics may alter the gut microbiome in children IF, which in turn may have an influence on important nutrition outcomes in pediatric IF. Further larger, multicenter studies are needed to determine the effect of scheduled antibiotics on the gut microbiome in this patient population and their overall effect on nutrition outcomes.

Opioid use is Associated with ICU Delirium in Mechanically Ventilated Children.

INTRODUCTION: Pediatric delirium is a significant problem when encounterd in an intensive care unit (ICU). The pathophysiology of pediatric delirium is complex and the etiology is typically multifactorial. Even though various risk factors associated with pediatric delirium in a pediatric ICU have been identified, there is still a paucity of literature associated with the condition, especially in extremely critically ill children, sedated and mechanically ventilated. AIM OF THE STUDY: To identify factors associated with delirium in mechanically ventilated children in an ICU. MATERIAL AND METHODS: This is a single-center study conducted at a tertiary care pediatric ICU. Patients admitted to the pediatric ICU requiring sedation and mechanical ventilation for >48 hours were included. Cornell Assessment of Pediatric Delirium scale was used to screen patients with delirium. Baseline demographic and clinical factors as well as daily and cumulative doses of medications were compared between patients with and without delirium. Firth's penalized maximum likelihood logistic regression was used on a priori set of variables to examine the association of potential factors with delirium. Two regression models were created to assess the effect of daily medication doses (Model 1) as well as cumulative medication doses (Model 2) of opioids and benzodiazepines. RESULTS: 95 patient visits met the inclusion criteria. 19 patients (20%) were diagnosed with delirium. Older patients (>12 years) had higher odds of developing delirium. Every 1mg/kg/day increase in daily doses of opioids was associated with an increased risk of delirium (OR=1.977, p=0.017). Likewise, 1 mg/kg increase in the cumulative opioid dose was associated with a higher odds of developing delirium (OR=1.035, p=0.022). Duration of mechanical ventilation was associated with the development of delirium in Model 1 (p=0.007). CONCLUSIONS: Age, daily and cumulative opioid dosage and the duration of mechanical ventilation are associated with the development of delirium in mechanically ventilated children.

Biosimilars in inflammatory bowel disease.

The advent of biologics has changed outcomes in many chronic conditions, including inflammatory bowel disease (IBD). Biologics have been used for the induction and remission of ulcerative colitis and Crohn's disease for almost two decades and are effective in patients who used to fail conventional treatment with steroids, immunomodulators. The use of biologics in the treatment of IBD has increased over the last few years, partly due to the rise in its incidence and the use of biologics as a first-line treatment in severe disease as well as in complicated diseases like penetrating/fistulating Crohn's disease. However, their use is associated with a significant burden to the society with respect to healthcare costs, resulting in the premature discontinuation of therapy in some patients, leading to exacerbations and complications. The introduction of biosimilars a decade ago seems to be a promising approach to reducing the costs related to therapy. Since their introduction, numerous studies conducted in adults and some in children show the efficacy of biosimilars with a similar side-effect profile to biologics. This review discusses the history of biosimilars in the treatment of IBD, enumerates several such studies and discusses the possibility of using biosimilars in the future.

Relations between disease status and body composition in pediatric inflammatory bowel disease.

To evaluate the effect of remission status on physical activity and body composition in pediatric patients with inflammatory bowel disease (PIBD) and healthy peers. Single-center cohort study, including 54 PIBD patients and 33 healthy peers. During the initial study visit, a brief demographic questionnaire, physical activity questionnaire completed by participants, and instructions on recording dietary intake were given. Physicians completed the Physician Global Assessment (PGA) for disease severity. Medical chart abstraction was done to obtain disease variables of interest. DEXA scan completed 1 week later to obtain information on body composition. Variables of interest were compared between the three groups (IBD-Remission, IBD-Active, and healthy controls) using an ANOVA or Chi-square test as appropriate. IBD patients were older than controls, reported lower quality of life (73.9 vs. 80.9), and engaged in less MVPA (195.4 versus 361.1). The IBD-Active group had a significantly lower lean body mass, bone mineral density, and time spent in MVPA compared to the IBD-Remission group and healthy controls. IBD-Remission group had a significantly lower percentage of biologic use (55% vs. 87%) and comorbidities (26% vs. 44%) compared to IBD-active group. IBD-remission group also had a lower fat mass percentage. In this study, we report significantly favorable LBM, BMD, and time spent in MVPA in patients with IBD in remission compared to those not in remission with the former demonstrating a body composition resembling that of healthy peers.Conclusion: While an improvement in BMD was observed with remission, the scores were still lower than controls. What is Known: • Body composition deficits in pediatric inflammatory bowel disease are common and some persist despite achievement of remission leading to long term outcomes including osteopenia and osteoporosis. • Weight restoration in patients with pediatric IBD is primarily explained by gains in fat mass without similar gains in lean mass. What is New: • While an improvement in bone mineral density was observed, the achievement of remission significantly improves affects physical activity and body composition in pediatric inflammatory bowel disease. • However, some parameters of body composition do not reach levels comparable to healthy peers.

Serum Transaminases at Presentation and Association with Acute Dialysis in Children with Hemolytic Uremic Syndrome.

Background: To determine whether serum transaminases at presentation predict the need for dialysis in children with hemolytic uremic syndrome (HUS). Methods: Single-center, retrospective chart review of pediatric patients with HUS. Data collected included demographics, clinical and laboratory parameters, and need for dialysis. These factors were compared between two groups: "dialysis" versus "no dialysis." Continuous data were compared using a t test whereas categoric data were compared by the chi-squared test. Multivariate logistic regression was performed on a prior set of variables to determine if serum transaminases independently predict the need for dialysis. Results: A total of 70 children were included in the study, of which, 39 (27%) received dialysis. The no-dialysis group had a higher proportion of white patients compared with the dialysis group (74% dialysis versus 94% no dialysis). The only clinical sign at admission associated with dialysis was reduced urine output (56% versus 16%, P<0.001). Univariate logistic regression identified admission serum creatinine, aspartate transaminase (AST), and alanine transaminase (ALT) to be associated with the need for dialysis. Multivariate logistic regression showed serum AST and ALT to be independent predictors of the need for dialysis, with both improving the performance of the regression model. Sensitivity analysis showed a cutoff of 129 U/L for AST and 83 U/L for ALT with high specificity. Conclusions: Serum transaminases at presentation are independently associated with the subsequent need for dialysis in patients with HUS. Our study suggests that when both serum ALT and AST are normal, the likelihood to need dialysis is very low; alternatively, when both serum ALT and AST are more than two times the upper level of normal, the need for dialysis is very high.

Perforated Meckel Diverticulum: A Rare Cause of Acute Abdomen in Children.

Meckel diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract and the most common cause of gastrointestinal bleeding in children. Although it usually follows the rule of 2's, exceptions to this rule are reported in the literature. Often asymptomatic, MD is commonly an incidental finding during surgical interventions. When symptomatic, the most common presentation of this condition is painless rectal bleeding. A myriad of other nonspecific symptoms are however possible, especially in adults, thus making this diagnosis difficult. Meckel diverticulum has been reported to mimic other abdominal pathologies like appendicitis, inflammatory bowel disease, and pancreatitis to name a few.We report a patient with acute abdomen in whom the more common causes of acute abdomen were ruled out and a diagnosis of MD was established on exploratory laparoscopy, only after he developed perforation. This report emphasizes the need for maintaining a high index of suspicion towards a possibility of a complicated MD in patients presenting with an acute abdomen, once other causes of acute abdomen are ruled out.